Primary nasopharyngeal mycobacteriosis is a rare disease. We present a case in which skull base bone erosion appeared and was alleviated during the course of the treatment. Bone complications occur in osteoarticular mycobacteriosis, but their occurrence in primary nasopharyngeal mycobacteriosis has not been reported. A 77-year-old immunocompromised Asian woman presented with a right occipitotemporal headache. An ulcerative mass covered with a thick yellowish discharge was found in the roof and posterior walls of the right nasopharynx. Because histopathological examination indicated the presence of mycobacterial infection, we began using antituberculosis medication for the treatment because of the possibility of primary nasopharyngeal tuberculosis. However, this was followed by glossopharyngeal and vagus nerve paralysis. Computed tomography (CT) showed a diffuse enhancing mucosal irregularity in the nasopharynx with bony erosion of the external skull base. Deep tissue biopsy was repeated to differentiate it from malignant lesions, and drainage of pus from the right nasopharynx was confirmed. Subsequently, the headache, neurological findings, and the yellowish discharge disappeared, and the bony erosion of the external skull base was alleviated. Surgical intervention should also be considered for drug-resistant mycobacteriosis. We concluded that mycobacteriosis should also be considered apart from carcinoma even if CT shows a diffuse enhancing mucosal irregularity with bone destruction in the nasopharynx. Mycobacterium tuberculosis (TB) nor nontuberculous mycobacteria (NTM).Nasopharyngeal mycobacteriosis is a rare disease, and the irregular and swollen mucosal lesion requires differentiation from nasopharyngeal carcinoma. In previous reports of nasopharyngeal mycobacteriosis, lesions were usually found to be confined to the nasopharyngeal area without invasion into the surrounding structures such as the skull base , 2, wherA 77-year-old Asian woman presented with a right occipitotemporal headache for 1 week (day 1) but reported no other aural or nasal symptoms. Her medical history included diabetes mellitus, hypertension, rectal cancer, colon cancer, liver cirrhosis, and hepatocellular carcinoma.Bacillus culture test and polymerase chain reaction test of the sputum and nasopharyngeal smear were negative, but the T-SPOT.TB test was positive (both panels A and B > 50 spots). Cervical computed tomography (CT) revealed neither a mass shadow in the nasopharynx nor cervical lymphadenopathy , granulomatous inflammation , and autoimmune disease  . In the The most characteristic feature of the present case was bony erosion of the skull base, which appeared and alleviated during the treatment course . SrivaniIn the present case, antituberculosis medication alone was insufficient treatment; thus, the infection spreaded to the parapharyngeal space, formed an abscess, and resulted in glossopharyngeal and vagus nerve paralysis . BacteriAlthough a drug susceptibility test could not be performed, we presumed the causative bacteria as a drug-resistant TB or an NTM based on the clinical course and the patient's medical history. In such cases, the potential benefits of the surgical procedure as an adjunctive treatment for drug-resistant mycobacteriosis have been suggested , 14. In This report describes a case of primary nasopharyngeal mycobacteriosis with bony erosion of the external skull base. Differential diagnosis considering both carcinoma and mycobacteriosis should be undertaken, even when CT shows a diffuse enhancing mucosal irregularity with bone destruction in the nasopharynx. Surgical intervention should also be considered as an adjunctive treatment for drug-resistant mycobacteriosis.


Fourth branchial cleft anomalies are an exceptionally rare cause of recurrent neck mass in pediatric and adult patients. In this report, we present a case of an infected fourth branchial cleft cyst in a 20-year-old woman that presented with recurrent throat pain and deep neck abscesses. After undergoing repeated incision and drainage procedures, the patient underwent definitive management with direct laryngoscopy, ablation of the left pyriform sinus tract, left hemithyroidectomy, and excision of the branchial anomaly without evidence of recurrence. In addition to diagnosis and management, this case report highlights the unique anatomical relationship between fourth branchial anomalies and the pyriform fossa as well as the superior and recurrent laryngeal nerves. Branchial anomalies are believed to arise from incomplete obliteration of the branchial elements. They may manifest as cysts, fistulas, or sinus tracts. While second branchial anomalies are the most common (representing over 95%), fourth branchial anomalies are exceedingly rare (less than 1%) .As there are no pathognomonic clinical or radiographic features, diagnosing branchial cleft anomalies can be challenging and is based on patient history and examination . When coWe report the case of an infected fourth branchial cleft cyst, which presented as a recurrent deep neck infection in a young adult female treated at a large academic center. This case demonstrates the diagnostic challenge of these anomalies and the keys to their successful management. A focused literature review highlighting the history surrounding these lesions is also provided. A 20-year-old female presented in early 2018 with a two-year history of recurrent left neck abscesses and treatment with antibiotics and steroids. She had recently undergone an incision and drainage procedure of a left deep neck abscess at another facility, but presented to our emergency department with recurrence several weeks later. Due to concerns for a possible congenital anomaly, she underwent percutaneous drainage by interventional radiology with resolution of symptoms. She remained asymptomatic with a normal physical exam through September 2018.She then re-presented to us in October 2018 with throat pain and left lateral neck swelling and induration. CT neck with contrast demonstrated an ill-defined heterogeneous lesion of the left paratracheal region with a fistulous tract extending toward the left pyriform sinus  antibiotics and repeat drainage. She was subsequently taken to the operating room (OR) for incision and drainage and direct laryngoscopy. Intraoperatively, an opening to the tract in the left pyriform sinus was identified. However, it was not cauterized at that time due to significant edema. Intraoperative cultures were obtained, and the patient was subsequently given culture-directed antibiotics.After improvement in her acute inflammatory symptoms, she was taken back to the OR for definitive excision of the branchial cleft cyst and endoscopic management of the fistulous tract. Direct laryngoscopy revealed a well-defined sinus tract opening in the apex of the left pyriform sinus Figure  that wasSubsequent open neck dissection was difficult due to scarring and obliteration of natural tissue planes as a result of her recurrent infections. At the level of the cricoid cartilage, medial to the internal carotid artery and jugular vein, an ill-defined soft tissue density, suspicious for the remnant branchial cleft cyst, was identified. This specimen was tightly adherent to adjacent strap musculature which necessitated resection with partial muscle sacrifice. As the superior thyroid pole was transected, we identified a distinct fistulous tract connecting the mass to the undersurface of the thyroid cartilage in the direction of the left pyriform sinus. The tract traveled in tandem with the recurrent laryngeal nerve Figure . This trSurgical pathology demonstrated suspected branchial cleft anomaly and benign thyroid tissue with adherent soft tissue, consistent with a tract. At most recent follow up nine months post-operatively, the patient has healed well and not experienced any pain or infection.Branchial anomalies are an important consideration in the differential of recurrent pediatric and adult neck masses. As demonstrated with this case, fourth branchial cleft cysts are commonly left-sided and course from the apex of the pyriform fossa to the upper aspect of the left thyroid lobe. As a result, patients can present with a recurrent abscess in the low anterior neck or recurrent suppurative thyroiditis .In the past, it has been debated as to whether third and fourth branchial anomalies represent two distinct entities. The terms "pyriform sinus tract” or “pyriform sinus lesion” have been applied to both. However, it has more recently been accepted that they indeed are different. Third branchial anomalies originate from the base of the pyriform sinus and pass above the superior laryngeal nerve to enter the larynx via the thyrohyoid membrane. In contrast, fourth branchial anomalies originate from the apex of the pyriform sinus and run in parallel with the recurrent laryngeal nerve, as demonstrated in this case . The tandard curative treatment for fourth branchial cleft anomalies is surgical excision. However, due to the anatomical course, complete excision is often difficult . In thisThis case illustrates the presentation, diagnosis, and management of a rare branchial anomaly presenting as recurrent neck infections in an adult. Fourth branchial anomalies are often misdiagnosed due to their rarity and lack of clinical or imaging features. Accurate diagnosis relies on keen attention to the history, thorough knowledge of the anatomy, and a high index of suspicion. This case highlights the unique anatomical relationship fourth branchial anomalies have with the pyriform sinus and the superior and recurrent laryngeal nerves. Successful, definitive management requires complete excision of left branchial cleft cyst and ablation of the associated pyriform sinus tract.


