SARS-CoV-2 infection can present with various clinical features, among which gastrointestinal manifestations such as nausea, diarrhea, vomiting, and mild abdominal pain have been reported. Recognition of rare presentations of SARS-CoV-2 infection has increased over time. These atypical and rare presentations may lead to difficulties in establishing the diagnosis in a timely manner; furthermore, they may lead to unnecessary investigations, extended hospital stays, adverse outcomes, and more strain on healthcare resources. We present three cases admitted to our hospital with a picture that mimicked an acute abdomen, necessitating surgical assessment and evaluation. All cases turned out to be SARS-CoV-2 positive and did not require surgical management. We discuss the management course, highlight the importance of abdominal symptoms in the setting of COVID-19, and discuss the implications of this association for medical practice amid the current pandemic in both resource-rich and resource-limited settings. We share our experience with three cases presenting with acute abdominal pain necessitating, abdominal imaging, and surgical evaluation.COVID-19 typically not only affects the respiratory system but can also affect other body organs. The manifestations differ according to the system predominantly affected. A meta-analysis including data of around 2,000 patients revealed that fever (89%), cough (69%), and muscle aches with fatigue (36%) are the most prevalent symptoms, whereas nausea, vomiting, and diarrhea were less common (4–5%).The first case was a 40-year-old woman who presented with a 2-day history of a vague right lower quadrant (RLQ) abdominal pain that was severe on the day of admission. On further inquiry, she reported fever, nausea, and vomiting but no diarrhea. Examination revealed RLQ abdominal tenderness, however, with no guarding or rigidity. Laboratory workup was unremarkable, except for a mild elevation of the C-reactive protein (CRP) . ComputeThe second case was a 44-year-old man who presented with excruciating right upper quadrant (RUQ) abdominal pain. Further questioning revealed only a 5-day history of mild dry cough preceding the pain. No other symptoms were present. The abdominal examination revealed guarding and right hypochondriac tenderness but no organomegaly. Other systemic examinations, including the respiratory system, were unremarkable. Initial laboratory tests were significant for a mild CRP rise . AbdominThe third case was a 53-year-old man known to have diabetes, hypertension, and end-stage renal disease. The chief complaint was severe mid-upper abdominal pain, associated with nausea, vomiting, and diarrhea of 2-day duration. A systemic query was insignificant otherwise. The examination was significant for fever, and mild epigastric tenderness that did not correlate with the severe pain the patient described. Other systemic examinations, including the respiratory system, were unremarkable. Laboratory evaluation revealed a mild CRP rise and an elevated lipase 230 UL . He was labeled as a case of acute pancreatitis, and conservative management commenced. An abdominal CT was performed two days later because of non-improvement and did not show evidence of pancreatitis. The case was discussed with infectious disease colleagues, and, in light of our experience with the previous two cases, we requested a chest X-ray, which showed patchy infiltrates in the left upper and lower lung zones. Hence, the patient was tested for COVID-19 RT-PCR, and it was reported to be positive. The patient was started on COVID-19 pneumonia treatment . The pain resolved within the first week of the admission. Thereafter, he remained asymptomatic until hospital discharge on day 29.Anti–COVID-19 therapeutic agents varied between cases. This was according to local guidance at the time of each hospitalization and to medication availability. All three cases did not need critical care unit admission, and their hospitalization course was uncomplicated otherwise.3 Vague presentations of COVID-19 in the midst of this pandemic, as depicted by our cases, make this evaluation even more challenging. We shared our experience with three cases that constituted an initial diagnostic dilemma. History of fever in cases 2 and 3 and the history of mild cough in case 2 was useful in reaching the diagnosis. However, the dilemma persisted until the management was commenced, and patients’ pain settled.The acute abdomen is a surgeon’s challenge. It can lead to grave consequences if not identified early and treated promptly.4 performed a retrospective analysis of all acute abdominal pain cases admitted to their institute. Their evaluation revealed that nine of 79 cases tested positive for COVID-19. These patients had no respiratory symptoms. Six of nine cases had a chest CT scan abnormality. The authors postulated the role of angiotensin-converting enzyme-2 (ACE2) receptor in the pathogenesis of abdominal pain. The virus binds to the ACE2 receptor, and the receptor can be found in the lungs and various GI system structures, including the intestines.5 Bhayana et al.6 reported abdominal imaging findings of COVID-19 patients. In their report, four patients were found to have findings suggestive of nonocclusive mesenteric ischemia. Moreover, on laparotomy, an atypical yellow color was present. Bhayana et al. suggested a role of ACE2 receptor, and the possibility of direct vasculature invasion by the virus or occlusion resulting from the microthrombus formation.Saeed et al.7 This may have been the cause of pain in case 3; however, he had mild lipase rise, no significant abdominal tenderness, and CT scans showing no evidence of pancreatitis.Consequent to this, microthrombus formation leading to pain can also be added to the causes of pain in our patients. It may have led to appendagitis in case 2 and mild undiagnosed mesenteric ischemia in case 3 . Pain in cases 1 and 3 could be radiating pain from lower lung involvement. However, the pain in case 1 was only limited to the RLQ, making it less likely to be the sole cause of this patient’s pain. Pancreatitis has been reported in association with COVID-19.Based on this limited experience, it seems prudent to have a low threshold for COVID-19 infection when dealing with acute abdominal pain, especially atypical. This is even of more importance in resource-limited settings, where there is a need to preserve valuable resources and avoiding burdening the patients and health systems. Notwithstanding this, appropriate evaluation of acute abdominal cases should proceed, maintaining personal protective equipment, as not to miss relevant surgical cases. Abdominal CT scan with the involvement of mid to lower cuts of the chest is of additional value in the evaluation of such cases whenever available. As time passes, more data will accrue that will help us better understand this disease and its implications on various medical and surgical services.In conclusion, severe abdominal pain can be the presenting feature of COVID-19. Clinicians should be aware of this presentation and should have a low threshold to diagnose it amid the current pandemic. Overlooking such diagnosis may lead to improper triage, leading to in-hospital SARS-CoV-2 transmission. In addition, it may lead to improper investigation and management that may be invasive.


A 56-year-old male with a history of infection of human immunodeficiency virus over ten years, was referred to our center because of intermittent epigastralgia and gradual increase of abdominal girth in the last two months. Physical examination revealed palpable abdominal mass at the right upper quadrant measuring around 20cm. Laboratory examinations of complete blood counts, urine tests, and tumor markers were otherwise normal. CT scan of the abdomen showed a huge fatty mass of 23.3 x 22.9 x 34.5cm with mixed density and pathological contrast enhancement arising in the retroperitoneum. The mass displaced right kidney in epigastrium    and mosRetroperitoneum is the primary site in about 15% of soft tissue sarcomas (STS)   . The diInfection with the human immunodeficiency virus (HIV) and the subsequent destruction of T4-positive helper cells are associated with the development of various malignancies.HIV-infected patients may be at greater risk for other forms of cancer because of changes in immune surveillance. In immunodeficient populations, other than Kaposi sarcoma and other sarcoma types, only leiomyosarcoma and angiosarcoma occur disproportionately in these patients   . Liposa


In this report, the case of a 24-year-old female with Beckwith–Wiedemann Syndrome (BWS) who was diagnosed with well-differentiated hepatocellular carcinoma (HCC) is described. While BWS has been associated with childhood embryonal tumors, most commonly Wilms tumors and hepatoblastomas, this is the first case report to describe HCC in an adult with BWS. Although HCC typically occurs in elderly adults or those with underlying liver disease, in this case, we show that HCC can occur in a young adult with BWS without any underlying liver disease. Beckwith–Wiedemann Syndrome (BWS) is a rare genetic disorder characterized by macroglossia, abdominal wall defects, facial nevus flammeus, hypoglycemia, organomegaly, and hemihypertrophy. The syndrome is also associated with many childhood embryonal malignancies, most frequently Wilms tumors and hepatoblastomas. In this case report, we describe the diagnosis of hepatocellular carcinoma (HCC) in a 24-year-old woman with BWS. This is the first case report to describe HCC in an adult with this syndrome.A 24-year-old Caucasian woman with a past medical history significant for congenital BWS presented with vague abdominal pain that waxed and waned in intensity beginning in August 2018. The patient had clinical manifestations of BWS at birth with characteristic umbilical hernia, macroglossia, and hemihypertrophy of her right lower extremity. Genetic analysis confirmed the diagnosis of BWS in January 2011 with uniparental disomy of the chromosome 11 region, hypermethylation of the H19DMR (IC1) loci, and hypomethylation of the KvDMR (IC2) loci. In addition to diagnosis of BWS, the patient's past medical history was significant for partial pancreatectomy due to pancreatic cystic lesions, left mastectomy, and several right-sided lumpectomies due to breast fibroadenomas, refractory immune thrombocytopenic purpura requiring splenectomy, and systemic lupus erythematosus.F-actin) immunoglobin G. Ceruloplasmin, antimitochondrial antibody, alpha-1-antitrypsin, and viral hepatitis serologies were negative. Liver function was preserved, but a marker of liver inflammation, aspartate aminotransferase, was mildly elevated to 65. Alanine aminotransferase was normal at 39, and the patient's alkaline phosphatase was elevated to 211.In May 2019, she was found on CT chest, abdomen, and pelvis to have interval development of parenchymal heterogeneity and multiple enhancing nodules and masses in the liver measuring up to 3.6 cm in the right dome . Two monGiven the above imaging and laboratory results, an ultrasound-guided liver biopsy was performed by interventional radiology in October 2019. The biopsy was described as a hepatocellular proliferation with thickened cell plates and pseudoglands . No portSerial sections of the surgical specimen revealed a 2.9 × 2.8 × 2.0 cm tan, soft, and hemorrhagic mass with irregular borders. Histological examination demonstrated similar findings to those seen in the biopsy, with diffusely thickened cell plates and pseudoglands Figures . The retTo our knowledge, this is the first case report in the literature to describe hepatocellular carcinoma (HCC) in an adult with BWS. BWS is a rare genetic syndrome which may present with macroglossia, abdominal wall defects, facial nevus flammeus, hypoglycemia, organomegaly, and hemihypertrophy.  The prevBWS is associated with many childhood benign and malignant malignancies, most commonly embryonal carcinomas. The overall estimated neoplastic risk is 8%.  The most−10. This indicates that HCC in a young adult with BWS is unlikely to be due to chance.There have been no case reports in the literature to describe HCC in an adult with BWS. HCC is the most common primary hepatic malignancy.  The majoThis is the first case report in the literature to describe HCC in a young adult with BWS. While the risk for malignancy is highest for patients with BWS during the first decade of life, this case report describes the case of HCC in a 24-year-old woman with this syndrome, highlighting the need for life-long cancer surveillance in this patient population.


