Background: Cholangiocarcinoma (CCC), a rare tumor arising from the viscera, has a poor prognosis. Although CCC is prone to metastasis, spread to the cranium and spine is exceedingly rare. Treatment for metastatic disease is palliative, with total resection of the primary lesion the only cure. We describe a case of metastatic CCC to the spine and cranium treated with surgical resection.Case Report: A 61-year-old male with a history of hepatitis C with liver transplant and incidental discovery of CCC presented with gradually increasing back pain. Physical examination revealed a palpable nontender mass in the parieto-occipital area. Computed tomography survey of the spine and head revealed mixed sclerotic and lytic lesions of the T9, T11, L2, and L5 vertebral bodies, a lytic lesion on the T6 vertebral body, and a 1.4-cm lesion in the right occipital calvarium. The patient underwent right occipital craniotomy for excisional biopsy of the calvarial mass with gross total resection and immunohistochemical confirmation of CCC. The patient was started on gemcitabine chemotherapy and radiation therapy for spinal metastases. Three months later, the patient died from metastatic disease complications.Conclusion: To our knowledge, only 6 cases of cranial CCC have been reported, and only 2 reported mixed cranial/spinal involvement. We report a rare case of CCC metastasis to the spine and cranium that was treated with surgery, chemotherapy, and radiotherapy. CCC should be considered an exceedingly rare etiology with treatment options aimed solely at palliation. This case supplements the existing literature to inform medical and surgical decision-making. Metastasis to the skull is typically asymptomatic but may cause severe disability with compression of the dural sinuses,19 disturbance of the cranial nerves,20 epidural hematoma,23 and expansion of the tumor secondary to intratumoral hemorrhage.24 Metastasis to the spine may present as neck pain,11 back pain,15 periscapular pain,3 or even complete neurologic syndrome.25 On cytologic examination, smears reveal a glandular arrangement of crowded clusters and sheets of malignant cells with large nuclei and prominent nucleoli.11 Immunohistochemistry of metastatic CCC cells shows strong immunopositivity for cytokeratin (CK)-7 and CK-19, AE1/AE3, CAM 5.2, MOC-31, and polyclonal carcinoembryonic antigen (pCEA).26 Equally important is the immunonegativity for thyroid transcription factor-1 (TTF-1), prostate-specific antigen (PSA), and CK-20 that helps to rule out metastasis from the lung, prostate, and colon, respectively.11Definitive diagnosis of CCC metastasis to the spine and cranium is reserved for pathology and immunohistochemistry, as radiologic imaging and clinical presentation are nonspecific. Despite poor specificity, positron emission tomography (PET) offers significant advantages as a method of detecting metastases,31 yet only 6 cases of metastasis to the cranium have been reported,32 and only 2 of those cases coinvolved the spine and cranium.12 We present a case of combined CCC metastasis to the cranium and spine.While documented evidence of CCC spine metastasis was previously limited, case series published between 2001 and 2018—largely from Thailand and South Korea—demonstrate hundreds of cases of CCC metastasis to the spine,A 61-year-old male presented with a 1-month history of increasing back pain that he associated with muscle spasm. The patient reported that the pain started gradually and had progressed to a 7/10 on the pain scale by the time he presented to the hospital. The patient's significant medical history included a liver transplant 9 months prior secondary to chronic hepatitis C. At the time of the patient's transplant, pathologic review of the explant was notable for a 0.6-cm pure hepatocellular carcinoma and a 0.7-cm mixed cholangiocarcinoma with hepatocellular carcinoma components, both confined to the right lobe of the liver. No adjuvant treatment was undertaken at the time of transplant, as both lesions were without lymphovascular invasion. Physical examination showed a palpable nontender mass in the parieto-occipital area but was otherwise unremarkable.Computed tomography (CT) imaging of the spine revealed mixed sclerotic/lytic lesions involving T9 and T11 Figures  and 1B aThe patient underwent a right occipital craniotomy with stealth navigation for excisional biopsy of the mass. Intraoperative visualization and resection of the tumor revealed a 1.4-cm red-brown mass that extended through both tables of the skull. The mass was adherent with but not attached to the dura and was easily scraped away. Special care was taken to clean and coagulate the involved dura. Resection was completed with bone edges free of tumor involvement, and the lesion was fully contained within the craniotomy excision. Pathology results demonstrated a metastatic adenocarcinoma virtually identical to the cholangiocarcinoma component seen in the patient's liver explant 9 months prior . Immunoh2 and gemcitabine 1,000 mg/m2 infusions the same day as his craniotomy; he had a second and final chemotherapeutic treatment 1 week postoperatively. One month following his operation, the patient began a course of radiation to a dose of 30 Gy in fractions of 3 Gy that targeted the metastases to the thoracic spine and the L5 vertebral body. The patient continued follow-up with neurosurgery following his operation; however, he succumbed to his illness and died from complications of the metastatic disease 3 months postoperatively.The patient was started on cisplatin 25 mg/m26 but present with nonspecific symptoms25 and imaging.12 Metastasis to the spine and/or cranium is rare; however, a small number of cases with spinal and cranial metastasis have been documented in the literature in case reports (15 and in retrospective clinical studies and review articles (32Metastases from CCC display unique cytologic and immunohistochemical properties that aid in