The Numb Chin Syndrome (NCS) is defined as facial and oral numbness restricted to the mental nerve’s distribution involving the lower lip, skin of the chin, or gingiva of the lower anterior teeth. Hypoesthesia can occur unilaterally or bilaterally. Although this syndrome is rare, its importance is related to the fact that it represents the clinical manifestations of malignant diseases. Breast cancer and non-Hodgkin lymphoma are the most common cause of NCS. The patient, a 58-year-old woman, treated for a Burkitt Lymphoma (BL) nine years ago, described a two-week history of change in sensitivity and pain in the chin region, without relief with the use of analgesics. She had no headache, speech disturbance, dysphagia, visual disturbance, or other neurological symptoms. No surgical intervention has been performed recently. The intraoral examination revealed a healthy oral mucosa and a small area adjacent to the right mental nerve region that was uncomfortable to palpation. No changes were found in the bone trabeculae at cone-beam computed tomography. The contrasted magnetic resonance features made it possible to identify a change in the mandibular body extending to the entire right side, coinciding with the patient’s complaint, indicating a probable mandibular medullary invasion. The patient was submitted to a biopsy to rule out a possible recurrence of BL. The microscopic findings were consistent with the diagnosis of BL. The present report described a very unusual presentation of late recurrent BL nine years after the first treatment, which manifested as an NCS. It causes a sensory neuropathy-like unilateral, or bilateral hypoesthesia, paresthesia or anesthesia in the area supplied by the trigeminal nerve’s mental branch. These symptoms reflect the anatomical substrate of this syndrome.Despite being a rare clinical manifestation, the importance of this syndrome lies in its frequent association with malignancies.,Lymphomas are malignant tumors derived from the lymphoreticular or immune system. BL belongs to the NHL group and is characterized by a very high and diffuse proliferation of B lymphocytes.Charles Bell first described NCS in 1988. This syndrome may be the first symptom of diseases arising from different etiologies, including traumatic, vascular, inflammatory, demyelinating, infectious, and neoplastic disorders.In the present paper, we present a rare case of late recurrence of BL initially presenting with bilateral NCS.The patient, a 58-year-old woman, treated for a BL nine years ago, described a two-week history of change in sensitivity and pain in the chin region, which did not relief with the use of analgesics. She had no headache, speech disturbance, dysphagia, visual disturbance, or other neurological symptoms.At first, she looked for her hematologist-oncologist because of her past medical history of BL. Physical examination revealed complete paresthesia involving the chin and lower lip bilaterally, without any trauma and no evident increase in volume or swelling, only the numbness in the mental region. There was no clinical evidence of palpable regional lymph nodes, and all other systems were found to be clinically normal.The patient was referred to the Oral Medicine Department, and the intraoral examination revealed a healthy oral mucosa, no recent tooth extraction, no trauma, and no oral surgery .As a result of the patient’s complaint, we identified a small area adjacent to the region of the right mental nerve that was uncomfortable on palpation. No other changes were found by means of cone-beam computed tomography A and cliAt this time, considering the medical history, an intraosseous biopsy  adjacentin-situ hybridization was negative for Epstein-Barr virus. Rescue chemotherapy treatment was initiated to enable an autologous transplant to be performed.The microscopic analysis of the bone biopsy demonstrated a diffuse proliferation of moderate-sized neoplastic cells, exhibiting hyperchromatic nucleoli, and scant cytoplasm. A focal starry-sky pattern was observed. The immunohistochemical profile  revealed,Lymphoma is a heterogeneous malignant disease of the lymphatic system, characterized by a proliferation of lymphoid cells or their precursors. Lymphomas represent the third most common malignancy affecting the oral cavity and jawbones after squamous cell carcinoma and the malignant tumor of the salivary glands.Short et al.,The first report of mental neuropathy associated with malignancies was filed in 1963 with the description of 5 cases of patients with metastatic malignant disease, who had NCS as the initial presentation.This syndrome has been attributed to benign and malignant conditions,Some cases of NCS may also be the first sign of recurrence or metastasis in patients with a history of malignancy, or sometimes the first manifestation in hematologic malignancies.,,This syndrome requires special attention from clinicians in BL cases to avoid misdiagnosis since there are other causes of NCS. A review of literatureFortunato et al.On the other hand, Sasaki et al.,In clinical practice, most clinicians do not request panoramic radiography of the jaw to start the investigation. In general, physicians do not start with either computed tomography (CT) or magnetic resonance image (MRI) of the jaw, face, and brain, which fails to place value on the patient's chief complaint. CT imaging of the brain and base of the skull makes it possible to identify lytic lesions involving the inferior alveolar nerve or the mental foramen. Some authors have recommended MRI scanning of the face if the etiology of the mental neuropathy is unclear after routine imaging studies have been performed.,,,According to the characteristics of each case, there were some possible diagnostic hypotheses to be considered, such as Fortunato et al.In conclusion, the present report described a very unusual presentation of late recurrent BL nine years after the first treatment, which manifested as an NCS.


Ewing's sarcoma (ES) of the hip and trochanteric region is a rare malignancy. The tumor has a poor prognosis due to the problems in early diagnosis and medical intervention.This paper reports a rare case of hip ES presented in a 34y/o female. The clinical, radiological, and histopathological features were all in favor of ES. Following treatment by neoadjuvant/adjuvant chemotherapy, and irradiation the patient is now with complete resolution of the tumor.The patient remained free of disease through 4 years of follow-up until now after diagnosis. ImmunohWe herein introduced a 34y/o female case of ES arisen in her left hip with no evidence of recurrence following treatment. Furthermore, a review of pertinent literature was done to make the clinicians aware of the clinical and histopathological spectrum of this rare tumor.2The case was a 34y/o female developed with left hip pain and inability to weight-bearing after simple falling while walking. Thus her weight-bearing was impaired severely. Following informed consent, she was visited at the Orthopedics department and complained of obscure hip pain for six past months. She had been visited previously by physicians several times and treated as muscle spasm and low back pain. Her history was unremarkable, and the patient did not mention any positive past medical, surgical, or drug prescription history. In addition, there were no signs of fever and weight loss. Physical examination detected hip tenderness, and restricted range of motion . The neuThe clinical and radiological assessment suggested a destructive bone lesion with a suspicion of malignancy. Then the patient was admitted to our institute at Jundishapour University of Medical Sciences, Ahwaz, Iran for further evaluation. Tumor follow-up was started and necessary evaluations such as pelvic MRI, related lab tests, chest, and abdominal pelvic CT scan, bone mass densitometry, and whole body scan were done. The results of lab tests including Hb, PLT, WBC, Ca, P, BS, TFT, LFT, BUN, CRE, ESR, PTH were in the normal range and viral markers were also negative. Besides, the obtained results of several measured specific bone turnover markers including serum bone-specific alkaline phosphatase (BSAP) (79.69 ± 3.9 U/L), and osteocalcin (23.58 ± 5.1 ng/mL) demonstrated a high level in serum of the patient. The obtained MRI images of the pelvis showed a large mass with a 131 ∗ 70 mm size and irregular border at T1/T2 intermediate signal intensity. Furthermore, MRI revealed an intramedullary lesion with heterogeneous enhancement at diaphysis and metaphysis of the proximal part of left femur and infiltration to adjacent anterior and posterior muscle compartment. There was no involvement of neurovascular bundle enhancement at diaphysis and metaphysis of the left femur proximal part .Fig. 2MRMRI scans  showed an expansion of the subperiosteal space, without spread to the underlying cortex. Whole body scan with Technetium-99m (TC_99m), demonstrated a hypoactive expanded bony lesion in left trochanteric region without involvement of other sites . Therefo2, Adriamycin 75 mg/m2, and cyclophosphamide 1200 mg/m2) alternating with IE (ifosfamide 1800 mg/m2 and etoposide 100 mg/m2) for 6 months. Then, radiation therapy consisted of 5400 cGy (180 cGy/fraction) was added to chemotherapy for local control of the tumor metastasis. The patient until now (4 years later) has remained alive without evidence of recurrence or metastasis.The patient was referred to an oncologist and so decided for adjuvant chemotherapy regimen, and radiotherapy without surgical resection, and closed follow-up. She was then treated with 7 cycles of adjuvant chemotherapy regimen consisted of VAC  were done based on the recommendations of the CESS/LESS groups that proposed a defined timing of different imaging procedures . Follow-3Ewing sarcoma (ES) is one tumor type of what is now regarded to be a category called Ewing sarcoma family of tumors (ESFT), which comprises peripheral primitive neuroectodermal tumors (PNETs) and Askin tumors. Many researchers have opinioned it as a different nature and the argument continued . InitialIn many non resectable Ewing sarcomas, radiation therapy is often used after chemotherapy to further kill cancer cells and control their growth. The surgical approach should be combined with chemotherapy and additional radiotherapy in cases involving a marginal resection and/or poor histological response. The limited number of patients above the age of 30 and the removal of these patients from the majority of trials render these findings difficult to generalize . In the The literature review revealed a few cases of pelvic Ewing sarcoma which were treated with several chemotherapeutic regimens. The heterogeneity of the used regimens reflects the rarity of the disease and the evolution of multi-agent chemotherapy for Ewing Sarcoma during the last few decades . ES DiagPrompt diagnosis, by multimodality approach, which includes appropriate imaging (bone scan with radioisotopes), biopsy (diagnostic or excision) is required to tailor appropriate treatment regimen for optimal profit. Collectively, these diagnostic techniques were critical in supporting the proper diagnosis of ES metastatic to the other sites .In ES, bone serum markers are helpful to assess the tumor diffusion, the response to therapy, and survival. The osteoblastic lesions are manifested by high levels of serum bone turnover markers. High levels of these markers forecast poor prognosis. The utilization of bone markers in malignant tumors permits an early diagnosis, fast onset of therapy, restricted complications, and predict the risk of relapse. However, the sensitivity of these markers is very low, proposing that they cannot be preferred over conventional bone scans in the diagnosis of bone metastases . We hereCancers of the bone and soft tissue, such as osteosarcoma, chondrosarcoma, merkel cell carcinoma, and leiomyosarcoma, have also been implicated with metastasis to the pancreas. However, ES metastasis to the pancreas is considered very rare. Endoscopic ultrasound  is both Based on the literature searches, 43 cases of ES, including Ewing's sarcoma of bone (ESB), extraskeletal Ewing sarcoma (EES), and PNET involving the pancreas (either as primary or metastasis), had been reported. Among these, only three pelvic cases appeared to be metastatic. These patients were treated with a combination of surgical resection, chemotherapy, and radiation. Disease progression and outcome data were unavailable in one of these three cases, as reported by In general, approximately 20–30% of ES patients present with metastases at the time of their diagnosis. Multidisciplinary treatment modalities have dramatically improved the 5-year survival rate of patients from 16 to 75% . Beam irIn our case, the patient did not have metastatic lesions. Consequently, she was treated successfully with trimodality therapy including combined radiation and chemotherapy. The patient until now has remained disease free 4 years after her diagnosis. The patient returned to pain free ambulation without any shortcomings in terms of diagnosis, evaluation, follow up, treatment, and/or quality of life post treatment except for slight limping. Angervall and Enzinger in 1975 announced the first case of extra skeletal ES . Askin i4Ewing sarcoma is a very aggressive tumor with variable prognosis relying on stage, site, size, microscopic feature, and biological characteristics. Prognosis has ameliorated thanks to modern chemotherapy and radiopharmaceuticals advancements. The classical, clinical and radiological manifestation of hip area in ES may not be the rule; one should be highly suspicious of the disease even if there is no direct indication to the disease as was encountered in our case. In addition, ES may be misdiagnosed as benign disorders. ES cases of the tibia may mimic as osteomyelitis. ES may manifest as knee monoarthritis, spondylolisthesis, and as postpartum back pain. ES of ilium mimic inflammatory hip arthritis or sacroiliitis. Orthopaedicians, rheumatologists and radiologists should be conscious to this infrequent incidence.EFSEvent-free survivalESEwing's sarcomaIEIfosfamide and etoposideIHCImmunohistochemistryOSOverall survivalROMRange of motionEUSEndoscopic ultrasoundThe authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.