Hepatoid carcinoma of the ovary (HCO) is a rare disease that originates from the ovarian surface epithelium. It is histologically characterized as hepatocellular carcinoma (HCC) with a hepatocyte-rich granular cytoplasm.A 65-year-old female patient was admitted with complaints of indigestion, abdominal bloating, and pain.The patient showed an elevated level of serum alpha-fetoprotein (AFP) with abdominal bloating and pain. After surgery and histopathology analysis, she was finally diagnosed with HCO, Figo stage IC.After cytoreductive surgery, she underwent adjuvant chemotherapy with carboplatin and paclitaxel. Although the disease was diagnosed at an early stage, it recurred 6 months after completion of adjuvant chemotherapy. Elevation of serum AFP level and removal of a mass from the lumbar vertebra confirmed the recurrence of this disease. Subsequently, the patient underwent radiation therapy and palliative chemotherapy.She died 31 months after the diagnosis due to disease progression.The aggressive nature of HCO was clearly observed in this case despite early diagnosis and treatment. Further studies are needed to understand the proper treatment and prognostic factors of HCO. It has been reported most commonly in the gastrointestinal tract, especially in the stomach. Other organs include the lung, bladder, kidney, uterus, fallopian tube, peritoneal cavity, and ovary.Hepatoid carcinoma of the ovary (HCO) is an extremely rare histologic subtype of ovarian cancer. Only a few cases of HCO have been reported. Nevertheless, the histogenesis of HCO has been controversial. Recurrence of HCO is common, and it often has a limited response to chemotherapy. Therefore, it is necessary to establish diagnostic and prognostic markers for HCO and an optimal treatment regimen based on histological and molecular biological studies of HCO cases.Treatment for HCO and epithelial ovarian cancer is similar and includes cytoreductive operation and chemotherapy (carboplatin and paclitaxel-based therapy).Herein, we report a case of HCO in a 65-year-old woman. The HCO was diagnosed relatively early, and adjuvant chemotherapy was performed after tumor removal. However, recurrence in the lumbar spine resulted in the removal of the mass, postoperative radiation therapy, palliative chemotherapy, and finally, death due to intraabdominal metastasis. We describe the 31-month-long treatment of the patient.Characteristics, treatment, and prognosis of this disease are described here along with a review of the literature.2A 65-year-old female patient was admitted to our hospital with complaints of indigestion, abdominal bloating, and pain that started a month earlier. She was diagnosed with hypertension a decade ago and was taking calcium channel blocker. She had undergone hysterectomy for a myoma 15 years ago. There was no other history . Noted signs of vitality were normal at the time of admission. On physical examination, she had abdominal distension without tenderness and rebound tenderness and no hepatosplenomegaly. Laboratory analysis showed normal liver and renal function including complete blood count. There was no abnormality in hepatitis test including hepatitis B. Serum levels of alpha-fetoprotein (AFP) were increased to 83,164.6 ng/mL . Moreover, the serum cancer antigen (CA)-125 level was increased to 340.8 U/mL . However, levels of carcinoembryonic antigen, CA-19–9, and human chorionic gonadotropin were within the normal range. There were no abnormal findings on the upper gastrointestinal endoscopy and colonoscopy.Based on pelvic and abdominal ultrasonograms, a large number of ascites in both paracolic gutters and a 13-cm mass of inhomogeneous echo were identified. Computed tomography (CT) of the abdomen showed a 12 × 8 cm sized mass composed of solid and cystic tissues . After the third cycle of chemotherapy, serum levels of AFP and CA-125 normalized to 5.9 ng/mL and 7.1 U/mL, respectively. There was no recurrence or metastasis detected in imaging studies performed after the third and sixth cycle. However, 6 months after the completion of chemotherapy, the patient was suspected of having recurrence based on elevated serum AFP level of 8872.4 ng/mL. 18Fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography (18F-FDG PET-CT) was performed, and FDG-uptake mass was observed in the lumbar spine (L2). Magnetic resonance imaging (MRI) of the whole spine was performed for accurate diagnosis that revealed a fracture with a mass protruding from the lumbar spine (L2) on the left psoas muscle. She underwent an operation for mass removal and posterior stabilization with Perfix system. A complication during the operation was massive bleeding from the mass. Biopsy and histological examination of the lesion site confirmed the diagnosis of metastatic hepatoid carcinoma. After surgery, AFP decreased to 2317.6 ng/mL. The patient refused further treatment due to weakness and pancytopenia. After 3 months, AFP again increased to 5526.9 ng/mL. The patient underwent external beam radiation therapy to the lumbar spine (L2) area. After radiation, AFP decreased to 1776.0 ng/mL. However, after 2 months, AFP again increased to 4726.0 ng/mL. CT of the abdomen revealed multiple enhancing nodules in the pelvic dependent portion. The patient received palliative chemotherapy with carboplatin  and gemcitabine (1000 mg/m2). After two cycles, abdominal CT revealed multiple, newly developed, intraabdominal metastatic nodules with a serum AFP level of 12,254.9 ng/mL which was rapidly increasing. She refused further chemotherapy due to a sudden decrease in performance. The patient continued to undergo symptomatic treatment such as ascites and pain control. She died 31 months after diagnosis due to disease progression.After surgery, adjuvant chemotherapy was administered with carboplatin  and paclitaxel  of cancer incidence, followed by those in their 40s 21.7%) and 60s (17.4%)..7% and 6In all case studies including this case, the size of the ovarian mass was >10 cm in 66.6% (10) of cases. It was found to be unilateral or bilateral. Abdominal pain was the most common symptom of patients at the time of diagnosis. In addition, there are cases of nonspecific symptoms due to gastrointestinal disturbances such as abdominal distension, abdominal mass palpitations, and anorexia. Although it is known to occur in older women aged 34 to 78 years old, there are cases in which women in the reproductive age were affected. At the time of diagnosis, serum levels of AFP and CA-125 were elevated in most cases (10/13) except for three cases where AFP and CA-125 were in the normal range. The stages in which HCO was identified were stage I, 15% (2 cases); stage II, 7.6% (1 case); stage III, 69% (9 cases); and stage IV, 7.6% (1 case). The majority (76.6%) of patients were diagnosed at an advanced stage (stages III and IV). It is commonly accepted that ovarian cancer has an epithelial origin due to its high incidence in postmenopausal women and a typical immunochemical staining pattern. However, an alternative theory of germ cell origin has been suggested. Thus, the histologic origin of ovarian cancer remains controversial. In addition, hepatoid adenocarcinoma has been reported along with carcinomas of other subtypes , although it develops independently in most cases.,13,21,22HCO is a carcinoma that is characterized by a high serum level of AFP with hepatocellular differentiation and is positive for cytokeratin on immunochemical staining. Cellular immunohistochemistry shows that HCOs stain positive for AFP, albumin, α-1 antitrypsin, and α-1 antichymotrypsin. To confirm HCO, it is necessary to differentiate it from other subtypes of AFP-producing tumors such as HCC, hepatoid yolk sac tumors (HYSTs), Sertoli-Leydig cell tumors, and dysgerminomas. Patients with HCC experiencing metastases to the ovary are rarely reported. Thus, it is necessary to exclude hepatic lesions clinically and radiologically and confirm bile component characteristics of HCC pathologically.Histologically, HCO is similar to HCC. It has a clear boundary with cuboidal tumor cells arranged in trabeculae or sheets. Non-metaplastic cell division is commonly observed. Furthermore, it contains a hyaline globule inside or outside the cell with an abundant eosinophil cytoplasm.HYSTs are germ cell tumors that commonly develop in the young age group (average age of 22 years). It is difficult to differentiate HYST from HCO as the size of the tumor and serum level of AFP rapidly increases with tumor progression in both cases. Nevertheless, testing paraffin-1 negativity for germ cell component and immunohistochemical studies are useful for differentiating the two.,12,14–17 Only three patients showed a good response without recurrence,8,16 but the others recurred several months later, two of relapsed patients showed a short response but worsened during treatment.,15The optimal approach for treatment of HCO remains unknown. In almost all cases, the patients received treatment similar to epithelial ovarian cancer, including maximal cytoreductive surgery, which would eliminate macroscopic disease such as hysterectomy, bilateral salpingo-oophorectomy, with or without lymph node dissection, and/or omentectomy. Most patients including our case (9 cases) have been treated with adjuvant chemotherapy regimens such as carboplatin and paclitaxel for ovarian cancer.,14,19 did not show intraperitoneal recurrence. This suggests that intraperitoneal chemotherapy may be as effective as ovarian cancer with peritoneal dissemination.Three patients who underwent intraperitoneal chemotherapy with or without adjuvant chemotherapyPlatinum and gemcitabine combination therapy, doxorubicin, and docetaxel were used as a secondary chemotherapy, but it did not show any good effect.Recently, there have been attempts to treat HCO with sorafenib, a liver cancer targeted drug, but it was stopped after 2 months due to HCO progression. Only one patient showed bone metastasis in the lumbar spine (L2) as in our case and was reported as alive after complete remission through radiation therapy without surgery. Of the two patients who received radiation therapy for bone metastasis, our case showed a decrease in AFP after radiation treatment, which could help predict the effectiveness of the treatment.The most common metastatic sites of HCO are peritoneum, intraperitoneal lymph node intraabdominal visceras such as liver, spleen, and distant hematogenous metastasis is less common.,15,17,18 However, some reports showed that patients who have been diagnosed in stage 3 or 4 survived more than 2 years through surgery and chemotherapy.,14,19 Pandey et al suggests that despite histopathological similarity between HCO and HCC, HCO should be treated similarly to epithelial ovarian cancer rather than HCC because HCO is biologically similar to ovarian cancer unlike HCC.The reported data suggest that the prognosis of HCO is very poor, and 5 out of 9 patients died within 3 years.In this case, the diagnosis of HCO was confirmed using histopathologic and immunohistochemical staining. Abdominal ultrasound, abdominal CT, upper gastrointestinal endoscopy, colonoscopy, and finally, surgery were used to exclude other organ origins.When compared to cases reported thus far, the patient was diagnosed with HCO at an early stage, FIGO stage IC and maintained a remission state for 6 months after the end of chemotherapy. However, the serum level of AFP increased, and bone metastasis was confirmed. Serum AFP level rapidly increased after multiple intraabdominal metastases. Thus, the serum level of AFP is useful as a prognostic marker for recurrence or metastasis evaluation Fig. . AlthougIn conclusion, HCO is a malignant ovarian tumor with noted hepatoid features originating from the ovary. It has noted nonspecific symptoms and signs. Recommended treatments for HCO and ovarian cancer are similar and include debulking operation and chemotherapy. Despite rare incidence, HCO recurs commonly. In addition, it is often resistant to surgery and chemotherapy. HCO was evaluated in this study with limited data based on case report review. There is no established consensus on its histological or immunochemical staining patterns for diagnosis or treatment directions. Therefore, further case reviews and associated histological and molecular biologic studies are needed to establish optimal diagnosis and treatment for HCO.4HCO is a rare, malignant tumor of the ovary. HCO is generally associated with symptoms such as abdominal pain when it is over 10 cm in size, and it is most often diagnosed in the advanced stages. It is likely that serum AFP level may be useful as a prognostic marker for disease progression and recurrence. Treatment of ovarian cancer is limited and includes surgery and adjuvant platinum-based chemotherapy, and new therapeutic approaches such as target-based therapy and immunotherapy are needed.None of the authors had a conflict of interest with respect to these data reported. This paper was supported by funds from the Research Institute of Clinical Medicine of Jeonbuk National University-Biomedical Research Institute, Jeonbuk National University Hospital.Conceptualization: Won-Ku Choi, Chang-Yeol Yim, Na-Ri Lee.Data curation: Won-Ku Choi.Formal analysis: Won-Ku Choi, Chang-Yeol Yim.Investigation: Dong-Hyu Cho.Resources: Dong-Hyu Cho.Software: Chang-Yeol Yim.Supervision: Dong-Hyu Cho, Na-Ri Lee.Visualization: Dong-Hyu Cho.Writing – original draft: Won-Ku Choi.Writing – review & editing: Na-Ri Lee.