diagnosis reports 3,10-15 aarticles .27-3215 followed by the first reported case of skull metastasis by Miyamoto et al in 2007.12 Including our own patient, there are currently 9 known cases of CCC metastasis to the spine in the United States,31 with hundreds more reported in Eastern Asia, and 7 cases of metastasis to the cranium32 with 3 combined cases of spine/cranium metastasis including our own.12 In these cases, the average patient age was 60.1 years with a range of 38 to 82 years and a female:male ratio of approximately 1:1 (Yeh et al described the first incidence of CCC metastasis to the spine in 2001,tely 1:1  and 2.3 This increase in incidence is supported by studies from 2010 and 201333 that correlated prolonged overall survival and progression-free survival with modern management strategies, particularly the use of cisplatin and gemcitabine in palliative situations. This theory is supported by the fact that 6 patients, including our own, had prolonged latency periods between the original presentation of CCC and metastasis to the spine and/or cranium. However, 4 patients presented with metastasis concomitant with the discovery of their primary neoplasm.15Although it is tempting to believe that spinal/cranial metastasis from CCC is a novel presentation of an existing tumor entity, we posit that the historic absence of immunohistochemistry to aid in past diagnoses may have obscured previous cases in the literature. Other sources argue that the increasing incidence of these less common metastatic sites may be attributable to an increase in overall survival of patients secondary to the advent of multimodal therapies.10 Of these, surgical excision is most commonly employed for symptomatic lesions, provided the lesion can be removed with little risk of morbidity or mortality.35 The goal of surgery in metastatic disease is to offer local control and symptomatic relief, with no expected change in overall prognosis.35 When lesions are too extensive or too difficult to resect, radiotherapy may be applied.10 Radiotherapy with or without chemotherapy was found to provide pain relief in up to 90% of patients with symptoms of short duration.36 Chemotherapy and hormonal therapy are considered to be the least effective treatments overall10; however, in studies from 2011 and 2017, the use of gemcitabine and cisplatin together demonstrated positive effects on systemic lesions and potential clinical improvement.37Pending definitive diagnosis with cytology and immunohistochemistry, the 4 treatments for metastatic CCC are surgery, radiation, chemotherapy, and hormonal therapy.4 However, improvements have also been found in metastatic disease. In CCC with metastasis, median survival is currently 7 to 12 months, with a 2013 study from Ramírez-Merino et al showing even longer overall survival and progression-free survival with newer treatment regimens.6 Still, the exact impact of cranial and/or spinal metastases on disease progression, morbidity, and mortality has yet to be established. The authors anticipate a negative prognostic effect secondary to the neurologic sequelae described in this report.As mentioned previously, the use of Whipple duodenopancreatectomy in extrahepatic CCC has increased the overall 5-year survival rate from 12% to 28% in local disease.CCC is a rare visceral tumor, not classically associated with spread to the spine and/or cranium. CCC metastasis is indistinguishable from more common primary tumor types by clinical or radiologic findings, making preoperative diagnosis impossible without the assistance of pathologic study. CCC metastasis to the cranium or spine has indeterminate impact on the disease course and prognosis, although our own experience demonstrates a poor prognostic outcome. Upon diagnosis, treatment of metastatic sites is considered solely palliative but may be combined with curative measures. This case supplements the existing literature to inform medical and surgical decision-making.


Sarcomas are a rare entity with heterogeneous clinical behavior. We herein present the case of a giant retroperitoneal dedifferentiated liposarcoma and aim to remind the clinical, histological, and therapeutic features of this rare tumor.An 80-year-old patient was complaining of significant abdominoscrotal swelling. On physical examination, a 25-cm swelling extending from the right iliac fossa to scrotums, was observed. CT scan revealed a retroperitoneal fatty mass with necrotic areas. Ultrasonography-guided biopsy was carried. Pathological report demonstrated a dedifferentiated sarcoma. An en bloc resection of the tumor was performed through a right iliac incision extended to the scrotum. After one year of follow up, the patient had a 30-cm local recurrence. After complete resection, the patient died two days after the procedure due to neurological distress.‘Giant’ liposarcomas over 20kg are extremely rare. CT-Scan and MRI are very useful for defining their size and limits. The resection of a retroperitoneal sarcoma of remarkable size is a challenge for the surgeon owing to the anatomical site that makes it hard to obtain safe margin and to the adherences with the contiguous organs. Dedifferentiated histologic subtypes and negative surgical margins are associated with poor prognosis. This explains the high rate of local recurrence after surgical excision.In rare cases, retroperitoneal dedifferentiated liposarcomas can extend through the inguinal canal to the scrotum. Surgical resection obtaining negative margins, remains the curative treatment that reduces the risk of recurrence. Careful follow-up to detect early recurrence is essential for optimal care. The genitourinary tract location represents only 5%. Approximately 12–15 % of soft tissue sarcomas are arising from the retroperitoneum. Among histologic subtypes, liposarcoma is the most common (30–50 %) [2An 80-year-old patient was referred to our emergency department complaining of asthenia, vomiting, and