Small bowel tumors presenting with hemorrhagic shock require urgent treatment with angiographic embolization to achieve hemostasis. Capsule endoscopy and double‐balloon endoscopy are useful for localizing the tumor, diagnosis, and guiding surgery. Small bowel tumors presenting with hemorrhagic shock require urgent treatment with angiographic embolization to achieve hemostasis. Capsule endoscopy and double‐balloon endoscopy are useful for localizing the tumor, diagnosis, and guiding surgery. Endovascular treatment was possible after abdominal angiography to visualize the specific bleeding site clearly, with coil embolization to achieve hemostasis. Further treatment by laparoscopic‐assisted partial resection of small bowel was successful.Small intestinal bleeding is difficult to diagnose. In many cases, hemostasis cannot be achieved easily. The etiology of small bowel bleeding in patients age >40 years varies widely. Common causes include vascular lesions, such as angioectasia, tumors, ulceration from nonsteroidal anti‐inflammatory drugs, and Dieulafoy lesions.2A 66‐year‐old Japanese woman presented to the emergency room with melena for 3 days. She had a history of diabetes, hypertension, cerebral infarction, and angina. Gastrointestinal bleeding is suspected, and she was admitted to the hospital for further investigation and treatment.On admission, vital signs were as follows: temperature, 36.2°C; blood pressure, 118/70 mm Hg; pulse, 84 beats per min; respiratory rate, 19 breaths per min; and oxygen saturation, 97%; she was breathing ambient air. Consciousness was clear, and physical examination showed pale palpebral conjunctiva; the abdomen was flat and soft without tenderness, and no mass was palpable. Laboratory evaluation was significant for hemoglobin at 4.3 g/dL and severe anemia with blood urea nitrogen at 33.6 mg/dL.An emergent upper endoscopy revealed no vascular abnormalities, mass lesions, or ulcerative lesions as the potential source of bleeding Figure . A lowerAfter admission, there was no hemorrhage, and the anemia improved with blood transfusion. On hospital day 5, however, a large amount of melena was observed and her blood pressure was 68/40 mm Hg. Computed tomography of the abdomen showed a mass lesion as Figure , but theA capsule endoscopy was performed on hospital day 7 to determine whether the oral or transanal approach was optimal until the patient recovered to a condition that would allow her to tolerate a small bowel endoscopy. The results of the capsule endoscopy were revealed on hospital day 9. At 1 hour and 4 minutes into the capsule endoscopy examination, active bleeding was observed, which seemed to be in the upper small bowel Figure . Thus, aA contrast agent from the superior mesenteric artery (SMA) indicated a vascular tumor in the jejunal artery branch. There were no other abnormal vessels in other parts of the body that appeared to be the source of bleeding. The patient underwent embolization with coils in the imaged area Figure , and hemAfter the patient's general condition improved, she underwent an oral double‐balloon small intestinal endoscopy on day 13. A 40‐mm submucosal tumor (SMT) was detected 80 cm from the dental line. A GIST with erythematous dilated vessels and erosions at the apex was suspected, and surgical resection was scheduled.Preoperatively marking clips were inserted on the oral and anal sides Figure . This peOn day 32, the patient underwent a laparoscopic partial resection of the small intestine. The tumor was protruding outside the intestinal wall. Therefore, the planned intraoperative imaging was not needed Figure . The porOn pathologic examination, hematoxylin and eosin staining showed spindle‐shaped, irregularly enlarged cells in bundles, and immunostaining was positive for c‐Kit. The rate of CD34 was positive, S‐100 was negative, and MIB‐1‐positive findings were 0.9%; therefore, the tumor was classified in the low‐risk group Figure .3The epidemiology of GIST is described as follows: mean age at diagnosis, 63 years; male‐to‐female ratio, 54:46; organs in which this tumor occurs are the stomach (51%), small intestine (36%), large intestine (7%), rectum (5%), and esophagus (1%). The 5‐year survival rate is 45%.The distribution of clinical presentation is as follows: gastrointestinal bleeding, abdominal distention, and abdominal discomfort. Further, 15%‐30% cases have been reported to be asymptomatic.An algorithm for the treatment of small intestinal bleeding is set out in the guidelines. A distinction is made between cases with and without shock. In cases of small bowel bleeding with shock, early angiography is recommended.Intraoperative imaging showed that the surgical resection site could be planned using coils and marking clips as indicators, which led to a laparoscopic‐assisted partial resection of the small bowel. A few reports have documented emergency surgery for hemorrhagic small bowel GIST; in this case, however, it was worthwhile to stabilize the patient first and then perform a curative resection of the tumor in minimally invasive surgery.4Diagnosis of small bowel GIST is possible using a combination of a capsule and double‐balloon endoscopy, depending on the patient's condition. Furthermore, with angiographic examination and treatment, hemostasis of the small bowel GIST was successful, subsequently allowing a laparoscopic‐assisted resection of the tumor to be performed.The authors declare that they have no competing interests.HK: drafted the manuscript. HT and KS: performed the operation. TI: performed the endoscopy. HT, KS, TI, and HY: revised the manuscript. HK, KS, TI, and HY: read and approved the final manuscript.Informed consent was obtained from the patient for the publication.No ethical issue in reporting of this case.


Congenital medulloblastoma is a rare brain tumor that appears in less than 1% of pediatric patients. Congenital medulloblastoma has a poor prognosis and should be suspected in patients with clinical manifestations of hyporeactivity, slow suction reflexes, and the presence of hydrocephalus. Herein we present the case of a 12-day-old female newborn who developed non-communicative hydrocephalus, hyporeactivity, and hyporeflexia. Magnetic resonance imaging of her brain showed a heterogeneous and cystic mass on the posterior cranial fossa. A suboccipital craniotomy was performed. The histopathologic analysis reported a congenital medulloblastoma. She remained in hospital until her death at 112 days old. This is one of the first case reports with clinical-radiological and pathological documentation. Awareness of this diagnosis can allow prenatal intervention, rendering a better prognosis. This case report exemplifies the importance of good prenatal follow-up. A congenital medulloblastoma is extremely rare compared with medulloblastomas in general. It is usually diagnosed postnatally, is a form of neoplasm that originates in the posterior fossa at the level of the cerebellum, and is a highly aggressive neoplasm characterized by undifferentiated cells.,In general, congenital brain tumors—one of the rarest of all tumors reported in the literature (which includes medulloblastoma)—are usually diagnosed within the first 2 months of life.This report presents one of the very first cases to use pathological images and imaging studies. The importance of this case report is that congenital medulloblastoma shows a strange pathology, which usually has a high mortality rate; it also can have a similar clinical presentation to other congenital brain tumors and shows a very similar pattern on imaging.A female baby was born by uneventful vaginal delivery at 36.5 weeks' gestation with an APGAR score of 8 and 9 at the first and fifth minutes, respectively. Her weight was 2.3 kg, her length was 48 cm, and her cephalic perimeter was 28 cm. The mother was 39 years old  who attended prenatal consultation during the first trimester of pregnancy. On the baby's first day, she had ocular secretion, jaundice, hyporeactivity, and slow suction reflexes. On day 3, she presented six apnea episodes. A transcranial ultrasound reported obstructive hydrocephalus. The MRI A showed The patient was submitted to a suboccipital craniotomy, and the intraoperative pathological assessment depicted a primitive and undifferentiated neoplasm with neuroectodermal features. The final pathology diagnosis was medulloblastoma  .Klebsiella pneumoniae pneumonia and required orotracheal intubation and an antibiotic regimen with vancomycin and levofloxacin. On day 33, she presented clinical improvement; however, 1 week later (day 40), the intracranial pressure increased again. Hyperchloremic metabolic acidosis ensued, and the fistula continued to drain serous-sanguineous fluid. The patient started with partial and focal seizures that manifested in the upper right limb and included sucking movements. Computed tomography of her brain was performed and revealed active bleeding and increased mass volume with medulla oblongata involvement , the patient presented respiratory distress and oxygen desaturation due to olvement B. Her clMedulloblastoma, an embryonal neuroepithelial tumor of the cerebellum, is known to be the most common malignant brain tumor in Pediatrics (WHO Grade IV), representing 20% of all brain tumors in this age group. The outcome is poor, and a fatality occurs in one-third of all the patients with this condition despite the therapeutic improvements.,-The association of maternal diet and the higher risk of medulloblastoma was raised but not confirmed.-Children are 10 times more likely to be affected by medulloblastoma than adults, and its incidence is calculated to be 1.5 per million population in the USA.,Symmetric ventriculomegaly and a midline cerebellar lesion  have been previously described and can be compared with our patient.Congenital anomalies and genetic syndromes have been described as being related to medulloblastoma; however, in a series of 173 patients with medulloblastoma, as reported by Evans et al.,,In Hispanic literature, we found a few cases with similar clinical presentation to our case.,-Other relevant diagnoses to take into account are teratoid/rhabdoid immature tumors (ATRT), a highly aggressive neoplasm that affects very young children, arises in the cerebellum and is composed of rhabdoid and other mesenchymal cells, and the loss of both copies of the INI1gene is it molecular signature; and embryological brain (PNETs), these tumors produce symptoms within the first 2 months of life (posterior fossa location) causing hydrocephalus, vomiting, and immunochemistry should be done to facilitate the tumor identification using the 2016 WHO classification of brain tumors.Congenital medulloblastoma is one of the most uncommon malignant brain tumors that can be diagnosed before birth. In general, its clinical presentation, imaging, and the outcome are very similar. When this condition is suspected, an MRI should be performed before birth, which may permit intrauterine intervention, and thus render a better prognosis Molecular and genetic research should be done in central nervous system tumors typification to understand their unique characteristics and treatment.