Rhabdomyosarcoma (RMS) arises from mesenchymal cells committed to skeletal muscle. It is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors. In this population, there are two main histological forms, which are the embryonal or the alveolar RMS. The retro auricular location is extremely rare. We present 2 cases of children with left retro-auricular RMS occurring at a very early stage of post natal development.Two children were included in the RMS 2005 Protocol. The first child, aged 5-days, was managed by surgical resection in two steps after a previous incomplete resection which was followed by 8 chemotherapy cycle. The second, aged 3-days, was managed by surgical resection of the tumor bed, completed by 8 chemotherapy cycle. On regular follow up for over 6 years after the diagnosis, both patients are free of recurrence.RMS is a fast-growing malignant and aggressive tumor originating from skeletal muscle. It occurs in the first decade of life and is associated with genetic conditions. With an unusual clinical presentation and anatomical location, both benign and malignant tumors can be suspected. Diagnosis can be performed with CT-scan or MRI and confirmed by biopsy. The treatment is based on chemotherapy followed by radiotherapy or surgical resection. In our two patients, the treatment program achieved complete disease control, with good prognosis especially because of anatomical location as well as early diagnosis. It is the most frequent soft-tissue sarcoma in children and represents 5% of all pediatric malignant tumors . RMS ariWhile the anatomical location of the primary tumor is variable, approximately 30% of all pediatric RMSs occur in the head and neck. Outer ear locations are considered extremely rare, thus making the early diagnosis challenging. Sometimes, inflammatory and infectious presentation can delay diagnosis and lead to unsuitable primary management ,3.Treatment of non-metastatic RMS follows the EpSSG RMS 2005 protocol , with a 2A retrospective analysis was performed on files of 2 patients followed for retro auricular RMS in our department over a 6-year period from August 2011 to June 2019. Consent was obtained for both patients. The purpose of this retrospective analysis was to describe the diagnosis, management and survival rate of both children with retro auricular embryonal RMS. It emphasizes both clinical and radiological findings of this rare condition.The extent of disease was determined by clinical examination, supplemented by radiological imaging including CT, PET-CT and MRI.The tumor, nodes and metastasis classification of pediatric soft-tissue sarcomas (IRS) of the Union for International Cancer Control (UICC) allowed the cancer staging. Survival rate was determined from the end of treatment and for both children.2.1A 3-month-old girl presented with a left retro auricular mass that increased in size in a period of 3 months. This mass was discovered at post natal day 5 and was watchfully followed by the GP. There was no relevant family history. Examination revealed a slowly enlarging left retro auricular swelling without inflammatory or general sign. There was no history of trauma nor infection.A temporal MRI was performed and discovered an oval mass (3 cm long axis) showing moderately intense and heterogeneous enhancement of the mass in post contrast T1 fat-saturated sequence . A lymphA biopsy performed under general anesthesia was realized without anatomopatholigal result. Surgical resection was then performed. Macroscopic examination found a 35 × 27 × 16 mm well-delineated non-cystic lesion. Fetal rhabdomyoma was first diagnosed. On immunohistochemistry, about 30% of cells were myogenin positive but more heterogeneous cells were desmin positive. In contrast to rhabdomyome, the degree of orderly zonation was not achieved. There was mild but undoubted nuclear atypia and quite frequent mitotic figures. In this case, an embryonal RMS was finally diagnosed 3 months after surgical resection .Fig. 2PaBrain and temporal MRI, head and neck and thoracic contrast-enhanced CT-scan, PET-CT and myelogram were performed. Brain MRI showed a 6 mm left retro-auricular nodular lesion but cerebral parenchyma was normal. There wasn’t any cervical lymph node or metastatic extension on chest CT-scan or PET-CT. A complete resection was performed. The lesion was classified IRS I according to the classification of UICC.The multidisciplinary team recommended implementing the EpSSG RMS 2005 lower risk group, subgroup A protocol . The patRegular follow up was established with clinical examination every 6 weeks and temporal and brain MRI every 3 months during the first year.Then, during the 2nd and the 3rd years, the same follow up was performed every 4 months. During the 4th and the 5th years an annual clinical examination with brain and temporal MRI was performed. Beyond the 5th year, clinical examination was performed annually without MRI. After regular follow up for over 6 years after the diagnosis, the patient is free of recurrence.2.2An 18-month-old girl presented with a left retro auricular cyst, noticed during her first days of life and followed by the GP.An ultrasound was initially performed and found a non-throbbing and non-adherent vascularized hypo echoic mass compatible with hemangioma. A CT-scan was realized to eliminate bone damage or vascular malformation. CT-scan showed a 41 × 36 mm left highly vascularized retro auricular tumor regardless of malignancy .Fig. 3PaSurgical resection was performed by retro auricular approach. Under general anesthesia, with patient in supine position, head turned to the right, a rhombic retro auricular incision was performed. Wide local excision of this vascularized tumor developed above the periosteal plan was successfully performed. Histopathological examination revealed embryonal RMS .Fig. 4PaBrain and temporal CT-scan found a subcutaneous left retro auricular enhanced-contrast tumor extended to occipital region.A PET-CT was performed and found a bilateral latero-cervical hyper metabolism in IIA area according to the Robbins’ classification . MRI fouRevision surgery was performed with left cervical node dissection. No metastatic lymph node was diagnosed.The tumor was classified IRS I according to the classification of UICC.The multidisciplinary team recommended implementing the EpSSG RMS 2005 lower risk group, subgroup A protocol . The patA regular follow up was established according to the protocol of patient #1. We noticed a good tolerance to chemotherapy. After 60 months of follow up the patient is free of recurrence.3RMS is a fast-growing malignant and aggressive tumor originating from skeletal muscle. RMS occurs in the first decades of life and is associated with genetic conditions like neurofibromatosis or Li Fraumeni syndrome . It is tPresenting symptoms varied patients and to the site of the disease. Orofacial RMS can be characterized by a painless cutaneous node or retro auricular swelling without inflammatory sign, as our cases. Because of non-specific presenting symptoms, they are frequently misdiagnosed and treatment can be delayed by several months.With an atypical aspect, clinical presentation and unusual location, benign tumors, such as glomus tumor, hemangioma, pilomatrixoma, or malignant tumors, such as liposarcoma, chondrosarcoma, neuroepithelioma, extra skeletal Ewing’s sarcoma, juvenile fibromatosis or malignant lymphoma can be suspected ,7. RMS iDiagnosis can be suspected with CT-scan or MRI and confirmed by biopsy . CT-scanOn CT-scan, RMS is a bulky tumor with multiple degrees of heterogeneous attenuation. On MRI, RMS is seen like a nonspecific low or isointense signal on T1-weighted sequences and high signal on T2-weighted images. In our case, differential diagnosis based on imaging were suspected because of characteristics comparable to a variety of other lesions (lymphangioma and hemangioma). Extension assessment can be completed by PET-CT, bone scintigraphy, chest CT-scan and osteomedullary biopsy to screen for second locations and evaluate metastatic extension. Metastasis of RMS is by hematogeneous, direct and lymphatic routes .Histopathological examination only permits definitive diagnosis. RMS can be classified as embryonal, alveolar or pleomorphic type. The embryonal type is the commonest lesion (60%) whereas the alveolar type has the worst prognosis. Embryonal RMS is characterized by small round blue cells, like neuroblastoma or Ewing’s sarcoma, with primitive spindle cells and a myxoid background.In case #1, 30% of cells were myogenin and heterogeneous desmin positive with nuclear atypia and frequent mitotic figures. Thus an embryonal RMS was diagnosed.Immunocytochemistry, including staining for desmin, smooth muscle actin and myogenin confirmed the diagnosis . There aThe intensity and duration of treatment for RMS is tailored on prognosis factors used to define risk groups. In Europe, a stratification system takes into account histologic type , initial post- surgical status , location (favorable or not), lymph node invasion, patient age (less or greater than 10 years) and tumor size (less or greater than 5 cm). The European paediatric Soft Tissue Sarcoma Study Group (EpSSG) defined four groups: low risk, standard risk, high risk and very high risk . This clMultidisciplinary approach in the management of RMS with chemotherapy, surgery and/or radiotherapy has improved the prognosis with a five-year survival rate of 74–77% . EuropeaThe International Society of paediatric Oncology published data describing significant improvement in terms of remission rates in case of non-metastatic RMS in paediatric patients . Side ef4Retro auricular RMS is rare. Early diagnosis is a challenge. Clinicians should be aware because of its ubiquitous distribution or diverse morphology. Biopsy should be performed rapidly in case of the slightest doubt.Histopathological examination with immunohistochemical studies are required to achieve diagnosis. Imaging provides an assessment about the extension and has a key role in the initial staging and moreover during the follow up.Treatment follows a rigorous international protocol associating surgery, chemotherapy and sometimes radiation therapy. Prognosis is good, as the site is accessible to a complete resection.All patients require long term follow-up by a multidisciplinary team to exclude recurrence and to recognize and treat post-treatment complications.No conflict of interest.No financial and personal relationship.No source of funding.No ethical approval because no research studies.Written informed consent was obtained from both of the patient's parents for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.M. ROUBAUD JC write the case report.It’s a case report.Pr MARIANOWSKI Rémi.Not commissioned, externally peer-reviewed.