significant abdominoscrotal swelling. He had no history of chronic disease or surgery. The initial swelling had started in the scrotum two years earlier following a benign scrotal trauma and thereafter abdominal distention was recognized and increasing gradually but neglected by the patient.On physical examination, the patient showed a severe nutritional deficiency with muscle and adipose tissue loss. The upper extremities and the face demonstrated cachexia, and the abdomen was severely distended, owing to an enlarged painful swelling with ill-defined margins measuring 25 cm extending from the right iliac fossa to scrotums. The diameter of the scrotal tumor was 50 cm with edematous skin, and the penis had sunk into the mass . Slight Laboratory investigations revealed a severe hypochromic and microcytic anemia , and a high serum level of lactic dehydrogenase (550 U/L).The CT scan revealed a heterogeneously enhancing fatty mass with solid nodules and necrotic areas, with craniocaudal extension of over 40 cm from the retroperitoneum to the inguinoscrotal region, compressing the abdominal aorta and the intestines to the left side of the abdomen. The testicles were hypo trophic and repressed. No local infiltration or distant metastasis was detected . An ultrThe case was presented at a multi-disciplinary team meeting including the urologists, general surgeons, pathologists and radiologists. Surgical management option was selected by the multidisciplinary team basing on the tumor resectability assessment and the ability of the patient to tolerate the surgery. The procedure was promptly planned and an en bloc resection of the tumor was performed through a right iliac incision extended to the scrotum. The main difficulty was to guarantee clear margins sparing major vessels or adjacent organs.The histological examination of the tumor confirmed the diagnosis of dedifferentiated liposarcoma (grade III of FNLCC). Surgical limits were clear , Fig. 4.The postoperative course was uneventful. The patient was discharged 10 days after the intervention. After one year of follow up, the patient was complaining of constipation and emerging abdominal swelling. Abdominal and pelvic Computerized Tomography scan showed a recurrent retroperitoneal mass measuring 30 cm compressing the intestines. The mass was completely resected through the same incision. The histological examination confirmed a local recurrence of a liposarcoma. The patient died two days after the procedure due to neurological distress.3Primary retroperitoneal tumors are rare and represent about 0.1−0.2% of all malignant tumors and about 15 % of soft tissue sarcomas in adults . The preLiposarcomas are often characterized by an indolent growth in the retroperitoneal space and may cause compression of the adjacent structures or migrate into the scrotum mimicking an inguinoscrotal hernia. In this situation, ‘giant’ liposarcomas over 20 kg are extremely rare. Only a few cases are reported in the literature. Many studies suggested that tumor size impacts the prognosis frequently using a cut-off value of 10 cm . A recenLiposarcomas are divided into four basic histological categories: myxoid liposarcoma, round cell liposarcoma, well-differentiated liposarcoma, and pleomorphic liposarcoma . The undCT and MRI appearances of liposarcomas can vary according to the combination of these histologic subtypes. CT-Scan and magnetic resonance imaging (MRI) are very useful for defining the size, consistency, and relation between the tumor and the adjacent tissue, and they also allowed us to detect residual tumor and recurrences. The CT imaging of the undifferentiated sarcoma demonstrates a heterogeneous lipogenic mass. In the case of diagnostic doubt and presence of recurrence, Magnetic Resonance Imaging (MRI) could detect with high sensitivity the satellite locations of the main lesion .Surgical treatment aims to achieve negative resection margins, removing any adjacent organs and structures involved. The resection of a retroperitoneal sarcoma of remarkable size is a challenge for the surgeon owing to the anatomical site and the absence of an anatomically evident vascular lymphatic peduncle that makes it hard to obtain safe margin and to the adherences with the contiguous organs. Therefore, the retroperitoneal liposarcoma shows a high rate of local recurrence after surgical excision. Dedifferentiated histologic subtypes and negative surgical margins are associated with poor prognosis .In the literature, 10 %–50 % of retroperitoneal sarcomas are resectable ,13, and A surgical excision followed by radiotherapy (60–70 Gy) resulted in a significant reduction in local recurrence compared with surgery alone . In anotAn aggressive surgical excision remains the main treatment of retroperitoneal sarcomas, and long-term follow-up examinations are also considered to be important . Tumor e4Dedifferentiated liposarcomas originated from the retroperitoneum are fully malignant tumors in which identification of dedifferentiated areas is crucial to establish an accurate prognosis. In rare cases, they can extend through the inguinal canal to the scrotum. A surgical resection obtaining negative margins, remains the curative treatment that reduces the risk of recurrence. A careful follow-up to detect early recurrence is essential for optimal care.The authors report no declarations of interest.No source to be stated.Ethical approval is not necessary for case report in our locality.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Mahjoubi Zied: Writing the manuscript, literature review, final approval of the manuscript and follow up.Walid Zakhama, Yassine Binous: Surgeons performing the intervention.Manel Njima: Pathologist performing the histological examination of the tumor.Walid Zakhama, Aymen Sakly, Aymen Mnasser: Final approval of the manuscript.Corresponding Author: Mahjoubi Zied.Mahjoubi Zied is the Guarantor.Not commissioned, externally peer-reviewed.