The perforation of a cecal diverticulum is a rare and challenging conditionfor the emergency physician.A 47-year-old man with a past surgical history of bilateral inguinal herniarepair presented to the emergency department (ED) with acute abdominal painof three days’ duration. Pain was localized to the right lowerquadrant (RLQ), with anorexia as the only associated symptom. Upon arrivalto the ED, his exam demonstrated focal RLQ tenderness to palpation, reboundtenderness, and guarding. Labs did not show any elevation in inflammatorymarkers, liver enzymes, or lipase. Computed tomography showed no evidence ofacute appendicitis, colitis, or hernia recurrence. After morphine andreassessment, the patient still had a focal peritoneal exam in the RLQ.Surgical consultation was obtained and recommended additional non-opioidanalgesia as well as serial abdominal exams. After several repeat abdominalexams, there was no change in the focality of the patient’s pain.Surgery was reconsulted and opted to take the patient to the operating roomfor exploratory laparoscopy with “appendicitis” as thepresumptive diagnosis. Pathology report revealed a perforated cecaldiverticulum that was adherent to the abdominal wall. The patient did welland was discharged on his third postoperative day.This case further underlines that even in the era of sensitive imaging tools,the diagnostic value of a targeted physical exam with clinical re-evaluationcan never be overestimated. The perforation of a cecal diverticulum is a rare and potentially challengingcondition for the emergency physician. It is uncommonly diagnosed in the emergencydepartment (ED) and generally undiagnosed pre-operatively. Most patients are takento surgery for suspected appendicitis, and only on pathology is the actual etiologydiscovered.A 47-year-old male with no past medical history and a past surgical history ofbilateral inguinal hernia repairs presented to the ED with acute right lowerquadrant pain of three days’ duration. Pain was constant, progressive, andassociated with anorexia. There was no associated nausea, vomiting, fever, chills,dysuria, or constipation. He had never experienced any similar episodes of pain inthe past. Upon arrival to the ED, his temperature was 37° Celsius, heartrate 85 beats per minute, blood pressure 114/77 millimeters of mercury, and hisrespiratory rate was 18 breaths per minute. On exam, his abdomen was soft andnon-distended with severe, focal right-sided tenderness around McBurney’spoint with associated guarding. Rovsing’s and psoas signs were negative.Coughing elicited pain in the patient, but there was no bulge suggesting recurrenceof his hernia. His laboratory values and urine analysis were relatively unremarkable.A computed tomography (CT) of the abdomen with intravenous contrast showed“no evidence of acute appendicitis.” The report also stated:“no evidence of recurrence of right inguinal hernia, and numerousfluid-filled small bowel loops in the right abdomen that in the appropriate settingcould reflect enteritis”  and 2.Upon reassessment, after one dose of four milligrams of morphine, the patient stillhad the same exam: focal point tenderness to an area near McBurney’s point.A decision was made to consult surgery. The surgical team recommended serialabdominal exams by the emergency physician as well as administration of ketorolac asan attempt to rule out a musculoskeletal etiology of the pain. However, after serialexams six hours later, there was no change in the findings, and peritonitis remaineda concern. Surgery was reconsulted and reassessed the patient, deciding to take himfor exploratory laparoscopy and appendectomy, with appendicitis as the presumptivediagnosis. In the operating room, findings were consistent with cecaldiverticulitis, and the surgeon removed the affected part of the cecum. The surgeonalso opted to remove the appendix. Pathology reports revealed a perforated cecaldiverticulum that was adherent to the abdominal wall, and a normal appendix. Thepatient was discharged three days postoperatively on oral antibiotics to complete atotal of four days of antibiotics. He had no complications during his stay.Cecal diverticulosis represents only 1–2% of diverticular disease inNorth America with the prevalence much more common in Western countries.What do we already know about this clinical entity?Cecal diverticulosis represents 1–2% of diverticulardisease in North America. It presents as right lower quadrant pain with noclear distinctive features.What makes this presentation of disease reportable?In this case cecal diverticulitis acted as a mimic to acute appendicitis,leading to laparoscopy that revealed a perforated cecaldiverticulum.What is the major learning point?The real diagnostic tools were the history and serial physical exams,which prompted us to not rely on a falsely reassuring workup.How might this improve emergency medicine practice?Even in the age of sensitive imaging tools, we should not entirely relyon those diagnostic means to make a final diagnosis. History and physicalexam remain the major tools of a good physician.In the ED evaluation and workup of abdominal pain, the emergency physician shouldconsider a perforated cecal diverticulum in an atypical presentation of right lowerquadrant pain with findings that do not completely fit the clinical picture ofappendicitis. It is imperative to involve a surgeon early. Finally, CT is helpfulbut not 100% sensitive; the clinician should keep in mind the importance ofa physical exam and of clinical reevaluation to help guide final diagnosis andtreatment.


Pulmonary sclerosing pneumocytoma (PSP) is a rare benign or low-grade malignant tumor, but it has the potential to present with multiple lesions, lymph node metastasis, extra-pulmonary metastasis, recurrence and even cause death. Herein, a case of PSP that was huge, presented with multiple lesions and had lymph node as well as extrapulmonary metastases  is reported for the first time. This patient was also the first one to die of respiratory and circulatory failure caused by the PSP tumor and its metastases which compressed the mediastinal tissue. In 2015, the World Health Organization (WHO) officially renamed Pulmonary Sclerosing Hemangioma (PSH) to Pulmonary Sclerosing Pneumocytoma (PSP) and classified it as a lung adenoma , 4. MoreThe patient was a 25-year-old male who was admitted to our hospital  in August 2019 with “a mass in the right lung, which had persisted for more than 10 years and had chest tightness as well as shortness of breath for more than 1 month.” In July 2009, the patient presented in a local hospital due to “fatigue” and chest Computed Tomography (CT) showed a shadow of multiple masses in the right lung, as shown in Although the chest CT suggested that the patient most likely had a malignant tumor in the lung, a clear pathological diagnosis was not made even after several medical examinations. Subsequently, a CT-guided lung biopsy was performed, where the tumor tissue was punctured multiple times at different tumor sites and the pathological diagnosis was confirmed to be PSP . It is nAdditionally, Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography (18F-FDG PET/CT) examination during hospitalization showed a malignant tumor in the right lung, with multiple metastases in the hilar and mediastinal lymph nodes, several bones, liver, and intra-abdominal lymph nodes .Color Doppler echocardiography also revealed mild pulmonary hypertension  and pericardial effusion, about 10 mm from the top of the right atrium.12/L (4-5.5/L), Hematocrit (HCT) was 0.285 (0.36-0.5), white blood cell count was 8.6 × 109/L (4 ~ 10 × 109/L) and the percentage of neutrophils was 76.3% (50 ~ 70%). In addition, the concentration of glutamic pyruvic transaminase was (ALT) 41.2 U/L (0 ~ 40 U/L), that of glutamic oxaloacetic transaminase was (AST) 16.5 U/L (0 ~ 40 U/L), Total Protein (TP) concentration was 56.8 g/L (60.0 ~ 85.0 g/L), albumin concentration was (ALB) 21.6 g/L (35.0 ~ 55.0 g/L) and Globulin (GLOB) concentration was 35.2 g/L (20.0 ~ 35.0 g/L). Moreover, the patient had normal concentrations of tumor markers such as the Prostate Specific Antigen (PSA), Carcinoembryonic Antigen (CEA), Alpha-fetoprotein (AFP), cytokeratin 19 fragment, Neuron-specific Enolase (NSE), and Carbohydrate Antigen 125 (CA125).Laboratory examination revealed that the level of Hemoglobin (HGB) was 84 g/L (120-160 g/L), the Red Blood Cell (RBC) count was 4.21 × 10Moreover, whole-exome sequencing was performed on the patient's PSP tumor tissue and the matched normal sample from the peripheral blood was analyzed through the Switching Mechanism at the 5' end of RNA Transcripts (SMART) method. Notably, activation of the AKT1- p.E17K pathway was observed in 31 genes .After hospitalization, physical examination showed that the patient was emaciated, had poor mental health, assumed a semi-recumbent position and had mild shortness of breath. The patient's temperature was 36.6°C, his pulse was 108 bpm, respiratory rate was 30 breaths per minute and blood pressure was 128/71 mmHg. In addition, a 15 cm surgical incision scar was seen on the right thorax and the chest cavity was symmetrical without deformity. Lung auscultation also revealed no respiratory sound on the right lung and the left lung was normal.Notably, the patient had undergone “right pulmonary bulla resection” surgery for the right pneumothorax, 19 years earlier and had no history of other diseases.Furthermore, the patient was diagnosed with: (1) Malignant PSP of the right lung with multiple metastases in the blood vessels, mediastinum, liver, bone, and celiac lymph nodes; (2) Hypoproteinemia; (3) Anemia; (4) Pulmonary hypertension; and (5) Pericardial effusion.After admission, the patient was placed on oxygen, administered with supplements and received supportive treatment. His symptoms improved slightly following this treatment. However, his physical condition was not suitable for surgical treatment and he chose to leave the hospital. Six months later, the patient died of breathing difficulties and cachexia.PSP is a rare tumor with unique histological characteristics, which can be summarized as “Two cell types, four patterns.” In addition, PSP typically comprises of a mixture of four architectural patterns  and two cell types, namely; cuboidal surface cells and rounded cells with either an eosinophilic or clear cytoplasm , 16. TheIn 1986, the Japanese scholars, Tanaka et al.  and SpenCurrently, the mechanism of PSP metastasis in patients is not yet fully understood although a few suggestions have been put forward. For instance, Suzuki et al.  suggesteBefore the patient was diagnosed with PSP in this case, it was speculated that he had adenoma. This may have been due to the limited specimen and at the time, only the papillary area was observed. However, repeated puncture of the lung lesions under CT-guided lung biopsy provided a large amount of tumor tissue and a final diagnosis of PSP was achieved. In addition, analysis of the chest CT images revealed multiple metastases in the mediastinal tissue, liver, abdominal cavity, bone, lymph nodes, and even vascular invasion. Examination through 18F-FDG PET/CT also showed a huge mass in the right lung. Additionally, there was an increase in FDG uptake and the Standardized maximum Uptake Value (SUVmax) was 8.21. There was also an increase in FDG uptake by multiple nodules in the remaining lobe of the right lung and the SUVmax was 3.91. Moreover, the hilar and mediastinal lymph nodes were enlarged, FDG uptake had increased unevenly and the SUVmax was 10.39. Multiple round nodules were also seen in the liver, the boundary was not clear, FDG uptake had increased and the SUVmax was 4.48. Additionally, both the L11 and S1 vertebrae had small flaky low-density lesions, there was an increase in FDG uptake and the SUVmax was 4.58. Furthermore, there were nodular FDG high-uptake areas on the left scapula, left iliac bone and left the ischial body, respectively, and the SUVmax was 3.67. There were also multiple, enlarged lymph nodes in the abdominal cavity, FDG uptake had increased and SUVmax was 6.79. These lesions indicated a significant increase in SUV uptake. Notably, given the patient's medical history, main diagnosis and information from previous literature , 26, theCurrently, no study has reported on PSP related deaths and most scholars still believe that PSP is a benign lesion with good prognosis and is not fatal , 28. HowIn summary, this was the first reported case of PSP that presented with multiple lesions, was large and had lymph node as well as extrapulmonary metastases . The tumor had the largest diameter ever reported and was accompanied by vascular invasion. This was also the first patient to die of respiratory and circulatory failure caused by the PSP tumor and metastases compressing the mediastinal tissue. This outcome differed from previous reports on PSP and therefore requires more attention from healthcare practitioners. Moreover, further research is required to identify more clinical features and mechanisms of metastasis in PSP.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.WZ and XW designed the entire study. WZ, YL, and DS conducted patient clinical management and sample collection. JC, YC, and KS analyzed the data. WZ wrote the manuscript. All the authors read and approved the final version of the manuscript for submission.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