Medulloepithelioma is a rare childhood embryonal tumor arising from the non-pigmented ciliary epithelium of the pars plicata. We report a case of an 11-year-old male who presented with painless loss of vision of the right eye for the last three years and progressively increasing mass on the superior aspect of the globe for the last three months. On ocular examination, a firm, non-tender mass of 4cm × 3cm was noted in the superior aspect of the globe. CT-Scan of the orbit was suggestive of a foreign body with a haemorrhage or infection. The patient underwent enucleation with minimal manipulation. Histopathological examination of the enucleated globe revealed medulloepithelioma.The intraocular medulloepithelioma presentation is often late and masquerading, which may lead to extraocular extension and metastasis and ultimately unfavorable prognosis. Extraocular extension in medulloepithelioma occurs in about 7-18% of cases, which has an unfavorable prognosis.8Though rare, medulloepitheliomas are the second most common type of pediatric primary intraocular tumors.Here, we would like to report a case of intraocular medulloepithelioma presented to us as a subconjunctival mass.An 11-year-old male presented with a painless mass over the superior aspect of the globe for the last three months which was progressively increasing in size. The child had progressive, painless loss of vision in the same eye for the last three years. On ocular examination, visual acuity was no perception of light in the same eye and 6/6 in the other. Firm, non-tender mass measuring 4cm x 3cm was noted in the superior aspect of the globe . It was After consent, enucleation was performed with minimal manipulation under general anesthesia with the orbital implant and conformer. The specimen was sent for histopathological examination.Gross examination of a cross-section of the enucleated globe revealed a solid grey lesion with exophytic and endophytic components . Histopathological examination revealed thick bands of polarized epithelium resembling medullary epithelium along with thinner cellular strands of acellular myxoid stroma . The tumor cells aggregated radially along the central space forming Flexner Wintersteiner rosette . It also involved the anterior chamber and vitreous cavity, but the optic nerve stump was free of tumor. This was highly suggestive of medulloepithelioma grade II. The origin of medulloepithelioma could not be confirmed.The child is under regular follow-up. He is healthy, and there is no recurrence seen at one year of follow-up.Medulloepithelioma is a rare intraocular tumor presenting in children. It has varying and delayed presentation making it a challenge to diagnose and manage the tumor properly.8 Kaliki et al., in 2012, in the analysis of 41 cases of ciliary body medulloepithelioma, 7% had an extra scleral extension on presentation. Two cases underwent enucleation, and one underwent exenteration. All three cases later had metastasis. The average duration of metastasis to diagnosis was nine months.7Brought and Zimmerman in 1978 studied 56 patients, of which 10 (18%) had invasion through the cornea or sclera or optic nerve. All 10 cases underwent enucleation, of which one case underwent additional orbital radiation and systemic chemotherapy and another patient underwent exenteration. Four cases lost their lives, and 3 cases lost to follow-up. Rest three were doing well two and nine years after therapy.9A tumor that is confined to the globe has an excellent prognosis, with a 5-years survival rate of 90-95% after enucleation. But an extension of tumor to extra scleral tissue dramatically increases metastasis and disease recurrence with an overall poor prognosis.9Though there has been a lack of standard treatment of extraocular medulloepithelioma in literature because of the rarity of the disease, chemotherapy has been used with variable prognosis.Our patient underwent enucleation with minimal manipulation technique without touching the tumor. Despite the extra scleral extension, the child has not shown any signs of recurrence or metastasis over one year.To conclude, subconjunctival mass as a presentation of intraocular medulloepithelioma is rare, and diagnosis is often delayed, making it a challenge to manage appropriately. When confined to the globe, it has a favorable prognosis. Unfortunately, delay in diagnosis may lead to extra scleral extension and an unfavorable prognosis.


The occurrence of sporadic colonic neurofibroma particularly in a patient without neurofibromatosis type 1 has been rarely reported. Therefore, the clinical significance of this disease has not been fully elucidated.An 81-year-old woman with a positive fecal occult blood test result was referred to our institution for the evaluation of anemia. On colonoscopy, a 50-mm submucosal tumor-like mass was found in the hepatic flexure of the colon. Superficial biopsy and boring biopsy showed unspecific granulation tissues, and immunostaining revealed that the mesenchymal tumor was negative for CD34, c-kit, desmin, and S100 protein. The patient underwent laparoscopic right colectomy with complete mesocolic excision (CME). Pathologically, the tumor was diagnosed as neurofibroma.Gastrointestinal neurofibromas are known to cause clinical symptoms. No colonic neurofibroma has been diagnosed before resection. Moreover, neurofibromas, particularly large lesions, reportedly undergo malignant transformation. Surgical extirpation with clear margins is the primary treatment, and laparoscopic surgery is considered acceptable for colonic neurofibroma and colon cancer.Based on our experience, a preoperative diagnosis was impossible for colonic neurofibroma. Laparoscopic surgery with CME is considered feasible for sporadic colonic neurofibroma. PreviouNF1 . Owing tHere we report a rare case of sporadic neurofibroma of the transverse colon in a patient without NF1. With this report, our experience can contribute to a better understanding of colonic neurofibromas. This work has been reported in line with the SCARE criteria .2An 81-year-old woman with positive fecal occult blood test result was referred to our hospital for the evaluation of anemia. The patient had a medical history of hyperlipidemia but no familial medical history. Laboratory examination revealed iron deficiency anemia . Moreover, her liver function, renal function, and tumor marker levels were within normal limit. On colonoscopy, a 50-mm submucosal tumor-like mass was found in the hepatic flexure of the colon . SuperfiAssessment of the surgical specimen revealed a well-circumscribed submucosal tumor-like mass. Pathologically, the tumor originated from the muscularis propria layer a and spiThe postoperative course was uneventful, and the patient was discharged 10 days after surgery. During the outpatient visit, her hemoglobin level was within normal limits. No evidence of recurrence and physical symptoms indicative of neurofibromatosis was observed 3 months after surgery. Thus, the patient will undergo an annual outpatient follow up.3Neurofibroma is a benign nerve sheath tumor of the peripheral nervous system, which comprises a mixture of spindle cells with wavy nuclei and collagen strands as well as Schwann cells, perineural-like cells, and fibroblasts ,3. NeuroAlthough neurofibromas are typically benign lesions, they reportedly undergo malignant transformation and develop into malignant peripheral nerve sheath tumors (MPNSTs), particularly when associated with large or plexiform lesion ,7. The tGastrointestinal neurofibromas are known to cause clinical symptoms, including bleeding, palpable mass, obstruction, and intussusception. In this case, iron deficiency anemia was caused by microhemorrhage. We believed that curative resection is recommended for the treatment of symptomatic neurofibroma. In contrast, there is no consensus on the exact surgical indications for tumor size owing to the rarity of neurofibromas.In this case, a high infiltration of lymphocytes and plasma cells into the tumor was observed, which might have caused lymph node enlargement on preoperative CT. However, no studies have reported regarding regional lymphadenopathy on preoperative CT. Therefore, regional lymphadenopathy is not a characteristic feature of neurofibromas. The diagnosis of neurofibroma may not be obtained via preoperative CT.Endoscopy is typically used to identify single or multiple polypoid lesions or submucosal masses of variable sizes and distributions . HoweverFor neurofibroma, complete surgical extirpation with clear margins is the primary treatment ,7. In thIn Japan, one case of colonic neurofibroma resected via laparoscopic surgery was reported . HoweverNF-1 is an autosomal hereditary syndrome characterized by several signs and symptoms , and theThe primary limitation of the present study was the inclusion of only a single case. Therefore, large-scale studies involving patients with colonic neurofibroma but without NF1 must be conducted.4We performed laparoscopic resection with CME for colonic neurofibroma in a patient without NF1. In many cases, a preoperative diagnosis of colonic neurofibroma is impossible. Moreover, neurofibromas should be distinguished from submucosal tumors when only granulation tissues are observed on superficial biopsy and boring biopsy. Laparoscopic surgery with CME for colonic neurofibromas is considered feasible.The authors have no conflict of interest to declare.This study did not receive any specific grant from funding agencies in the pubic, commercial, or not-for-profit sectors.This study was approved by the ethics committee.Written informed consent was obtained from patient and her family for publication of this case report.TI, SS and TN performed surgery. TI drafted the manuscript. MK participated in the correction of the work. MS, TT, YE and TK were responsible for perioperative management. KI was responsible for endoscopic examination. SI was responsible for pathological diagnosis.No research study involved in this case report. Not applicable.Toru Imagami.Editorially reviewed, not externally peer-reviewed