Primary laryngeal aspergillosis in an immunocompetent host is a rare entity. On the other hand, pulmonary tuberculosis (PTB) among healthcare workers in a tuberculosis endemic zone with high risk of exposure is not uncommon but may be underdiagnosed especially when masquerading as another disease. In this report, we are presenting a 45‐year‐old physician who presented with chronic dry cough, hoarseness of voice, and progressive vocal fatigue. Fibreoptic laryngoscopy (FOL) showed whitish patches on both vocal folds and he was initially diagnosed with laryngeal aspergillosis following histopathological examination. As there was no significant improvement on antifungal treatment, we re‐evaluated the case and, on further investigation, concomitant PTB was detected. Patient responded to category‐I anti‐tubercular drugs with complete recovery. A 45‐year‐old physician presented with dry cough and progressive vocal fatigue and was diagnosed as primary laryngeal aspergillosis. Further work‐up revealed coexistent pulmonary tuberculosis. Aspergillus colonization, which mostly occurs in advanced stages of bronchopulmonary infection in immunocompromised hosts. Aspergillosis primarily involving the larynx in an immunocompetent individual is very rare [The larynx is an unusual site for ery rare . Cough aery rare . CoexistAspergillus were found in necrosed tissue , and inhaled bronchodilator, his symptoms were partially relieved but he developed hoarseness of voice a few days later. A chest X‐ray was normal. Due to persistent symptoms, he consulted an otorhinolaryngologist and was referred for fibreoptic laryngoscopy (FOL). FOL showed whitish patches over anterior third of both vocal folds Fig. . Fungal sue Fig. . No granMycobacterium tuberculosis was detected in BAL GeneXpert with rifampicin sensitive. The final diagnosis of PTB with primary laryngeal aspergillosis was made. Treatment with category‐I anti‐TB drug was started after resolving hepatitis and he was advised to stop ICS and practice prevention measures at the workplace. He took voriconazole for a total of four weeks. His condition gradually improved with resolution of cough, fever, vocal fatigue, and hoarseness of voice. Follow‐up bronchoscopy done one year later showing complete resolution of vocal cord lesion  patients. There was no history of diabetes mellitus, malignancy, or immunodeficiency. HIV serology was negative. He is non‐smoker, had allergic rhinitis, mild persistent asthma, and took low‐dose inhaled fluticasone (500 μg/day) irregularly. On examination, he looked ill and chest examination revealed normal findings. Chest X‐ray and computed tomography (CT) scan were unremarkable Fig. . Sputum ion Fig. .Aspergillus, having very little pathogenic capability in a healthy host. The organism gains access to the respiratory mucosa via inhalation of spores with subsequent invasion causing necrosis, ulceration, haemorrhage, and thrombosis. The larynx is an unusual site of Aspergillus infection, which may be involved in advanced stage of bronchopulmonary aspergillosis in an immunosuppressed individual.Aspergillosis is a fungal infection caused by the genus Primary laryngeal aspergillosis in immunocompetent individual is extremely rare and often mimics premalignant or malignant condition of the larynx. Globally, only 38 cases have been documented over the last five decades . PatientAspergillus colonization [Our patient had a history of using occasional ICS for bronchial asthma. A substantial portion of steroid may be deposited in the larynx, leading to a breach in local immunity of the throat, alteration of the normal flora of the larynx, or cause erosion of the laryngeal mucosa, allowing nization . Althougnization . We alsonization . As the nization . Globallnization . So our nization . So, it Our patient is unique in perspective of harbouring two diseases—primary laryngeal aspergillosis and PTB. To the best of our knowledge, this is the first reported case having both diseases in the same patient. Primary laryngeal aspergillosis in immunocompetent patient is an emerging trend and HCWs are at increased risk of TB infection. This curious case had dual pathologies. Rational suspicion leads us to a definite diagnosis and favourable outcome.Appropriate written informed consent was obtained for publication of this case report and accompanying images.


Melanotic neuroectodermal tumor of infancy is a rare, rapidly growing, painless, pigmented neoplasm with neural crest derivation. It usually occurs during the firstyear of life and there is a prominent predilection for the maxilla. The purpose of the present report is to describe additional case of melanotic neuroectodermaltumor of infancy of maxilla in a 6-month-old infant male. The treatment included surgical excision with safe margins. No attempt was made for immediate graftingof the surgery site due to high proliferation rate of tissues and self-renewal during infancy. The facial growth was normal and the surgical cleft was tightly closed.Due to the rarity of tumor, essential knowledge on characteristics of this lesion would contribute to a proper diagnosis and benefit treatment planning. The melanotic neuroectodermal tumor of infancy (MNTI) is a rare pigmented tumor that usually arises during the first year of life. The lesions are more common in the maxilla  - 4. ItA sixmonthold male was referred to the private oral pathology center  for evaluation of rapidly growing, painless expansion in the left maxilla. His parents noticedthe first sign of expansion four months ago without any systemic signs such as fever or malaise. His delivery and development was normal. Upon oral examination,a firm, projecting mass measuring 2.5×2.5×2cm was detected on the left maxillary anterior alveolar ridge extending to the hard palate and displacing the upper lip.It was not tender on palpation. The overlying mucosa was intact and bluish in color . The decThe occlusal radiograph revealed a large radiolucent lesion with ill-defined borders encompassed unerupted tooth buds. With provisional diagnosis of MNTI,the excisional biopsy was performed. Under general anesthesia, a partial maxillectomy with removal of three involved deciduous teeth was done. During surgery,an inner brown-colored aspect of the lesion was observed, which confirmed the initial diagnosis. The lesion was well demarcated from adjacent bone and separated easily.Palatal bone defect was dressed with Surgicell and fixed with absorbable sutures. It is noteworthy that the surgery site was not grafted due to high proliferation rateof tissues and self-renewal during infancy. The gross specimen showed a mass with black cut surface surrounded by a white rim . MicroscThe facial growth has been normal and the palatal cleft was closed, but due to loss of several tooth buds, frequent dental follow-up examinations are essential and this situation may cause facial development problems in future.MNTI has neural crest origin due to neuroblastic appearance of cells, presence of neurosecretory granules in electron microscopy evaluation, and elevated levelsof vanillyl mandelic acid (VMA) in urinary excretion1. However, many cases have been described with normal levels of VMA. These mOn gross examination, it seems dark blue and there is a suggestion of pseudo-encapsulation because of reactive bone formation  . MNTI iThe alveolar and tubular structures are lined by large cuboidal epithelioid cells, with pale abundant cytoplasm, vesicular nuclei, and granules of brown melanin pigment. The second cell type is neuroblastic or similar to mature lymphocyte and it contains small, round cells with hyperchromatic nuclei and little cytoplasm. These cells are frequently surrounded by the larger pigmented cells  .Microscopic differential diagnosis in this age group contains metastatic neuroblastoma, Ewing sarcoma, rhabdomyosarcoma, primitive neuroectodermal tumor, and lymphoma  , 5.No histologic findings and biological indicators have been found to highlight the clinical manner  - 5, 8Generally, the prognosis of MNTI is excellent  . SurgicThe recurrent MNTI appears to grow more destructively, to have a propensity for indistinct borders, and it might demonstrate osteoid formation  . AdjuvaAll appropriate patient consent forms were obtained from his parents.In our case, MNTI indicated a benign course, and the surgical cleft was closed without any grafting that indicate high proliferation rate in neonates.