A 60-year-old Japanese woman was admitted because of the polycystic mass with right flank pain localized in the upper portion of the right kidney. Right nephrectomy was performed because the mass lesion had continuously increased in size over the past 10 years. A surgical specimen showed histology consistent with a mixed epithelial and stromal tumor, which is closely related to multilocular cystic nephroma, and was diagnosed by a defined capsule between the cystic mass lesion and normal renal tissue by CT and MRI, and histology. Localized renal cystic disease that does not have a capsule was excluded from differential diagnosis. Localized renal cystic disease (LRCD) has been reported; cysts form in only one kidney and not in other organs, and patients do not have a family history of cystic disease, unlike autosomal dominant polycystic kidney disease (ADPKD). Two types of LRCD have been reported: segmental renal cystic disease, which involves just one part of a kidney, and unilateral renal cystic disease (URCD), which involves a whole kidney , 2. HereA 60-year-old Japanese woman was admitted to our hospital for evaluation of a polycystic mass in the upper portion of the right kidney and right flank pain. The mass lesion was first identified when the patient was aged 50 years, and regular computed tomography (CT) scans had shown that the lesion had gradually enlarged over time. The patient had no cysts in the liver, left kidney, or the remaining area of the right kidney. This patient does not have a history of long-term estrogen replacement.3/μL; total protein, 7.2 g/dL; albumin, 4.4 g/dL; serum urea nitrogen, 27 mg/dL; serum creatinine, 1.3 mg/dL; and estimated glomerular filtration rate(eGFR), 35.0 ml/min/1.73 m3. Urinary protein excretion was 0.1 g/day and the urinary sediment contained no erythrocytes or casts.On admission, the patient was 154 cm tall and weighed 70 kg. Her blood pressure of 150/82 mmHg; and her temperature, 36.6 °C. She had a history of hypertension, dyslipidemia, hyperuricemia, primary hyperparathyroidism, and Graves’ disease, but she had no family history of cystic disease. Laboratory findings were as follows: white blood cell count, 7000 /μL; hemoglobin, 14.0 g/dL; platelet count, 30.2 × 10CT showed a polycystic mass in the upper portion of the right kidney that measured 17 × 15 × 12 cm and had well-defined margins. CT angiography revealed large, elongated, well-developed renal arteries around a large mass lesion but did not detect any hypervascular stain, i.e. it did not indicate the presence of a malignant tumor. The abdominal aorta was shifted and elongated towards the left as a result of the polycystic mass , multicystic dysplastic kidney, and low-grade cystic renal cell carcinoma were proposed as possible differential diagnoses.Our nephrology team decided to remove the right kidney because the mass lesion had continuously increased in size over the past 10 years and the patient was experiencing pain.Macroscopic examination of the resected kidney showed a polycystic mass measuring 17 × 15 × 12 cm , and CK7 as markers of the loop of Henle and the distal convoluted tubule; and CK34βE12 as a marker of the collecting duct [The epithelial component was positive for CD138, E-cadherin, CK7, and CK34βE12 but negative for CD10, EMA, ER, and PR. The stromal element was positive for CD10 but negative for CK7, ER, and PR  but not LRCD.3.Nine years after nephrectomy, the patient still has good renal function, with a creatinine level of 1.53 mg/dL and eGFR of 27.8 ml/min/1.73 mWe encountered a case of MCN/MEST/REST but not LRCD. Information necessary for differential diagnosis of these disease was examined. Slywotzky et al. summarized 18 reported cases of URCD  : MRI shoIn the differential diagnosis of URCD, physicians also need to consider multilocular cystic nephroma (MCN). MCN is a rare, benign cystic lesion of the kidney with a bimodal age distribution, i.e. it occurs in both infants and adults. Patients usually present with nonspecific symptoms, but abdominal pain, hematuria, and urinary tract infection are common in adults. CT shows a multicystic architecture with well-defined margins. MRI usually shows a hypointense signal on T1-weighted sequences and a hyperintense signal on T2-weighted sequences .According to the World Health Organization classification of renal neoplasms, adult-type MCN is grouped histologically with the mixed epithelial and stromal tumors (MEST) . The terIn conclusion, the surgical specimen of this patient’s polycystic mass localized in the upper portion of the right kidney diagnosed MEST histologically. MEST is close related to MCN, which has been confirmed to have a defined capsule between the cystic mass lesion and normal renal tissue by CT and MRI, and by renal histology.


Few cases have been reported of colorectal cancer with inferior mesenteric artery (IMA) branching abnormalities; therefore, the lymphatic flow in such cases remains unknown. We report the first case of locally advanced rectal cancer in which the IMA arose from the superior mesenteric artery (SMA) in which we achieved to visualize the lymphatic flow.A 65-year-old woman complaining of bloody stools was investigated in our hospital and suspected with rectal cancer. Colonoscopy and abdominal enhanced computed tomography (CT) revealed a circumscribed, localized ulcerative tumor in the rectum. 3-Dimensional contrast-enhanced computed tomography (3D-CT) showed that the IMA arose from the SMA. The patient was diagnosed with rectal cancer  and laparoscopic low anterior resection was performed. The sigmoid colon was resected using the medial approach. Only the plexus of the colic branch of the lumbar splanchnic nerve was observed at the site where the root of the IMA usually exists and showed interruption of the indocyanine green (ICG) fluorescence-illuminated lymphatics. The root of the IMA was ligated, and Japanese D3 lymphadenectomy was performed, preserving the accessory middle colic artery. All fluorescent lymph nodes were resected. The pathological diagnosis was pT4aN1aM0 stage IIIb. The patient’s postoperative course was uneventful. Adjuvant chemotherapy was administered, and the patient was recurrence-free at 1.5 years after surgery.We were able to perform safe and appropriate surgery oncologically, despite abnormal vascular anatomy, due to preoperative identification using 3D-CT and intraoperative navigation using ICG administration. Japanese D3 lymphadenectomy is a standard treatment with respect to the extent of lymphadenectomy performed for advanced colorectal cancer in Japan. Similarly, complete mesocolic excision (CME) with central vascular ligation (CVL) is established in Europe. Both are achieved by ligating the root of the inferior mesenteric artery (IMA), for example left-sided advanced colorectal cancer , 2. TherWe report the first case of locally advanced rectal cancer in which the IMA arose from the superior mesenteric artery (SMA) diagnosed by preoperative 3-dimensional contrast-enhanced computed tomography (3D-CT), and visualization of the lymphatic flow was achieved by the indocyanine green (ICG) navigation.A 65-year-old woman presented with a complaint of bloody stools. The attending physician suspected a diagnosis of rectal cancer, and the patient was referred to our hospital for further investigation. On physical examination, her abdomen was soft and no mass was felt during palpation. Laboratory data on admission showed that the levels of tumor markers in the blood, including carcinoembryonic antigen and carbohydrate antigen 19-9, were within normal ranges. Colonoscopy revealed a circumscribed, localized, ulcerative tumor, 9 cm from the anal verge and 7 cm from the dentate line Fig. A. No enlThis case was enrolled in the following studies which aimed to investigate the usefulness and limitations of treatment by laparoscopic colorectal surgery using the detection rate of ICG fluorescence markings with near-infrared light . This stIn this patient, the ICG solution was administered into the submucosal layer at two points around the tumor using a colonoscope 6 days before the laparoscopic colorectal surgery. Using a 26-gauge needle, 0.2 ml of normal saline solution was injected into the submucosal layer to form a submucosal elevation at two sites on the colonic wall opposite the tumor. Subsequently, 0.1 ml (ICG dose: 0.5 mg) of an ICG solution was injected into the elevated areas [A small incision was made in the umbilical region, and surgery was initiated using a 5-port approach. The intra-abdominal cavity was observed, and no liver metastases or peritoneal dissemination were observed. The sigmoid colon was resected using the medial approach. The root of the IMA was not present at abdominal aorta; instead, at this site, we observed the presence of a plexus of the colic branch of the lumbar splanchnic nerve Fig. A. The ICOur department at the Sapporo Medical University is a part of a multi-institutional prospective observational study to validate the quality of pathological diagnosis, especially circumferential resection margin (CRM), by transverse slicing of semi-opened rectal specimens (UMIN000031735), and this case was included in this study. The tumor diameter was 80 mm × 30 mm. The pathological diagnosis was pT4aN1aM0 pStage IIIb because of serous membrane involvement of in the anterior wall and the presence of a positive lymph node (1/26 lymph nodes)  were administered, and the patient was recurrence-free 1.5 years after surgery.Variations or anomalies in the IMA anatomy are rare. In the report by Kakihara et al., abdominal angiography in 3182 patients revealed occasional IMA bifurcation anomalies, consisting of a defect in one patient (0.03%), running anomaly due to visceral retroversion in two patients (0.06%), and bifurcation from the root of the SMA in two patients (0.06%) . SimilarUsing “variation” and “inferior mesenteric artery” as search keywords, we identified six papers describing anatomical abnormal branching of the root of IMA in autopsy cases reported on PubMed from 1968 to 2021 . HoweverIt has also been reported that preoperative 3D-CT is informative and helpful for surgeons to assess the vascular anatomy, especially the vascular variability around the SMA , 16, 17.ICG fluorescence imaging has been widely used to evaluate the blood and lymphatic flow for colorectal cancer surgery –21. The Tandler , 24 repoBased on the above, we suspected that the IMA originated from the abdominal aorta and was accompanied by lymphatics during fetal life; however, the root of the IMA disappeared during the developmental process, and lymphatics ascending along the IMA persisted even after the disappearance of the root of the IMA and did not get redirected towards the SMA. Although the extent of lymphadenectomy is not established in such cases, ligation at the bifurcation of IMA was considered to be oncologically appropriate in this case. Since surgical cases of colorectal cancer with abnormal branching of the IMA are rare, further studies are required to validate these findings.We reported the first case of locally advanced rectal cancer with abnormal branching of the IMA from the SMA in whom safe and appropriate surgery was performed oncologically due to preoperative identification using 3D-CT and intraoperative navigation using ICG administration.