Extracranial meningioma is very rare with few cases reported, especially in the oral cavity. Its diagnosis is considered a challenge owing to the unusual site of occurrence.  We report, to the best of our knowledge, the first case of extra-cranial meningioma as a primary tumor in the hard palate with no detected intracranial extension. A 59-year-old Egyptian female patient presented with a 22-year history of a large painless swelling at the right side of the hard palate, which could not be seen on radiographs.  An incisional biopsy was taken and, after assessment with a panel of immunohistochemical markers, the lesion was diagnosed as extracranical grade I mengiothelial meningioma. The patient did not show up for surgical excision and follow-up was not performed because of the lose of contact with the patient. Intraoral meningioma is a rare tumor. Immuohistochemical markers are important for confirming this diagnosis. Meningioma may develop as a direct extension of a primary intra-cranial meningioma or as a true primary extra-cranial meningioma2.Meningioma is a benign neoplasm of meningothelial cells3. The most common extra-cranial site is the orbits. Meningioma arising in the oral cavity is extremely rare4. To the best of our knowledge, 19 cases have currently been reported in the oral cavity20 and we are reporting the first case in the hard palate.Extra-cranial (ectopic) tumors are mostly seen in the head and neck region with no connection intra-craniallyA 59-year-old female patient presented to the outpatient clinic in the Oral and Maxillofacial Surgery Department, Cairo University in January 2019 complaining of a large painless swelling in the hard palate . The patAn incisional biopsy of the lesion was performed. Hematoxylin and eosin stained sections revealed meningothelial cells arranged in lobules. The cells exhibited round to oval nuclei . PsammomNo therapy was administered to the patient during her admission. Unfortunately, the patient did not show up for surgical excision and follow-up.4. The first intraoral meningioma reported was by Brownet al. in 1976, which presented as a periapical radiolucency in the anterior maxillary region5.Primary extra-cranial meningioma is an unusual tumor, especially in the oral cavity20; in the present case, the patient was 59 years old. Regarding the reported cases of intraoral primary meningioma, 6 of the 18 were in the maxilla16, 10 were in the mandible20, 2 in the buccal mucosa18 and one in soft palate8. To the best of our knowledge, we report the first case in the hard palate.To the best of our knowledge, 19 cases of primary meningioma in the oral cavity have been reported. Of these, 13 were in female patients, which is also true of the present case. However, the age range was wide in the reported cases – between 10 and 77 years old20 observed that the tumor cells stained positive for monoclonal antibodies against EMA and vimentin, with no immunoreactivity for S-100 protein, which was similar to our findings. However, EMA and vimentin are not useful to differentiate between meningioma and perineuroma as they both express positivity for EMA and vimentin but perineuroma the cells are spindle and elongated however, in our case they are rounded and polyhederal .The histopathological criteria of extracranial meningiomas are similar to those of their intracranial counterparts. All documented cases shared the same characteristics: whorls of spindle cells or epithelioid cell proliferation and psammoma bodies. In our case, diagnosis was challenging because of the tumor’s similarity with other tumor entities of peripheral nerve origin, as well as the uncommon location of the tumor. An immunohistochemical panel of tumor-associated markers was used to confirm the diagnosis and to avoid unnecessary aggressive treatment. Most of the 19 cases reported in the literature were diagnosis using immunohistochemical markers. All reported cases that used immunohistochemistry techniques to diagnose meningiomaet al.20, preferred only to follow-up with the patient rather than conduct surgical intervention. However, others preferred to perform aggressive treatment, such as as segmental mandibulectomy or segmental resection12Unfortunately, our patient did not show up for surgical excision and follow-up was not done because of the loss of contact with the patient. However, most of the documented cases were treated successfully without recurrence by surgical excision. Some of the studies, such as that by RommelIn conclusion, meningioma is a rare intraoral benign neoplasm. Immunohistochemical markers are an important tool to achieve a final diagnosis, especially for the differentiation from histological mimic entities of peripheral nerve origin, such as perineurioma and neurothekeoma and to avoid unnecessary aggressive treatment. Vimentin and EMA are the two important markers to confirm extra-cranial meningioma diagnosis.All data underlying the results are available as part of the article and no additional source data are required.Written informed consent for publication of their clinical details and clinical images was obtained from the patient. Ectopic Meningiomas are seen less frequently in oral regions, so it can be considered as a case report. However, the lack of follow-up of the case reduces the power of the article.Clinical data and radiographic findings can be given in more detail.Differential diagnoses should be discussed in the discussion section.Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?PartlyIs the case presented with sufficient detail to be useful for other practitioners?YesIs sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?PartlyIs the background of the case’s history and progression described in sufficient detail?YesReviewer Expertise:maxillofacial surgeryI confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. The requested changes were made.Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?PartlyIs the case presented with sufficient detail to be useful for other practitioners?PartlyIs sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?PartlyIs the background of the case’s history and progression described in sufficient detail?YesReviewer Expertise:NeuropathologyI confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. Case Report: Case Report: Rare site for intraoral meningiomaIs the background of the case’s history and progression described in sufficient detail? The patient’s background can be improved if relevant findings are reported such as previous exposure to radiation as a potential risk for development of meningioma.Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes? This is one of the unfortunate incidents where patient is not committed to treatment.Although it seems that the patient is asymptomatic except for the size of the palatal swelling, absence of radiograph (even if non-indicative) and not mention neurofibromatosis-type2, undermine the reader’s understanding of the case.The clinical picture is good and clear. Is sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?1,2Discussion is fine although it would’ve been beneficial to cite the other reported palatal lesions by Kishore A (2000) and Sinha (2002), for example.The immunohistochemistry test panel discussion would be more useful for the non-pathology reader if there were brief comment on the value of such tests of uncommon oral lesions with perplexing presentations.Consequently, a short list of lesions to be included in a general dentist’s record when similar cases are detected, would be useful.Few editing or English language corrections would help. For example “follow-up was not done because of loose of contact” in which “loss not loose” is meant. The statement is repeated in abstract and discussion and significantly alters the meaning of the sentence.Is the case presented with sufficient detail to be useful for other practitioners? Extracranial meningioma are rare and intraoral lesions are even rarer. It is of high pathological interest of both specialist and general practitioner to get updated on occurrence, presentation and diagnostic methods of such tumors. The case and the tumor presented are useful, relevant and with corrections, the report would be more impactful.Expect the unexpected when similar lesions are encountered.Suggest an effective strategy to ensure patient’s return when the outcomes are suspicious.Conclusions must be more powerful and instructive for the practitioner to: The case report presents an extracranial meningioma of the hard palate. The patient’s gender is conformed to the tumor but there are (likely) no contributing or risk factors associated with its occurrence. The lesion is asymptomatic and incisional biopsy was examined routinely and certain microscopic features were suggestive of meningioma. The specimen was further stained with a panel of markers and was diagnosed as meningioma of the palate. Unfortunately the patient did not return for surgical removal. Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?PartlyIs the case presented with sufficient detail to be useful for other practitioners?PartlyIs sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?PartlyIs the background of the case’s history and progression described in sufficient detail?PartlyReviewer Expertise:Oral Pathology, Oral medicine, medical and Dental EducationI confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above. Firstly, thank you doctor for your valuable comments. We added that data and answers that you commented to the new version of the manuscript. And I will answer them too here as a respond to your report.   1- There was no previous exposure to radiation as a potential risk for development of meningioma.This is one of the unfortunate incidents where patient is not committed to treatment. 2- CT scan was done and there was no bone involvement and  neurofibromatosis-type 2 was excluded because the lesion is single and been present in her oral cavity for 22 year. 3- Discussion is fine although it would’ve been beneficial to cite the other reported palatal lesions. 4- The immunohistochemistry test panel discussion was added  5- Benign salivary gland neoplasm add to the  list of lesions to be included in a general dentist’s record when similar cases are detected. 6-  English language corrections were done. 7- Conclusions  were also corrected to include the consequence of wrong diagnosed. Thanks again for your comments which add a lot to our knowledge and for your time too Radiological findings should be included. The authors merely mention that the lesion was not seen by radiology. Please clarify the technique used and relevant findings.The grade of meningioma was not givenThe differential diagnosis of meningioma from mimics was not sufficiently addressed. Different entities in the differential diagnosis were mentioned at different parts of the article without discussing the differentiating points. And the performed stains would not help in differentiating meningioma from perineurioma, both are positive for EMA and vimentin.here. Grammatical and spelling mistakes are notedThe case report is well written and presents an interesting case of ectopic meningioma. Some amendment is needed however.Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?PartlyIs the case presented with sufficient detail to be useful for other practitioners?PartlyIs sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?PartlyIs the background of the case’s history and progression described in sufficient detail?YesReviewer Expertise:Neuropathology.I confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above. Thank you Dr. Eman for your valuable comments and for your time. We added that data and answers that you commented to the new version of the manuscript. And I will answer them  here too as a respond to your report.  1-  All the English language corrections were done.2- CT scan was done and there was no bone involvement.3- Meningioma grade I.4- The list of differential diagnosis was done and discussed.