Internal herniation of small intestine in the lesser pelvis alongside iliac vasculature is a rare occurrence. Skeletonization of iliac vessels during pelvic lymph node dissection (LND), as part of surgical staging or treatment of patients with uterine, ovarian or urogenital cancer, is a strict prerequisite for orifice formation.A 68-year-old woman presented at the emergency department with complaints of constipation for the last 3 days and acute-onset abdominal pain, nausea and vomiting since few hours. She had a history of laparoscopic hysterectomy, bilateral salpingo-oophorectomy and para-aortic and pelvic LND 7 years ago. A distended abdomen with diffuse tenderness on palpation was noted. A CT scan demonstrated bowel obstruction secondary to an incarcerated hernia underneath an elongated right external iliac artery. During an emergency exploratory laparotomy, the incarcerated bowel was reduced and the hernial orifice closed with a running suture. The patient had an uneventful postoperative period and was discharged on the fifth postoperative day.This rare internal hernia can manifest with non-specific symptoms of small bowel obstruction at any given point after index surgery, sometimes even after several years free of complaints. Contrast-enhanced computed tomography is the method of choice for fast and reliable diagnosis and helps in planning the necessary emergency laparotomy.This life-threatening complication adds to the current controversy of pelvic and para-aortic lymphadenectomy in patients with endometrial cancer. Primary closure of peritoneal defects should be considered to potentially prevent internal hernias, especially when elongated iliac vessels are present.The online version of this article (10.1007/s00404-020-05724-x) contains supplementary material, which is available to authorized users. Internal hernias originate from passage of intestinal structures, usually small intestine, through congenital or acquired apertures within the boundaries of the peritoneal cavity. Clinical complaints result from obstruction of intestinal loops, which may end up necrotic due to incarceration if left untreated. Similar to external hernias, they oftentimes present with signs of severe passage disorder: acute or subacute onset abdominal pain, nausea and vomiting as far as fecal vomiting. However, they can as well remain asymptomatic for a long time or present with intermittent or constant vague epigastric pain. Left paraduodenal herniation of jejunal loops through Landzert’s fossa is the most common form of internal hernia .Herniation of small intestinal loops in the lesser pelvis alongside the iliac vessels is a rare entity that has only been reported in eight cases in English-language literature today Table . RemovalLatency between surgery and onset of abdominal symptoms was variable in all prior cases published, ranging from 3 months to 4 years. We report the first case of a patient who underwent surgery for endometrial cancer, had an unusually long latency until onset of symptoms and featured a yet unreported orifice of herniation between the external iliac artery (EIA) and vein (EIV).2) presented with a few-hours history of acute-onset abdominal pain, nausea and vomiting in our clinic’s internal medicine emergency department. She reported symptoms of constipation for 3 days prior to arriving at our clinic. Her medical and surgical history was empty apart from treatment of endometrioid endometrial adenocarcinoma with deep myometrial invasion (pT1b pN0 (0/30), L0, V0, R0; FIGO IB; Grade 2). She had undergone laparoscopic hysterectomy with bilateral salpingo-oophorectomy (H-BSO), para-aortic and pelvic LND as well as adjuvant vaginal brachytherapy over 3 weeks  7 years ago in 2011. There was no abdominal surgery of any cause or abdominal complaints in the meantime.A 68-year-old severely obese lady . First of all, antibiotic treatment for nitrite negative urinary tract infection was initiated due to leukocyturia (500/µl) and microhematuria in urine strip testing. A return-flow enema was performed due to the reported constipation.Clinical examination revealed a distended abdomen with diffuse tenderness on palpation of the hypogastric region and slight muscular guarding. Initial abdominal X-ray revealed no classical signs of mechanical ileus. An abdominal sonography reported no pathological findings. Lab test indicated systemic infection with raised C-reactive protein (62 mg/l) and procalcitonin levels (0.1 ng/ml) as well as elevated leukocyte count  under antibiotic therapy and in absence of pain relief despite the enema, a diagnostic contrast-enhanced computed tomography (CT) of the abdomen was performed the next day (Online Resource 1).CT revealed mechanical SBO in the lower right abdomen with significant dilation, wall edema and contrast-enhancement of distal loops of jejunum and proximal loops of ileum. The cause was identified to be a herniation of the proximal ileum between the EIA and EIV in the right iliac fossa with consecutive significant stenosis of the bowel lumen, abrupt change of caliber and total collapse of the terminal ileum Fig. . StrikinThe same day, the decision for exploratory laparotomy was made to confirm the radiologic findings. Intraoperatively, the parietal peritoneum presented macroscopically reddened and showed vascular injections in the right upper and lower abdomen. A conventional abdominal lavage using 10 L of Ringer’s lactate solution was performed to remove ubiquitous cloudy ascites and to decrease bacterial counts in diluted peritoneal fluid. Inspection of the right iliac fossa revealed a distended, gangrenous ileum loop caused by incarceration due to an internal herniation of the ileum between the skeletonized EIA and EIV Fig. . ThorougThe incarcerated loop