Intestinal obstruction considered to be one of the most common surgical presentation. Adhesions secondary to previous operations, hernias, neoplasms, inflammatory bowel disease, intussusception, or volvulus are the usual causes of intestinal obstruction but bezoar can presents in 0.4-4%. Bezoar can be trapped in different locations throughout the gastrointestinal tract and it can be solitary or multiple lesions.This is a 37-year-old male, known case of diabetes mellitus, Presented to the Emergency Department complaining of generalized abdominal pain for 2 days duration. Associated with abdominal distention, fever, nausea, vomiting and obstipation. There was a history of persimmon intake. Unremarkable past surgical history. On examination, He was tachycardic, other vital signs were within normal. Abdominal examination showed abdominal distention and Sluggish bowel sound. Abdominal X-ray revealed multiple air-fluid levels. An abdominal CT scan with IV contrast revealed an intra-luminal mass in the ileum and intra-gastric mass with suspicious of bezoars. He underwent exploratory laparotomy, gastrostomy to remove intra-gastric bezoar, and enterotomy to remove the ileal bezoar.Intestinal obstruction is considered to be the most common complication of this entity; other possible complications include gastric ulcer, gastritis, and gastric perforation. Due to limitations of endoscopy and barium enema in the diagnosis of bezoar, Abdominal CT-scan is considered to be the gold standard in the diagnosis. The management of phytobezoar can be either conservative or surgical, depends on the lesion size and location.Although intestinal obstruction secondary to bezoar is rare, multiple levels of gastrointestinal obstruction should raise the suspicion of bezoar. •Bezoar can be trapped in different locations throughout the gastrointestinal tract and it can be solitary or multiple lesions.•The predisposing risk factors for bezoar formation include previous gastric surgery, gastroparesis, peptic ulcer disease, or psychiatric illness.•Abdominal CT-scan considered the gold standard in the diagnosis for bezoar.•The management of phytobezoar can either be conservative or surgical depends on the lesion size and location. Bezoar can be trapped in different locations throughout the gastrointestinal tract and it can be solitary or multiple lesions. We are reporting a case of an adult male with intestinal obstruction secondary to multiple gastrointestinal phytobezoars, managed surgically.The work has been reported in line with the SCARE 2020 criteria .2This is a 37-year-old male, known case of diabetes mellitus, Presented to the Emergency Department complaining of Generalized abdominal pain for 2 days duration. Associated with abdominal distension, fever, nausea, vomiting and obstipation. He denied any history of eating from outside, but he gave a history of persimmon intake. Unremarkable past surgical history. His family, drug and psychological history were unremarkable. On examination, He was tachycardic, other vital signs were within normal. Abdominal examination showed abdominal distention and Sluggish bowel sound. Digital rectal examination showed an empty rectum. Laboratory investigations showed leukocytosis 16 thousands per cubic milliliter, other labs were unremarkable. Abdominal X-ray revealed multiple air-fluid levels. Abdominal CT scan with IV contrast revealed dilated small bowel loops reaching up to 3.8 cm with intraluminal mass in the ileum measuring 3.4 × 2.2 × 3.3 cm  and intr3Bezoar is a rare entity, usually defined as a mass formed by undigested foreign materials that accumulated and trapped inside the gastrointestinal tract . There a[The predisposing risk factors for bezoar formation include pyloric dysfunction, decrease gastric motility and decrease acid secretion secondary to previous gastric surgery, peptic ulcer disease, pyloric stenosis, gastrointestinal amyloidosis, poor mastication, dental implants, inadequate fluid intake, high amounts of indigestible fibers, psychiatric illness, or gastroparesis secondary to diabetes mellitus, hypothyroidism, or connective tissue diseases . Erzurum.Usually, the patients present with abdominal pain, epigastric fullness, nausea, vomiting, dyspepsia, anorexia, or symptoms of bowel obstruction . The incDue to limitations of endoscopy and barium enema in the diagnosis of bezoar in an emergency sitting which can worse symptoms in complete bowel obstruction, increase risk perforation, and worse peritonitis in the presence of perforation. Abdominal CT-scan is considered to be the gold standard in the diagnosis with a sensitivity of 90% and a specificity of 57% . In a stThe management of phytobezoar can either be conservative, endoscopic or surgical depends on the lesion size and location. The conservative options may include dissolution by Coca-Cola, papain, L-cysteine, metoclopramide, cellulose, pineapple juice, sodium bicarbonate, hydrochloric acid, pancrelipase, pancreatin or 1–2% zinc chloride. Endoscopic option is considered in the removal of small-size gastric bezoar. Surgical options include laparoscopic or open removal, which is considered for large-size gastric or small bowel bezoar refractory to conservative management, or patients presenting with emergency conditions i.e. (bleeding or peritonitis). During surgical intervention, some surgeons prefer to mobilize the bezoar distal to the ileocecal valve using milking technique but this technique may cause serosal, mesenteric laceration or distal obstruction , other s4Although intestinal obstruction secondary to bezoar is rare, multiple levels of gastrointestinal obstruction should raise the suspicion of bezoar.This study did not receive any funding.IRB approval is not needed for Case reports in our center.Written informed consent was obtained from the patients for publication of this case report, A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not applicable.Dr. Neamat Alturki.Dr. Abdullah G. AlSahwan.Not commissioned, externally peer-review.Abdullah G. Alsahwan: Main Author of the paper, study concept and design, data collection, data interpretation, literature review, drafting of the paper, final review of the manuscript.Ameen Almarhoon, Jihad Alsafwani, Hanan Alsahwan: study concept and design, data collection, data interpretation, literature review, drafting of the paper, final review of the manuscript.Neamat Alturki: Supervisor; Treating physicians of the patient, study concept and design, data collection, data interpretation, literature review, drafting of the paper, final review of the manuscript.No declarations of interest.


We present a case and discuss the management of a posterior cloacal variant not as yet described in the literature. A 5-week-old infant presented to our institution with a posterior cloacal variant and transposition of the clitoris and labia. After initial radiological investigations, staged operative intervention was performed over a 1-year period. This included an initial laparotomy (with drainage of hydrocolpos and formation of a colostomy), a left ureteric reimplantation and a posterior sagittal anorectoplasty due to a rectoperineal fistula. The child is under continued long-term follow-up by our specialist pediatric surgical team. The first case of a posterior cloaca was reported in 1991.A 5-week-old infant was referred to our tertiary institution from a rural setting with an initial suspicion of a disorder of sexual differentiation due to abnormal external genitalia.Initial examination revealed a clinically well infant with fused superior labia and an inferiorly displaced clitoris. The child was noted to have a thickened pubic symphysis measuring ∼3 cm . ExaminLaboratory investigations confirmed a 46XX karyotype with a normal endocrine work-up. Renal dysfunction was noted with an elevated urea and creatinine but with normal electrolytes. An abdominal ultrasound revealed a small echogenic right kidney, a bulky echogenic left kidney with gross left hydroureteronephrosis, and a complex pelvic fluid collection suspicious for hydrocolpos. X-rays demonstrated a normal sacrum.A urinary catheter was placed into the urogenital sinus and clear urine was drained. The renal dysfunction improved with the administration of intravenous fluids.Ultrasound was not able to confirm whether the balloon of the catheter was indeed within the bladder or within the vagina. As a result, the decision was made to take the child for an examination under anesthesia and perineal stimulation. The anal opening was confirmed to be significantly anterior to the sphincter complex. A laparotomy was therefore performed to fashion an end colostomy and drain the hydrocolpos. At laparotomy, a normal uterus and fallopian tubes were identified and the vagina appeared thickened and hypertrophic without significant fluid content. A formal vaginostomy was therefore not a feasible option as the vagina was not dilated enough to reach the abdominal wall. Hence, a vaginotomy was done and a catheter was inserted via the common channel into the vagina under direct vision.Unfortunately, the child developed wound sepsis and sheath dehiscence and required a relook laparotomy and sheath closure 4 days after the initial surgery. The child recovered well and a repeat abdominal ultrasound 10 days postoperatively revealed resolved hydrocolpos but residual left-sided hydronephrosis.A cystovaginoscopy was performed at 3 months of age. The common channel measured 1.5 cm. The urethra measured 3.5 cm with a normal bladder neck. An adequate vagina with a normal cervix was also demonstrated. On entering the bladder, only the right ureteric orifice was visualized.A mercaptoacetyltriglycine renogram was completed which revealed a nonfunctional right kidney, with slow drainage from a hydronephrotic left kidney. A voiding cystourethrogram demonstrated Grade 5 vesicoureteric reflux on the left.Repeat cystoscopy with a STING  procedure was therefore attempted—this was however unsuccessful as the left ureteric orifice was unable to be visualized. Throughout this time, the child was regularly seen by the pediatric renal unit who monitored her renal function which continued to remain normal.At 11 months of age, the patient underwent an uncomplicated left cross trigonal Cohen's ureteral reimplantation and recovered well postoperatively. The plan was to concomitantly perform a posterior sagittal anorectoplasty (PSARP). This was however postponed due to a severe nappy rash.At 1 year of age, the patient underwent a PSARP along with a total urogenital mobilization and4.The patient recovered well postoperatively. She was discharged with anal dilations being performed by her caregiver. She is followed up regularly. Her colostomy closure was successfully performed at the age of 20 months (delayed due to the coronavirus pandemic). She is under continued renal follow-up and plans for a genitoplasty to correct the transposition of the clitoris and labia will be made going forward.Posterior cloacas and posterior cloacal variants are incredibly rare congenital malformations. They were initially referred to as type A and type B malformations,Our patient displayed many similarities to previously published cases. Urological anomalies have been shown to be present in 88% in reported cases. Of these, 34% have had unilateral reflux and 31% have had a single/nonfunctional kidney.Pubic symphysis hypertrophy is almost universally present. This frequently necessitates carving of the pubic bone and cartilage during reconstructive surgery.Although there are documented cases of posterior cloacal variants associated with abnormal external genitalia,The surgical recommendation based on the literature is to, wherever possible, avoid mobilization of the anus (if the anus is correctly placed within the sphincter). This is to avoid damaging the muscles and the nerve supply which would in turn give the child the best outcome in terms of continence. Transperineal or transanorectal total urogenital mobilization is therefore the preferred approach.As yet, there is no definitive evidence/guidelines that exist for the management of posterior cloacal variants.The surgical management of posterior cloacal variants and their associated anomalies is complex and is best handled by a specialist pediatric colorectal surgical team. The presence of hydrocolpos always needs to be suspected, investigated, and treated. A PSARP should only be performed in the case the anus is outside of the sphincter complex. Clinical and surgical management should be tailored to individual patients according to the anatomy of the perineum and presence of associated anomalies.