Mature Cystic Teratoma (MCT) is a benign tumor that can lead to malignant transformation (MT) in 1–3% of cases. Management of MT is a big challenge for gynecologic oncologists due to the lack of specific diagnostic and treatment protocols.We reported two Iranian cases of MT of MCT with two different stages and prognosis. Our both cases presented the same symptoms, including chronic abdominal pain and distention, loss of appetite, and weight loss. In case number 1, despite the large size of the tumor, the disease was at stage Ia and had a good prognosis; while, case number 2 was at stage IIIc of the disease with a poor prognosis.The stage of the disease is the most important prognostic factor, and early diagnosis and treatment are very critical for better survival. Mature cystic teratoma (MCT), a benign tumor commonly called the dermoid cyst, is the most common type of ovarian germ cell tumors. Over 80% of cases present in reproductive periods, especially in women under the age of 40. Malignant transformation (MT) is a rare and the worst complication of MCT that occurs in 1–3% of cases, especially postmenopausal women. Nearly 80% of the histological type of malignant transformations is squamous cell carcinoma (SCC), followed by adenocarcinoma, carcinoid tumor, melanoma, and sarcoma [A 51-year-old, gravida 2, para 2, Iranian woman, suspected to have an ovarian tumor, was referred to our hospital. She complained of pelvic pain, anorexia, and weight loss of 10 kg over the past 3–4 months. The patient’s past medical, familial, social, environmental, and medication history was insignificant. The patient reported no history of smoking , drug abuse, or drinking alcohol. In all clinical examinations, except abdominopelvic examination, there were no significant findings, and the vital signs  were normal. In abdominopelvic examination, a mass was palpated above the pelvis, from the midline to the lateral side, causing a mild compression effect against the rectum. All routine blood and urine laboratory tests were normal. The serum levels of cancer antigen 125 (CA125) and carcinoembryonic antigen (CEA) were above the normal ranges , while the amount of the other tumor markers, including human epididymis protein 4 (HE4), human chorionic gonadotropin (HCG), and alpha fetoprotein (AFP), were in the normal range . Abdominopelvic computed tomography (CT) scan showed a complex large solid-cystic (125 × 118 mm) mass with fat components, originated from the right adnexa, suggesting an ovarian dermoid cyst. This mass contained an oval-shaped, enhanced, solid component, sized 40 × 35 mm at its right lateral wall. Also, the CT scan detected an intrauterine device (IUD) in its normal location  5 intravenously, every 21 days).On gross examination, the surface of the right ovary was smooth and contained a mass with a size of 130 × 110 × 50 mm. On the cut section, a unilocular cyst filled with hair shaft and greasy brown material was observed. The cyst wall contained a solid tan mural nodule measuring 80 × 50 × 40 mm. Microscopic examination revealed stratified squamous epithelial lining of the cyst. Sections from the solid nodule showed moderately differentiated squamous cell carcinoma arising in MCT Fig. . The resWe followed the patient for 6 months after the completion of chemotherapy with thoracic, abdominopelvic, and spinal CT scans. Due to the detection of multiple diverticula of the sigmoid colon, the patient immediately underwent a colonoscopy that was fortunately negative for malignancy. Also, we performed a bone scan for the patient that was in favor of vertebral lytic lesions. So, we considered a positron emission tomography/CT scan (PET/CT) for her that it also found no evidence in favor of metastasis. The results of the thoracic and pelvic CT scans were reported normal, and the levels of the CA125 (= 8.3 u/ml) and other tumor markers were in their normal ranges. So, we closely followed the patient during the last 20 months, and fortunately, no recurrence has been occurred until now.A 53-year-old, gravid 4, para4, postmenopausal Iranian woman with a suspicious ovarian tumor referred to our hospital’s oncologic clinic. She complained of chronic abdominal pain and distention, loss of appetite, and weight loss of 7–8 kg during the last 8 months. She has a history of chronic hypertension. The patient’s past familial, social, environmental, and medication history was insignificant. The patient reported no history of smoking , drug abuse, or drinking alcohol. In all clinical examinations, except abdominopelvic examination, there were no significant findings, and the vital signs  were normal. On abdominopelvic examination, a mass was palpated that was extended from the pelvis to above the umbilicus. External genitalia and cervix had a normal appearance, and the Pap-smear test was negative for malignancy. The routine blood laboratory tests were within normal limits. Among the tumor markers, only the CA125 and ROMA were slightly elevated , and the rest were in the normal range. Ultrasound and magnetic resonance imaging scans showed a large solid-cystic intra-abdominal mass (sized 198 × 96 mm), with fat foci, originating from the right ovary, omental thickening in the vicinity of the tumor, and mild free fluid in the pelvis . Unfortunately, 9 months after the diagnosis of the SCC, the patient passed away due to multiorgan failure related to the widespread chemoresistant disease.Four weeks after the operation, the patient underwent adjuvant chemotherapy with paclitaxel 175 mg/mAt the present study, we reported two cases of ovarian SCC arising from MCT with two different stages of the disease and prognosis. It is well known that MT of MCT is a rare condition that occurs in less than 3% of MCT cases. Our both cases presented the same symptoms, including chronic abdominal pain and distention, loss of appetite, and weight loss. In case number 1, despite the large size of the tumor, the disease was at stage Ia and had a good prognosis; while, case number 2 was at stage IIIc of the disease with a poor prognosis, and progression of the disease occurred despite chemotherapy. As it has been mentioned in previous studies, large tumor size is one of the risk factors for aggressive behavior and poor prognosis; however, both of our cases with definitely large tumors had different stage of disease and prognosis. Also, the level of the of previously introduced tumor markers is unreliable, as in our case that had stage IIIc of disease, the serum levels of all markers were normal, and only the CA125 and ROMA were slightly elevated.Unfortunately, the lack of specific clinical signs, tumor markers, or imaging features makes the preoperative and operative diagnosis of the MT very difficult, or even impossible; however, some risk factors are suggested, including age, imaging features and size of the tumor, serum level of some tumor markers, staging of disease, and residual tumors , 3. In tStudies have shown that serum levels of some tumor markers, such as SCC, CA125, CEA, and CA19-9, might be higher than the normal range in this malignancy, but there is no relationship between the level of these tumor markers and tumor size or stage of the disease; however, it can be associated with worse prognosis . In our Previous studies have reported that imaging characteristics most suggestive of malignancy are the presence of solid components and involvement of adjacent organs . In our According to the literature, the stage of the disease is the most important prognostic factor for the overall survival rate , 6, 8. TOther risk factors that can influence the prognosis are the presence of ascites, capsule invasion, vascular invasion, rupture, and adhesion . In our et al., similarly to our study, has used the combination of carboplatin and paclitaxel regimen [Regarding retrospective design and limited data available in previous studies, there are still no agreements on the effectiveness of a specific treatment like adjuvant chemotherapy or radiotherapy in this disease. According to a recently published systematic review, it seems that early hysterectomy and platinum-based chemotherapy could be associated with better survival; however, this result should be interpreted with caution . The mos regimen . Our firWe should remind that the diagnosis and treatment of SCC arising from MCT is still a diagnostic and therapeutic puzzle, especially in women with advanced disease, since, despite extensive initial surgery and optimal debulking, the prognosis in these patients is poor. We need to gather patient information internationally and share our experiences about this disease to reach a standard treatment protocol. Therefore, relying on our experiences and previous reports, we must remind that postmenopausal women with large dermoid cysts are more likely at risk of developing malignancy.


We report a rare case of gastrointestinal stromal tumors (GISTs) in Carney’s triad, successfully treated using laparoscopic gastrectomy with lymph node dissection after chemotherapy.A 21-year-old woman presented to our hospital for treatment of recurrent GISTs. The patient had been admitted for treatment 11 years prior, with black stools being the chief presenting complaint at that time. On examination at that time, multiple submucosal tumors in the pyloric antrum and multiple pulmonary tumors had been observed. She underwent open partial gastrectomy, and the diagnosis of GISTs was confirmed. She was administered tyrosine kinase inhibitors to treat lung metastases from 2 months after surgery. Due to the increasing size of the lung tumors, a right upper lobectomy was performed 9 years after the index gastric surgery. Histopathological examination of the lung specimen, in combination with re-examination of the gastric specimens, was indicative of incomplete-type Carney’s triad. Eleven years after the index gastric surgery, multiple GISTs were observed in her entire stomach. Tumor biopsy revealed a succinate dehydrogenase deficiency, confirming the diagnosis of recurrent GISTs. For treatment, the patient underwent laparoscopic completion gastrectomy, with D1 plus lymph node dissection.This is a first case report of completion gastrectomy performed laparoscopically for the treatment of GISTs associated with incomplete-type Carney’s triad. The recurrent GISTs developed over a protracted period of 11 years from the index gastric surgery to tumor recurrence. In 1977, Carney et al. reported on the association between gastrointestinal stromal tumors (GISTs) and the presence of functioning extra-adrenal paraganglioma and pulmonary chondroma .Generally, the medical course of Carney’s triad is protracted, with multiple tumor recurrence , 4. One The patient was a 21-year-old woman, with an unremarkable family medical history. She had first undergone treatment for GISTs associated with an incomplete-type Carney’s triad 11 years prior, at the age of 10 years.At the time of her first admission and treatment, the patient had presented with black stools as her chief complaint. Endoscopic examination revealed the presence of two submucosal tumors in the pyloric antrum Fig.  9]. Mag. Mag9]. However, 2 years after her right upper lobectomy, at the age of 21 years, new gastric tumors were observed with ultrasound examination. Endoscopic examination of the upper gastrointestinal tract revealed the presence of multiple submucosal tumors on the residual side of the lesser curvature Fig. a, b. Bion = 8), with the largest being 52 × 30 × 25 mm in size (Fig. There was no evidence of extra-adrenal paraganglioma. A clinical diagnosis of recurrent GISTs, as a component of an incomplete-type Carney’s triad, was made. We decided to proceed with laparoscopic examination and treatment, as appropriate. On laparoscopic examination, further multiple nodules were observed on the serous surface of her stomach, with mild adhesion around the gastroduodenal anastomosis that could be easily divided Fig. a. Based ize Fig. . The tumThere was no complication after surgery, and the patient’s postoperative course was uneventful. She was discharged from the hospital in good condition and has been monitored carefully in follow-up, with no adjuvant chemotherapy treatment. Over the 7 months since the gastrectomy procedure, she has had no difficulty with oral intake, and there is no evidence of tumor recurrence.In our case, tumor recurrence occurred at a latency of 11 years after the index gastric surgery; this is a relatively longer latency period than is previously reported . RegardiCurrent guidelines for the treatment of GISTs in Japan do not recommend lymph node dissection . HoweverAlternative therapies have previously been reported for the treatment of metastatic Carney-type GISTs . HoweverIn summary, we report on the recurrence of GISTs in a case of incomplete-type Carney’s triad. The novel information presented herein is the successful performance of a completion gastrectomy and D1 plus lymph node dissection with laparoscopy. The postoperative course was uneventful.