was removed from the hernial orifice and a segmental resection of 50 cm ileum was performed approximately 200 cm distally to the ligament of Treitz with primary side-to-side jejuno-ileal anastomosis. A running suture with resolvable suture material (PDS Gauge 5–0) was used for primary closure of the hernial orifice to prevent future re-herniation.The patient’s aftercare was uneventful with rapid recovery of abdominal symptoms and inflammatory parameters. She was discharged 5 days after surgery in good general condition with normal bowel activity and antibiotic treatment (Ceftriaxone/Metronidazole) completed 8 days after surgery.The most common cause of postoperative bowel obstruction is adhesions. Different forms of internal or abdominal wall hernias are much rarer conditions, each with a challenging diagnosis and high mortality rate, exceeding 50 % in some series . RegardiStrangulation of small bowel loops by an external or common iliac artery is an even rarer, but nevertheless important differential diagnosis for ileus in patients with history of surgical procedures that involve pelvic LND. The removal of lymphatic tissue and the resulting defect in the peritoneum frequently create a significant free space between the arching retroperitoneal vessels, which is increased even further in patients with severe kinking and elongation of pelvic arteries . Several. Lymphatic metastases are one of the most important prognostic factors and LND may be beneficial by identifying lymph node positive patients in need of adjuvant treatment, including radiotherapy and chemotherapy. However, over 30 years since their first recommendation by the FIGO, the role of systematic lymph node staging, as well as its therapeutic benefits, remain highly controversial. In 2008–2009, findings of two large independent randomized trials suggested that pelvic LND merely adds increased morbidity without unambiguous influence on survival outcomes [Furthermore, this rare complication adds to the controversy of pelvic and para-aortic LND as part of the surgical staging in patients with endometrial cancer, as recommended by the “Fédération Internationale de Gynécologie et d’Obstétrique” (FIGO) since 1988. According to their 2018 cancer report, as a minimum, any enlarged or suspicious lymph nodes should be removed in all patients. For high-risk patients , complete pelvic LND and resection of any enlarged para-aortic nodes is recommended . Lymphatoutcomes , 8. In 2outcomes . In womeoutcomes , 12, sugoutcomes .However, in patients with early-stage non-endometrioid (type II) endometrial carcinoma and carcinosarcoma which tend to metastasize in pelvic and para-aortic lymph nodes more frequently, systematic pelvic and para-aortic LND independently and significantly prolonged the patients’ survival .The concern about a potential overtreatment of many patients , who oftentimes are burdened with an adverse surgical risk profile due to obesity, old age and other co-morbidities led to sentinel node mapping being increasingly utilized for endometrial cancer staging in the recent years  .In 2019, a meta-analysis of six comparative studies displayed non-inferiority of sentinel node mapping to standard LND in detection of para-aortic nodal involvement and recurrence rates while even suggesting a possible superiority in detection of pelvic lymph node involvement . AnticipRight now, systematic pelvic and para-aortic LND is recommended for high-risk patients . In our case, it was performed for Grade 2 endometrioid endometrial adenocarcinoma (FIGO IB), where it is neither absolutely necessary nor dissuaded.SBO due to herniation beneath skeletonized pelvic vasculature is an odd complication that further adds to the morbidity of patients after pelvic LND. This uncommon complication may be considered as differential diagnosis in patients who present with signs of bowel obstruction and have a positive history of lower pelvic surgery including operative treatment of prostate, bladder, ovarian, cervical or endometrial cancer. The latency between the index operation and symptomatic herniation shows a wide range of variation according to former case reports and may be even misleadingly long, as our case demonstrates. This circumstance reinforces the weight of a thorough and reflective anamnesis allowing for a shortened diagnostic process and urgent surgical intervention, which both are imperative to treating patients with this particular complication. While this hernia was initially overlooked in our patient in an ultrasound examination due to its uncommon location, contrast-enhanced computed tomography, being the diagnostic method of choice, showed unmistakable results.Clinicians should be aware of the existence of rare iatrogenic hernias through artificial orifices due to the inevitable separation of pelvic vessels during lymphadenectomy.Supplementary file1 (MP4 18210 kb) Online Resource 1 – CT scan (video) Contrast-enhanced CT-Scan performed on a Somatom Definition Edge Single Source CT Scanner  in a portal-venous phase using a bolus-tracking technique after i.v. contrast agent injection ; axial orientation. There is evidence of a mechanical SBO in the lower right abdomen with significant dilation of the upper GI-tract including a massively fluid-filled stomach. In the right iliac fossa, a herniation of the proximal ileum between the elongated, arching EIA and EIV can be seen with an abrupt change of caliber and total collapse of the terminal ileum. An extensive streaky infiltration of visceral fat, enlarged lymph nodes and significant amounts of diffuse ascites can be detected with emphasis on the right paracolic gutter, caecum and perihepatic space, consistent with the diagnosis of peritonitis.Below is the link to the electronic supplementary material.