Primary hepatic squamous cell carcinoma (SCC) is a rare malignancy with aggressive clinical features. This is the first case report of a primary hepatic SCC diagnosed by endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA), which is a reliable and safe procedure for the histopathological diagnosis of liver lesions, even if the percutaneous approach is difficult due to ascites or hypervascularity at the puncture site. A 52-year-old man presented to the emergency department of a tertiary referral hospital with right upper quadrant abdominal pain and abdominal distention. Given the laboratory data, a diagnosis of spontaneous bacterial peritonitis (SBP) was made. Concurrently, an abdominal computed tomography (CT) scan revealed an 8 cm hypodense mass with delayed peripheral enhancement in the left hepatic lobe and paraaortic and perihepatic lymphadenopathy. As persistent ascites precluded percutaneous liver biopsy, we performed EUS-FNA of the liver mass, and the obtained specimen showed SCC. As otorhinolaryngological consultation and whole-body investigations, including chest CT, upper and lower endoscopy, and positron emission tomography CT, were all unremarkable except for the liver lesion and lymph nodes, a diagnosis of primary hepatic SCC with systemic lymph node metastasis was made. After treatment of SBP with antibiotics, we initiated chemotherapy concurrent with radiation therapy, adapted to his liver function. Radiation and three cycles of chemotherapy were not effective as the disease progressed, as seen on the follow-up CT scan, and the patient died of hepatic failure on the 134th day after diagnosis. In conclusion, EUS-FNA was a reliable method for tissue sampling in liver malignancies, particularly in selected patients with contraindications for percutaneous biopsy. Histopathologic examination of biopsy specimens obtained from atypical liver masses is known to provide essential diagnostic information, thus significantly improving patient care . OccasioμL; platelet count, 92,000/μL; alkaline phosphatase, 358 U/L; serum albumin, 2.6 g/dL; prothrombin time, 14.6 s; international normalized ratio, 1.27; alpha-fetoprotein, 19.5 ng/mL; carbohydrate antigen, 19-9, 132 U/ml; and protein induced by vitamin K absence-II of 16%. Ascitic fluid analysis showed a white cell count of 10,800/μL (56% neutrophils). Blood and ascitic fluid cultures were negative. With regard to imaging studies, an abdominal ultrasound showed a hypoechoic, mosaic-patterned mass in the lateral segment of the left lobe of the cirrhotic liver  and bilateral hepatic lobe lesion with multiple lymph node metastases was made. For SBP, IV antibiotics (ceftriaxone 2 g/day) were started. Based on the imaging findings, the radiologist suspected the formation of mixed type hepatocellular carcinoma or intrahepatic cholangiocarcinoma mass. On transabdominal ultrasound, the hepatic lesion was exposed at the hepatic surface without intervening normal hepatic component and ascites was noted at the hepatic surface. These factors precluded percutaneous liver mass biopsy because of the high risk of bleeding. On the other hand, EUS view from the gastric station showed no significant amount of ascites . EUS-FNAAs we initially suspected metastatic SCC of the liver, an extensive workup including chest CT, esophagogastroduodenoscopy, colonoscopy, and otorhinolaryngological examination was performed, all of which showed negative findings. Given the above results, a diagnosis of primary SCC of the liver was made.2) and fluorouracil (500 mg/m2). Given the patient's comorbidity of cirrhosis, the dose of the chemotherapy was reduced. In total, three cycles of chemotherapy were administered. Radiation with three cycles of chemotherapy was not effective as the disease progressed, as seen on the follow-up CT scan, and the patient died of hepatic failure on the 134th day after diagnosis.After 2 weeks of antibiotic treatment for SBP, the patient's liver function gradually improved to a Child–Pugh's score of 6. The patient was fully informed that the diagnosis was primary SCC of the liver and was in agreement with the adjusted chemoradiation therapy. The patient was started on radiation therapy (50 Gy) for the liver mass and paraaortic lymph nodes, followed by chemotherapy comprising cisplatin .Primary hepatic SCC is rare. Zhang et al.  enlistedPercutaneous liver biopsy remains an essential tool for the evaluation of focal liver masses . Sensiti3 or a PT-INR of above 1.5 should force clinicians to consider suspending the procedure to avoid bleeding [The contraindications of percutaneous liver biopsy are thrombocytopenia and coagulopathy . It is sbleeding . With rebleeding . Additiobleeding .p=0.841).In contrast, a prospective study reported the diagnostic accuracy of EUS-FNA in hepatic masses to be between 82% and 100% and the sensitivity, specificity, NPV, and PPV to be 94%, 100%, 78%, and 100%, respectively . AdditioIf the liver tissue sampling is aimed at a “benign-malignant assessment,” a 22-gauge aspiration needle can obtain an adequate specimen for diagnosis . HoweverIn the present case, we decided to perform EUS-FNA as the liver masses were mainly located in the left lobe and the visibility from the gastric station on EUS provided us with an excellent view of the liver mass without worrisome vasculature or ascites as compared to the percutaneous view. Based on clinical studies which showed that both 19-gauge and 22-gauge needles attain equivalent feasibility in histological diagnosis  and thatGiven the rarity of the primary hepatic SCC, adenosquamous cell carcinoma was a differential diagnosis in the present case; it was less likely from the histopathological evaluation that the specimen was negative for individual cell keratinization, intracellular bridges, glandular differentiation, or intracellular and intraluminal mucin accumulation, which were the characteristic features of adenosquamous cell carcinoma .Primary hepatic SCC is rare and is known for its aggressive clinical course. We concluded that EUS-guided fine-needle biopsy was a reliable method for tissue sampling in liver malignancies, particularly in selected patients with contraindications for percutaneous biopsy.


Imaging findings have a prominent role in early and correct identification of ovarian dysgerminoma, the most common ovarian malignant germ cell tumor (OMGCT). Despite Computed Tomography (CT) is widely used, Magnetic Resonance Imaging (MRI) has proved to be superior in adnexal masses characterization. Limited data and small series are available concerning MRI aspects of dysgerminoma.From January 2012 to December 2018, a database of solid ovarian masses was retrospectively reviewed. Eight patients with histologically proven pure ovarian dysgerminoma and complete imaging available were identified and analyzed. Imaging findings were evaluated separately by two radiologists expert in female genito-urinary MRI.MRI findings of a lobulated, purely solid, encapsulated mass with hyper-intensity of lobules and hypo-intensity of septa on T2w images contribute to differentiate dysgerminomas from other ovarian neoplasms. Dysgerminoma, the ovarian counterpart of testicular seminoma, is the most common OMGCT, accounting for 1-2% of primary ovarian neoplasms and 32.8-37.5% of all OMGCTs . TherefoThe onset symptoms include acute or chronic pain, abdominal distension, menstrual irregularities and infertility. This tumor is commonly associated with elevated levels of lactate dehydrogenase (LDH) and occasionally with elevated beta human chorionic gonadotrophin (beta-HCG). Elevations in alpha-fetoprotein (AFP) and Cancer Antigen 125(CA-125) are less common .The main imaging findings of dysgerminoma include a well-marginated solid lobulated mass with fibrovascular septa. However, this feature is unspecific for dysgerminoma . DespiteThe aim of the present study is to describe MRI characteristics of dysgerminoma in a consecutive series of patients, with proven histological diagnosis of this rare tumor.From January 2012 to December 2018, a database of solid ovarian masses histologically proven was retrospectively reviewed and imaging characteristics of ovarian dysgerminomas confirmed at histology were collected. Eight patients with ovarian dysgerminoma and complete imaging were identified and analyzed.Demographic, clinical and laboratory data of these patients are reported in Table n = 3; 38%), infertility , irregular menstruation , abdominal distension , acute pelvic pain . Four patients (50%) complained of two of those symptoms. In 3 patients (37.5%), a palpable mass was detectable at physical examination.The median age was 22.6(13-39) years. One patient was asymptomatic; the remaining patients demonstrated the following symptoms: chronic pelvic pain (One patient was pregnant at the time of diagnosis. Seven patients were nulliparous (87.5%), one was multiparous (12.5%). None was previously submitted to abdominal-pelvic surgery. One patient had uretero-pelvic junction syndrome with right hydronephrosis.Serum tumor markers showed an elevated LDH value in 6 patients (75%), elevated CA-125 in one patient (12.5%), elevated beta-HCG in one case (12.5%), normal values of AFP in all cases.After the admission, all the patients were submitted to trans-abdominal sopra-pubic US completed with Doppler ultrasonography, with the evidence of a pelvic mass. Two patients with acute pelvic pain underwent urgent abdomino-pelvic CT. Finally all the patients underwent MRI.CT examinations were performed using a 64-detector-row scanner. After plain scanning, images were acquired at 30s-60s-90s after contrast medium administration.MR exams were performed on 6 h fasting patients, pre-medicated with Hyoscine-N-butylbromide antispasmodic agent, via 1.5 superconductive magnet  with phased array surface coil. The sequences included followed the ESUR recommendations for MRI of indeterminate adnexal masses . The conMR images were retrospectively evaluated separately by two radiologists with 15 years of pertinent clinical experience in female imaging, who were blinded to clinical, histopatological, endoscopic and other imaging findings.The following characteristics were collected: maximum diameter of the neoplasm (cm), morphology  internal structure , the presence of capsule, interlobular septa, papillary projections, parietal irregularities, calcifications (yes/no). The lesion signal intensity was compared to that of muscle tissue and judged as hypo, iso or hyper-intense and homogeneous or heterogeneous. Endolesional areas of hypo-intensity on T1-weighted images(T1w) and hyper-intensity on T2w  were classified as cystic. Areas of iso-intensity on T1w and mild hyper-intensity on T2w were classified as solid. Areas of hyper-intensity on T1w, although punctiform, with a corresponding hyper or hypointense signals on T2w, were classified as hemorrhagic.The analysis included the evaluation of DWI signal with corresponding ADC values and the enhancement patterns