Central Neurocytomas (CN) are a rare intracranial tumour, most often arising in the lateral ventricles and presenting with obstructive hydrocephalus. Isolated lesions in the third ventricle are uncommon. We present the fourth reported case of posterior third ventricular CN successfully managed surgically via endoscopy, allowing for concurrent biopsy and therapeutic endoscopic third ventriculostomy (ETV). Stereotactic radiosurgery was administered for the residual lesion. A brief review of CNs and previous similar cases is also provided.A 58-year-old male presented with progressive decline in cognition and gait. Subsequent Magnetic Resonance Imaging revealed obstructive hydrocephalus secondary to a posterior third ventricular lesion. An endoscopic biopsy and concurrent cerebrospinal fluid diversion by ETV was performed. Pathological analysis was consistent with a CN with positivity to Synaptophysin. MIB-1 proliferation index was 1%. There was good clinical recovery, and the patient underwent adjuvant stereotactic radiosurgery 1.5 months post-surgery.Due to the rarity of CNs arising from the third ventricle, there are only three previous reports of these approached endoscopically. Such a technique allows for good visualisation of the lesion, and therapeutic ETV to relieve obstructive hydrocephalus. This case supports this approach as a valid, minimally invasive option. Additionally, this is the first case to report the MIB-1 proliferation index, contributing to future outcome evaluation of endoscopic approaches to typical (MIB-1 < = 2%) verses atypical (MIB-1 > 2%) CNs.Endoscopic biopsy with concurrent ETV and adjuvant stereotactic radiosurgery is a valid treatment option for deep seated isolated small posterior third ventricular CNs. Lesions are typically located in the anterior half of the lateral ventricles, with attachment to the septum pellucidum . Due to 2A 58-year-old male presented to the emergency department with progressive decline in cognition and gait with recurrent falls over days, on a background of generalised lethargy for 3–4 months. There was a sight headache but no nausea. Past medical history was significant only for asthma, with no regular medications. Clinically the patient had a Glasgow Coma Score of 15, though there was a slowness of thinking below baseline. He had a wide based unsteady gait, but no other focal neurology.A Computed Tomography scan of the brain was significant for triventriculomegaly with no obvious lesion. Subsequent Magnetic Resonance Imaging (MRI) brain revealed a 7 mm round lesion in the posterior third ventricle obstructing the aqueduct. It was hyperintense on T2 and FLAIR, and hypointense on T1 with no contrast enhancement . The lesGiven the deep-seated location and the resultant obstructive hydrocephalus, an endoscopic approach was planned to allow for both an ETV and endoscopic biopsy. Under navigation guidance, a standard ETV was performed via a right frontal burr hole with balloon fenestration. A second, more anterior right burr hole was required to achieve the trajectory needed to reach the lesion. Intraoperatively, the lesion appeared as a pinkish, friable mass just anterior to the opening of the cerebral aqueduct. Biopsy samples were obtained using rongers. As the lesion extended periaqueductally towards the fourth ventricle, gross total resection (GTR) could not be attempted safely.Post operatively, the patient noticed a marked improvement in cognition and gait. A day one brain MRI with cerebrospinal fluid (CSF) flow studies confirmed a patent ETV, with a reduction in the degree of hydrocephalus. The lesion itself appeared similar.Histological analysis of the tumour showed mild increase in cellularity with rosetting, but no high-grade features on standard Haematoxylin and Eosin (H&E) stain. Immunohistochemistry was moderately positive for Synaptophysin  and posiA 1 month MRI brain and spine showed ongoing ETV patency with a further reduction in hydrocephalus. The lesion was unchanged, and there was no spinal metastasis. The patient underwent stereotactic radiosurgery with 29 Gy in 5# to the residual lesion 1.5 months after surgery. At 5 months post operation, MRI brain demonstrated a decrease in size of the lesion with new rim enhancement reflecting treatment effect. The ETV remained patent and there was resolution of hydrocephalus . The pat3CNs were first proposed as a distinct tumour type by Hassoun et al. in 1982 . ClinicaHistopathologically, CNs can resemble oligodendrogliomas or ependymomas under light microscope with standard stains, making diagnosis complex. Immunochistochemistry is generally required to guide diagnosis, though it is recognised that markers can have cross-positivity with other tumours and more research is required to develop diagnostic accuracy ,2. PositThe mainstay management of CNs involves GTR. An analysis of 438 patients by Rades and Schild  found thLesions of the posterior third ventricle are deep and challenging to reach surgically. Small lesions are amendable to an endoscopic approach which allows for minimal access surgery, good visualisation of the lesion, and ability to perform concurrent CSF diversion via an ETV. Due to the rarity of CNs, and its unlikeliness to arise from the third ventricle, there are only three previous reports where endoscopic technique was utilised for management of isolated third ventricular CNs  [[5], 6. Of noteJavedan et al.  were thePark et al.  describeIn the report by Romano et al.  a 37-yeaA side noteworthy paper to mention is by Gomes et al.  where twThis current case report represents the fourth reported successful usage of endoscopic approach for a small posterior third ventricular CN and CSF diversion by an ETV. Because the lesion extended down the left periaqueduct to the superior fourth ventricle, GTR was not achievable. The patient underwent adjuvant radiosurgery of the residual lesion as recommended in previous reviews of this rare tumour. Of the four reports, this is the only case to have a documented MIB-1 proliferation index, which will contribute to future evaluation of endoscopic approaches for typical versus atypical posterior third ventricular CNs. Consideration of use of a diode laser to aid endoscopic resection was felt as an important learning point from the review of previous cases, though this is operator dependent and requires technical training.None.This study is exempt from ethical approval in our institution.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Jolyn Khoo – Primary author.Gert Tollesson – Reviewer.N/A.Jolyn Khoo.Not commissioned, externally peer-reviewed.None.


Bulimia is an eating disorder with a great prevalence in young women. Due to its multifactor ethiology, bulimia has systemic consequences. In the literature, necrotising sialometaplasia is seldom associated with bulimia. Its etiopathogenesis is discussed by several authors; nevertheless, the consensus does not consider the relevance of local trauma associated with induced vomiting. A case of necrotising sialomethaplasia, presented with a single hard palatal ulcer in a bulimic woman is described in the present report. The patient did not present significant systemic laboratorial values, nor physical weight variations, which highlights the relevance of performing a complete medical clinical history when diagnosing this rare pathology. Necrotizing sialometaplasia (NS) is a rare pathology with a benign behavioral nature, which affects the salivary glands. It is generally more frequent in men in the fourth decade of life, with a reported average age of 49 years for men and 46 years for women (based on figures for men and women between 15 and 79 years of age) [The etiopathogenesis of NS is somewhat controversial: it appears neither to be an intrinsic pathology of the salivary gland tissue, nor the manifestation of a specific systemic condition. Despite the possibility of it occurring spontaneously, local irritative or traumatic factors such as smoking, alcohol abuse, drugs and prosthetic trauma seem to be directly involved in a significant number of cases ,3.Regarding its histological appearance, it is considered, in general, that systemic diseases with vascular compromising effects may also be associated with NS; diabetes, atherosclerosis, drepanocytosis, hypertension and Buerger’s disease all induce vascular alterations, ischemically compromising some organs or anatomical areas of the human body, which is why they are associated with NS lesions ,5. Some Previously, NS was associated with bulimia and anorexia ,9,10. WhA single lesion in the transition between the hard and soft palate is the most frequent presentation, although bilateral and even multiple lesions with random distribution on the hard palate may appear . The palPain is the most frequent symptom, although cases presenting local paresthesia, local anesthesia or cases that are completely asymptomatic are also described. Pain intensity does not appear to be related to the morphological severity of the lesion .Differential diagnosis should take into account the two clinical presentations of NS. The tumor form can be confused with periodontal abscesses or tumors of the minor salivary glands such as pleomorphic adenoma, cystic adenoid carcinoma and epidermoid mucus carcinoma, which are more frequent pathologies than NS. The ulcerated form may resemble malignant tumors in the same location , major oral ulcerations or rarer pathologies, such as syphilitic or tuberculosis ulcerations ,18.The name necrotizing sialometaplasia reflects the histological aspect of the lesion. The anatomical–pathological diagnosis follows five main criteria: ischemia or lobular necrosis, conservation of the nucleus morphology of epithelial cells, metaplasia involving both mucous canals and acini, presence of inflammatory infiltration and granulation tissue and, finally, conservation of the lobular aspect, even though inflammatory and metaplastic changes reach multiple glandular lobes . It shouAs a rule, NS has a limited self-evolution, not requiring aggressive therapeutic measures. Enhanced local hygiene measures should be used during the ulcerated phase of the disease . When paUlcerated lesions heal without leaving significant scars, although, in some cases, a small depression, in tandem with pre-existing ulceration, may be observed. The disease is not recurrent.As mentioned by the authors, the importance of a correct diagnosis of NS lies in the fact that it can be confused with a malignant tumor whose treatment, which is more aggressive, can leave significant sequelae.A 22-year-old female patient, followed by the Psychiatric Department for Bulimia, presented at a stomatology appointment for unpainful oral ulceration (15 mm × 7 mm) in the central palatine region that had lasted for 3 weeks. She reported that she was orphaned and adopted at 3 years old. Her physical aspect was compatible with her real age without alterations, and her mucosa appeared slightly pale. The patient stated that, in the initial phase, the lesion was a small non-painful ulceration that had increased in volume in the past two weeks. The patient denied experiencing systemic symptomatology in particular: fever, marked weight loss, joint pain, nor the ingestion of medicines or recognized contact with contagious infectious patients. The patient declared a one-year history of increased food intake followed by vomit provocation, with a loss of 10 kg during this period.During clinical examination, a unique crateriform ulcerated lesion, localized centrally on the hard palate , which was about 8 mm in diameter (anterior–posterior) was observed. The base of the lesion was filled with whitish necrotic remains, with no exposure of the underlying bone. The edges of the lesion had well-defined limits, a hard consistency and were slightly hemorrhagic upon contact. Surrounding the entire lesion, an erythematous halo with well-defined limits was identified .Laboratory tests that were performed to investigate hematological (anemia) or immunological alterations provided negative results. Human immunodeficiency virus (HIV) testing was also negative. The patient’s C-reactive protein levels and Erythrocyte Sedimentation Rate were slightly elevated. The diagnostic hypotheses considered were necrotizing sialometaplasia vs. an ulcerated tumor of the minor salivary glands vs. a major mucosal ulceration. An incisional biopsy of the lesion was performed covering the most anterior area of the ulceration, involving the edges and healthy tissue of the palate and including a portion of tissue from the central area of the lesion . The postoperative period occurred without complications and without aggravation of the clinical characteristics of the lesion.The biopsy exhibited a slightly hyperplastic epithelium, limiting the ulceration surface. In the underlying connective tissue, we observed the foci of the necrosis and some minor salivary glands in pre-necrotic status. Most acinar cells exhibited absent cores and the presence of vacuoles in the cytoplasm, while maintaining their lobular architecture. At greater magnifications, the necrosis and inflammatory infiltration of acinar cells, the foci of malpighian metaplasia in the residual channels and some acini in canalicular transformation were confirmed .These histological findings were characteristic of necrotizing sialometaplasia. Pain treatment was performed with paracetamol if needed. Adequate oral hygiene (tooth brushing and chlorhexidine rinses) was prescribed to prevent the superinfection of the ulcerated area. The patient performed periodic controls every two weeks. After four weeks, a marked improvement in the lesion was observed  and, at Bulimia can be considered an eating disorder  , vascular frailty is not significant and therefore should not be included, per se, in the vascular etiologies of NS . These p