Pigmented villonodular synovitis (PVNS) is a less common but known cause of joint pain in the adult population. PVNS in pediatric patients is even more rare, with only case reports of occurrence in persons under the age of 18 years. Presentation is typically that of more insidious pain and limited range of motion, and is primarily seen in the knee joint. Diagnosis can be suspected with imaging, but ultimately surgical intervention is needed for tissue confirmation. We present a case of PVNS in a pediatric patient with acute symptoms concerning for a septic joint. The patient’s workup revealed a large effusion on hip ultrasound, with operative intervention pursued and further imaging deferred given the patient's symptom burden. A 4 × 1 × 1.5 cm intra-articular pigmented mass excised from the synovium in the operating room. The patient’s symptoms improved after the procedure, with pathology showing sheets of plump mononuclear cells in a collagenized stroma with hemosiderin deposits, confirming the diagnosis. This case highlights the importance of keeping non-infectious etiologies in the differential diagnosis of acute onset joint pain. Acute joint pain is a complaint that is common place in the pediatric population. Often in the acute setting the focus is differentiating between an infectious process and an inflammatory one. A common workup includes checking laboratory markers of inflammation, multimodal imaging such as ultrasound and MRI, and a physical exam focused on the joint of concern. While infection is the most concerning and thought of cause of severe joint pain, atypical causes should be considered in a clinical context that does not collaborate with the patient's history. Pigmented villonodular synovitis (PVNS), also known as tenosynovial giant cell tumor, has a varying reported incidence most commonly cited at two new cases per million annually. Typical presentation is in the second to third decade of life. Pain, limited range of motion, and joint swelling/fullness are most often reported. This case is unique because of the acuity of our patient’s onset of symptoms and their rapid progression. It also identifies a possible genetic predisposition towards this diagnosis, as the patient’s father reported a similarly resected mass from his hip during adolescence.An eight-year-old African-American male presented as a transfer to the emergency department with a chief complaint of left hip pain. His symptoms began three days prior to presentation after getting hit with a dodgeball in the thigh or hip, rapidly progressing to inability to bear weight two days prior. No other trauma was reported. Aside from subjective swelling of the hip, no other infectious symptoms were reported. Past histories were relevant for attention deficit hyperactivity and behavioral disorders with the patient prescribed stable doses of Adderall and Risperdal. The patient was non-toxic appearing, and his exam was significant for a left hip that was kept flexed and abducted due to pain with any range of motion. Imaging from the transferring facility included routine radiographs of the hip that were normal, and a contrasted CT of the pelvis obscured by motion artifact but with no acute abnormalities was noted. 3/microliter without leftward shift. An ultrasound of the hip was obtained, revealing a normal right joint and a 17-mm complex effusion of the left hip with surrounding hyperemia  of 16 mm/h , a C-reactive protein (CRP) of 1.00 mg/dL , and white blood cell count of 9.91 x 10The differential diagnosis of acute onset of non-traumatic hip pain in a child eight years of age includes septic arthritis or periarticular infections, such as osteomyelitis or myositis, transient synovitis, and synovitis from Legg-Calve-Perthes disease. Less likely is juvenile idiopathic arthritis (as the hip is very rarely the presenting joint) and synovial-based lesions such as PVNS .Given the clinical picture, particularly in regard to exquisite pain to range of motion of the hip, the decision was made to proceed with aspiration of the hip under anesthesia, rather than proceeding with further advanced imaging such as MRI, which would have resulted in a delay in treatment. Aspiration was productive of almost eight milliliters of straw colored fluid. At our institution, stat joint fluid analysis after aspiration under anesthesia generally takes between 30 and 45 minutes to return. For this reason, it is not unusual for the surgeon to proceed with formal irrigation and debridement of the joint before joint fluid analysis returns if there is clinical suspicion for septic arthritis. In this case, the decision was made to open the joint based on the range of motion exam and the large effusion. Upon opening the capsule through a medial approach, a large soft tissue mass was seen to be extruding from the joint. It was resected, the joint was irrigated, a drain was placed, and the skin incision closed. The mass was 4 x 1 x 1.5 cm in size Figure . By thisWhen a child presents with acute onset of atraumatic joint pain and on examination has significant pain to joint range of motion, ruling out septic arthritis should be the initial focus. To that end, our patient underwent the appropriate laboratory evaluation for osteoarticular infection, including complete blood count (CBC), ESR, CRP, routine radiography, and ultrasonography. Although the child was afebrile and inflammatory markers were normal, there was still concern about the extreme pain to range of motion and the “complex” effusion noted on ultrasonography. Delay in treatment of septic arthritis, particularly septic arthritis of the hip, can lead to poor outcomes; thus, the decision was made to proceed with immediate aspiration with irrigation and debridement and forego MRI, which would have resulted in a significant delay. Capsular incision with discovery of the mass was both diagnostic and therapeutic. We feel that the current case is important because it adds to the differential diagnosis of acute hip pain in children. It also highlights the challenging nature of obtaining a correct diagnosis in a pediatric patient presenting