of the mass and septa on T1w contrast enhanced images. Furthermore, the invasion of adjacent structures, lymphnodes number and size and the presence of endoperitoneal fluid effusions were evaluated.Proper surgically resected specimens were submitted to an accurate hystological and cytological examination performed by a gynecologic pathologist.All the neoplasms were unilateral. The mean diameter of the lesions was 11.9 cm (3.8-23.5 cm).At US examinations, lesions appeared as solid masses with non-homogeneous eco-structure, with multiple endolesional hyperechoic components and vascularized septa. At CT scan the masses appeared as solid, lobulated, isodense to muscle tissues, with a moderate enhancement of wall and of endolesional fibrovascular septa Fig. .Fig. 1OThe morphology of the lesions was lobulated in 5 patients (62.5%), roundish in 2 patients (25%) and ovular in one patient.The internal structure of the neoplastic masses was considered purely solid in 7 patients (87.5%) and mixed cystic-solid in one patient (12.5%). In 6 patients (75%), both interlobular fibrous septa and an identifiable thin and regular capsule were observed.No solid papillary mural projections, parietal irregularities or calcifications were detected Fig. .Fig. 2On = 8 pts.,100%), suggesting a solid appearance. On T2w sequences, septa were clearly identifiable as linear hypo-intensities in 6 (75%) cases. In 6 lobulated masses (75%) the signal was described as homogeneous; in 2 cases (25%) as heterogeneous.MR signal of the lesions was characterized by a predominant iso-intensity on T1w sequences and a moderate hyper-intensity on T2w (2) on the solid areas were 0.81 × 10 - 3 mm2 . High signal intensity within the solid component on DWI and low signal intensity in corresponding ADC maps were observed in 8 patients (100%)  in the other one. Mean ADC values, calculated from three b-values  Fig. . No contIn 2 cases (25%), a small amount of fluid effusion was observed in recto-uterine Douglas pouch.In 2 patients (25%) lymph-nodes with short axis > 1 cm were documented in the right groin and in the left internal iliac region, respectively.MRI findings are reported in Table Radiological aspects of ovarian dysgerminoma have poorly discussed in current literature. The largest multicenter radiological series have evaluated sonographic characteristics of dysgerminoma in 21 patients .MR features of dysgerminoma were previously described in small series as solid masses divided into lobules by hypointense or isointense on T2w fibrovascular septa, which showed a marked enhancement after Gd-DTPA injection –10. TsubIn the most of our cases dysgerminoma appeared as a purely solid encapsulated mass with hyper-intensity of lobules and linear hypo-intensity of septa on T2w imaging. This sign reflects the hyper-cellularity of the lobules and the fibrous nature of the septa and may help to differentiate dysgerminomas from other OMGCTs , 13. TheOur findings may also contribute to differentiate dysgerminomas from solid non-OMGCTs tumors including fibrotecoma, Brenner’s tumor and granular cell tumor. The first two are hypo-intense on T2w and the latter is frequently characterized by hemorrhagic endolesional areas, hyper-intense on T1w images . MoreoveHigh signal on DWI within the solid mass with corresponding low ADC values was reported in all our cases and reflect the hypercellular environment. Nevertheless, high DWI signal and low ADC values may be observed in false-positive non-malignant lesions with high cellular density . Thus, DThe present study has some limitations, such as the retrospective design, that however represents a limitation typical for this very rare pathology, the small number of patients and the subjective interpretation of images.In conclusion, our series is one of the largest available in literature including only one pure subtype of OMGCT, which is in its own very rare, analyzed by the same group of radiologists and pathologists and collected in a single center. In our series, multiparametric MR imaging is able to express its full potential in the detection and characterization of ovarian masses. Furthermore, MRI may contribute to differentiate dysgerminoma from others OMGCTs, solid non-OMGCTs and epithelial ovarian tumors. Concurrently, MRI plays an important role in local and extra-ovarian staging of dysgerminoma, in the preoperative planning and in the detection of recurrencies.


Rapunzel syndrome is an extremely rare condition seen in adolescents or young females with psychiatric disorders consisting of a gastric trichobezoar with an extension within the small bowel. The delays in diagnosis are common since in its early stages, it is usually asymptomatic. We report the case of a 13-year-old girl admitted in our clinic for abdominal pain, anorexia, and weight loss. The clinical exam pointed out diffuse alopecia, a palpable mass in the epigastric area, and abdominal tenderness at palpation, the patient weighing 32 kg. The laboratory tests showed anemia. The abdominal ultrasound showed a gastric intraluminal mass with a superior hyperechoic arc. The upper digestive endoscopy revealed a mass formed by hair, mucus, and food occupying the gastric cavity with the extension into the duodenum confirming the diagnosis of Rapunzel syndrome. The giant trichobezoar of 511 g, measuring 17 × 7 × 6.5 cm with a tail of approximately 3 cm, was successfully removed through laparotomy. Although rare, Rapunzel syndrome must never be forgotten as a differential diagnosis for digestive symptoms since its early detection hinders the occurrence of further complications. The word “bezoar” originates from the Arabic word “bedzehr” or the Persian “padzhar,” which means “protecting against a poison,” since historically, bezoars from animal guts were useful as antidotes to poisons and, nowadays, as part of traditional Chinese medicine , 2. In hRapunzel syndrome was described for the first time in 1968 by Vaughan et al., and it is almost exclusively seen in young females , 6, 7. MIn terms of treatment, multiple options were reported, among which are medical treatment and enzymatic degradation, endoscopic, laparoscopic, or laparotomic removal .The aim of this case report was to increase awareness of Rapunzel syndrome as a rare cause of weight loss, anorexia, abdominal pain, and anemia in children.Written informed consent was obtained from the patient's mother, the minor's legal guardian, for the publication of any potentially identifiable images or data included in this article.We report the case of a 14-year-old female admitted in our clinic for weight loss (approximately 5 kg within the last month), anorexia, and abdominal pain. The anamnesis revealed that the patient was diagnosed with mild developmental delay without an identifiable cause despite her management by a neuropsychiatrist. The patient's socioeconomic level was appropriate, and we found no evidence of child neglect.The clinical exam at the time of admission pointed out diffuse alopecia, a palpable mass in the epigastric area, and abdominal tenderness at palpation. The patient weighed 32 kg.Based on the presenting concerns and the clinical findings, we raised the suspicion of trichotillomania and trichophagia resulting in a possible trichobezoar. Initially, the patient denied to have ingested her hair, but after a thorough anamnesis, she admitted to have this habit, but rarely. The laboratory tests showed anemia . The abdominal ultrasound showed a gastric intraluminal mass with a superior hyperechoic arc. The upper digestive endoscopy revealed a mass formed by hair, mucus, and food , 2 occupThe size of this mass did not allow us to remove it endoscopically, and therefore, the patient was referred to the pediatric surgery, and the trichobezoar was surgically removed by median supraumbilical laparotomy with longitudinal gastrotomy , 4. We fThe patient presented a postsurgical favorable evolution tolerating well the oral progressive feeding, without requiring nutritional support, but the main burden consists in a proper long-term psychiatric monitoring for an effective prevention of both trichotillomania and trichophagia. Thus, once she fully recovered after the surgical intervention, she was referred to the Pediatrics Neuropsychiatry Clinic where an oral treatment with alprazolam was initiated. In terms of anemia, the laboratory tests at 1 month after the surgical intervention revealed considerably improved parameters: hemoglobin 10 mg/dl, hematocrit 36.6%, and medium erythrocyte volume 70.9 fl. Nevertheless, we decided to introduce oral iron supplementation for 6 weeks.Trichobezoars, the most common bezoars in humans, usually occur in preadolescent or adolescent girls with psychiatric comorbidities or developmental delay , 17, 18.Anamnesis is a key factor in raising the suspicion of hair consumption and its relevance relies especially on the physician's communication skills  since thThe diagnosis is usually suspected as a result of thorough anamnesis and clinical exam, but it requires imaging tools for its confirmation. Upper digestive endoscopy is considered as the gold standard for diagnosis in trichobezoars revealing a mass formed by hair, which appears black due to the effect of the gastric acid on the hair protein, mixed with mucus and food , 27. We The usefulness of medical treatment and enzymatic degradation is limited to small trichobezoars, but usually fail in proving their effectiveness. Studies reported that Coca Cola might be useful for the degradation of gastric phytobezoars, but not trichobezoars . EndoscoRapunzel syndrome is an extremely rare cause of digestive symptoms, such as abdominal pain, anorexia, or weight loss in children. Delays in diagnosis are relatively common taking into account that it is rarely considered in the differential diagnosis of children with digestive symptoms. Increased awareness regarding risk factors for trichotillomania and trichophagia, such as developmental delay and anemia as a cause of pica, is crucial for the early diagnosis. Laparotomy remains the treatment of choice in the case of large bezoars. Thus, albeit rare, Rapunzel syndrome must never be forgotten since its early detection hinders the occurrence of further complications.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author/s.Written informed consent was obtained from the patient's mother, the minor' legal guardian, for the publication of any potentially identifiable images or data included in this article.CM, LM, and MS conceptualized and designed the study, drafted the initial manuscript, and reviewed and revised the manuscript. CM performed the gastroscopy. MS designed the data collection instruments, collected data, carried out the initial analyses, and reviewed and revised the manuscript. ZD and RM performed the surgical intervention. All authors approved the final manuscript as submitted and agreed to be accountable for all aspects of the work.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.