Pleomorphic adenoma is a benign mixed tumor composed of epithelial and myoepithelial cells, less commonly occurring in minor salivary glands or in children. A case of juvenile pleomorphic adenoma missed clinically and incidentally found in cone beam CT images is described. Clinical, radiographic, and histologic images are presented. Pleomorphic adenoma (PA) is a benign mixed tumor composed of epithelial and myoepithelial cells with variable morphological patterns, surrounded by fibrous capsule. Salivary gland tumors are rare; comprising 3% of all neoplasms of the head and neck region, most are benign 65-70%) . PA is t-70% . PAPediatric PA in minor salivary glands is rare and can occur in the palate, upper lip, buccal mucosa, tongue, and gingiva , 5. WithThere are few case reports in the dental literature on PA with radiographic examination. However, these reports are of children who initially presented complaining of an intraoral swelling. To the knowledge of the author, this is the first report of a pleomorphic adenoma in a child found incidentally in cone beam CT imaging.Nine-year-old girl was referred by her dentist to the Interdisciplinary Airway Clinic at the University of Alberta, Canada, for symptoms of sleep-disordered breathing and dental malocclusion. In this tertiary center, patients with maxillary-mandibular jaw disproportions with sleep-disordered breathing symptoms are evaluated by orthodontist, paediatric respirologist/sleep medicine specialist, and pediatric otolaryngology surgeon. Cone beam CT tomography of the maxillofacial complex was completed and reviewed by an oral and maxillofacial radiologist. The scan was obtained using Next Generation iCAT®  with 0.3 mm voxel, 4 s of exposure, 120 kVp, and 5 mA. Acquisition of CBCT scan was based on orthodontic reasons where conventional radiography failed to provide adequate information.There were no head and neck asymmetries. Palpation of the neck lymph nodes was negative for tenderness or enlargement. Temporomandibular joint examination was unremarkable.The lips, tongue, labial, buccal, and vestibular mucosa were within the range of normal. Early mixed dentition with no evidence of caries, the gingiva, and alveolar mucosa are of normal color and texture. Examination of the oropharynx reveals hypertrophy of the tonsils. The examination of the soft and hard palate was initially unremarkable.There were two incidental findings: first, a foreign body in the right external ear canal; second, a well-defined concavity in the right palatal process of the maxilla-premolar area .In its greatest dimensions, it measured around 10 mm (width) × 10 mm (depth) × 5.7 mm (height).The concavity extended to the mid palatine suture medially and superiorly displaced the floor of nasal cavity. The soft tissue shape and density within and immediately surrounding the concavity was unremarkable, partly due to the adherent tongue-to-palate position during scan. The radiographic features were consistent with benign-cystic tumor. A careful inspection of the intraoral photographs showed mild asymmetry of the hard palate due to small swelling with normal looking mucosa .Punch biopsy revealed irregular sheets, trabeculae, and islands of ovoid or polygonal cells associated with duct-like structures with areas of squamoid differentiation. The supporting fibrous stroma shows zones of myxoid change .Excisional biopsy specimen was negative for immunohistochemical marker S100 and confirms pleomorphic adenoma. Surface ulceration with fibrinoid necrosis and pseudoepitheliomatous hyperplasia was present.The patient underwent general anesthesia for the removal of adenoid hypertrophy, foreign body of the ear, and excisional biopsy of the palatal swelling. No complications were reported.Four months postsurgery, the patient showed adequate healing. Eighteen months postsurgery, there were no clinical signs of recurrence.Pleomorphic adenoma has been reported in patients as young as 3 months up to 18 years, with slight female predilection, 1 : 1.4 . The symInitially, the bone immediate to the soft tissue swelling would not be affected. Over time when the tumor grows enough, it may cause pressure resorption and ultimately significant erosion and perforation of the palatal bone/floor of the nasal cavity. The reported case was noted as an incidental finding in the cone beam CT due to pressure resorption of the hard palate, without break in the floor of the nasal cavity. Soft tissue changes were not evident in the cone beam CT due to lack of soft tissue resolution, and tongue position against the hard palate at the time of scan hindered the observation of a swelling silhouette. Few case reports of pediatric PA causing an erosion or displacement in the hard palate were found in the literature –12. AlthComputed tomography and MRI are typically used in tumors of major salivary glands or large tumors of minor salivary glands. CT shows well-defined, smooth or lobulated, heterogeneous, and hypoattenuating masses compared with surrounding soft tissues and rarely presenting with mineralized foci . MRI T2 Histologically, PA can be classified into myxoid, classic, or cellular, depending on the amount of stroma and cellular components. Myxoid type consists of myxomatous stroma, classic type is a mixture of ductal structures and myoepithelial cell in a myxomatous stroma, and cellular type has a large number of cellular elements relative to the stroma . Howeverα-smooth muscle actin (SMA), CD 117, and CD 43 was not beneficial in differentiating between PA, adenoid cystic carcinoma, and polymorphous low-grade adenocarcinoma [The palate contains soft tissues other than minor salivary gland tissues. As such, fibroma, lipoma, neurofibroma, neurilemmoma, and other salivary gland tumors should also be considered in the differential diagnoses for this case. Although hematoxylin-eosin (HE) staining is the gold standard for diagnosing salivary gland tumors, immunohistochemistry (IHC) can enhance its accuracy. However, a histopathological study showed that the use of glial fibrillary acidic protein (GFAP), arcinoma .α-Smooth muscle actin (SMA), calponin, and muscle-specific actin (HHF35) are highly specific [The following are markers for myoepithelial cells: vimentin and S100 but with low specificity, glial fibrillary acidic protein (GFAP) that is highly positive in PA and myoepithelioma, and p63 and CK14 that can be positive for basal and squamous epithelial cells. specific . It is sspecific .Combining the clinical-radiographic presentation, histological features and negative immunological assessment (S100) for the reported case favored the diagnosis of PA, with features of myxoid and classic types.The biological behavior of PA in young patients seems to be similar to that in adults with low chances of recurrence after adequate surgical excision , 7. HighThis report brings attention to a salivary gland tumor that was not noticed by the patient and parents or on initial oral examination. It was the bony erosion caused by the tumor that was incidentally noted upon systematic examination of the cone beam CT images. This highlights the importance of thorough clinical and radiographic examinations beyond the area of chief complaint.Pediatric pleomorphic adenoma in the maxillofacial complex is uncommon, however should be considered in the differential diagnosis of young patients with swellings in the oral cavity. Important sites to consider are the palate, lips, tongue, and buccal mucosa. In rare occasions, diagnostic imaging could be the first line to detect such lesions incidentally by careful examination of the osseous structures and surrounding soft tissues. Consistent follow-up to monitor recurrence or malignant transformation is paramount in this young population.


Background and Objectives. Kaposiform hemangioendothelioma (KHE) is a vascular tumor of very low incidence, which occurs mostly in children and infants. The tumor is recognized for its locally aggressive, yet rarely metastatic behavior. It may cause consumptive coagulopathy known as Kasabach-Merritt phenomenon. We report a distinctive case, where an 11-year-old boy is presented with progressive thoracolumbar scoliosis without any symptom or neurological sign. Case Report. The patient underwent spinal deformity correction via posterior pedicle screw instrumentation and fusion, along with tumor biopsy. The pathology report confirmed KHE. The patient did not show a prominent progression of scoliosis after the surgery without any further treatments. Conclusions. Many of scoliotic patients do not have any apparent cause, thereby regarded as idiopathic scoliosis. The presented case is where kaposiform hemangioendothelioma is likely to be linked to the patient's scoliosis. We demonstrate the possibility of secondary scoliosis should always be kept in mind of orthopaedic doctors. We also conclude that secondary scoliosis does not show exacerbation after growth completion. Kaposiform hemangioendothelioma (KHE) is a rare tumor of vascular origin, which usually presents in childhood . KHE is A boy with mild scoliosis has attended the outpatient clinic for the first time. He had no medical history of being treated for other diseases and no particular genetic predisposition. He was 11 years and 10 months of age at the first visit. A thoracolumbar curve convex to the right side was identified. Cobb's angle was measured 14.8 degrees, and Risser stage 0 was confirmed via whole spine plain radiograph . He was At the age of 15 years and 3 months (Risser stage 3), Cobb's angle had worsened despite the brace therapy he had received for 18 months. He had neither pain nor neurological signs. The only symptom is that he felt the back was a bit curved; however, he still managed to participate in sports activity without much trouble. Laboratory evaluation at the time of hospital admittance included CBC, electrolyte panel, infection panel (CRP and ESR), and coagulation labs, which were all within normal range. His right thoracic hump was assessed 9.0 cm from the midline, and 31 degree of hump inclination was measured. In the whole spine simple radiograph, the scoliotic curve apex was at L2, with end vertebrae at T8 and L4. Cobb's angle of 52.0 degrees was recognized, which decreased to 35.7 degrees on the bending film .Because the scoliosis had progressed despite the patient had approached to growth termination, MRI scan was taken. It showed high-signal intensity lesion from the left side of T11 and T12 vertebral bodies, along the left pedicles to laminae on T1-weighted images . HypoplaThe official pathology result, however, revealed KHE. The pathology slide shows infiltrating nodules with compact spindle cells surrounded by slit-like luminae . The immAfter a follow-up for more than 3 years with an annual MRI examination, KHE showed equivocal progression . Cobb's KHE is known to be a tumor of locally aggressive character. Patients are mostly presented with KHE early in childhood; about half of the incidences occur at birth , 5. It rThe tumor is well recognized for not going into spontaneous regression, unlike infantile hemangioma . No consFirst, the severity of complications regarding KHE relies on the age of onset. About 90% of all KHE incidences rise before the age of 10 , 6. Two-Second, the instrumentation and fusion without tumor removal would be sufficient to suppress the coiling effect of the KHE, if any. Zhu and his colleagues had reported a case of KHE in a 14-year-old girl, in where the KHE was located on the convex side, spanning 9 levels of vertebrae including the apex of her scoliotic curve . The girThird, another surgery might stimulate KHE in dormant state . It is pIdiopathic scoliosis is the most common cause of spinal deformity among adolescents. It should always be kept in mind to weigh the rate of scoliosis progression and remnant growth of the patient altogether. If they do not match, then suspicion to the cause of the scoliosis should be raised, followed by tests to identify the culprit.