with acute joint pain, and the importance of multidisciplinary involvement when considering invasive procedures for diagnostic and therapeutic reasons.PVNS is a rare entity that causes chronic joint pain and limited range of motion. It is less commonly associated with acute arthralgia as we present here. PVNS in pediatric patients is even more rare, with only case reports of occurrence in persons under the age of 18 years . Cases aSimilar cases and presentations are rare in the literature. A case series from a patient population with similar demographics reviewed 17 cases of pediatric PVNS over an 11-year period. In this series, there was an average of 16 months from symptom onset to diagnosis. Only five patients presented with the hip as the affected joint, and of those only one patient experienced acute symptoms of pain and inability to bear weight raising concern for infection and resulting in diagnosis through operative investigation . While tThe patient had immediate improvement in his pain postoperatively and was discharged 48 hours after his procedure with a rolling walker. The family has transportation challenges and has not made their postoperative follow-up appointments, but the mother has been contacted on a number of occasions and she reports complete resolution of his symptoms. He has been scheduled to return for MRI of the hip to evaluate for residual disease. This case recognizes PVNS is an underthought and possibly underdiagnosed cause of joint pain in the pediatric population presumably due to limited growth/size in pediatric patients sufficient enough to result in symptoms. Surgical resection appears to have good outcomes in return of function and lower risk of recurrence in pediatric patients when compared to adults. While presentation and progression of symptoms is typically insidious, PVNS should be in the differential for an acute joint pain without infectious symptoms.


Schwannomas are neuroectodermal tumors that rarely occur in the retroperitoneal space. We report two cases of patients who presented with abdominal pain. Radiological findings revealed a retroperitoneal mass in both cases. Both patients underwent complete surgical excision with an uneventful postoperative course. The histopathological study confirmed the nature of schwannoma. Complete surgical excision remains the gold standard for the management of these tumors. The preoperative diagnosis is usually difficult; however, the definitive diagnosis is made upon histopathological examination. Schwannomas are neuroectodermal tumors that usually affect nerves in the head, neck, and extremities. The retroperitoneal location is rare and accounts for approximately 3% of all schwannoma . RetropeCase 1A 70-year-old female patient, with a history of diabetes, presented with chronic right flank abdominal pain. Abdominal ultrasound and computed tomography scan (CT) revealed a heterogenous right retroperitoneal mass, localized in the lateral and retrocaval area and medial to the right kidney, measuring 10 x 5 cm Figures .Laboratory tests including tumor markers and urine catecholamine were normal. The patient underwent a laparotomy surgery, showing a bilobed mass connected to the intervertebral foramina, and making us suspect the possibility of schwannoma. Thus, a complete resection of the tumor was performed Figure .The postoperative course was uneventful. Microscopic findings revealed the presence of spindle-shaped cells with a typical palisading pattern, arranged in short bundles of interlacing fascicles. The final pathologic diagnosis was a benign schwannoma. The patient experienced no recurrence over six months of follow-upCase 2A 29-year-old male patient, with no noticeable medical or surgical history, presented with a one-year history of chronic diffuse abdominal pain, physical examination was unremarkable. Abdominopelvic CT scan and abdominal MRI showed a well-circumscribed, heterogenous retroperitoneal mass developed in the right lateral aortic area measuring 14 x 7 cm  while other physicians managed patients conservatively due to benign biopsy results ,13,14. IThe final diagnosis is solely based on histopathologic examination of the specimens. RS is histologically characterized by alternating Antoni A and Antoni B areas . In AntoSurgical management consists of total resection. These tumors are usually solitary, well-circumscribed, and noninvasive, so complete surgical excision provides good results . The surRecurrence of benign schwannoma is rare, it follows usually incomplete resection, which was reported in 5%-10% of cases . In caseRetroperitoneal schwannomas are rare tumors. They are mostly benign, but can rarely be malignant, especially when associated with neurofibromatosis. The final diagnosis is based on histological and immunohistochemical findings of the specimens. Complete surgical resection remains the gold standard for the management of RSs. Despite the rare location of schwannoma in the retroperitoneum, they should be considered in cases of retroperitoneal abdominal masses.


Synovial sarcomas are infrequent malignant tumors occurring mostly in adolescents and young adults. The occurrence of synovial sarcoma in the hand-wrist area is rare .This report documents an uncommon case of synovial sarcoma occurring in the hand-wrist of a 69-year-old Caucasian woman. She was subsequently treated with surgical excision and radiotherapy without recurrence after follow up.This paper aims to characterize the demographic, pathologic, and clinical features with a literature review. The present literature review confirms that hand-wrist synovial sarcomas are more frequent among males and subjects 10 to 40 years old. Most cases in this location are usually not larger than 5 cm in size. The five-year survival rate is higher than that reported in a previous review on hand synovial sarcomas, and this suggests an improved survival in recent decades. Synovial sarcomas are malignant neoplasms with mesenchymal and variable epithelial differentiation. They are infrequent, accounting for 5-10% of all soft tissue sarcomas . This tuThis paper documents an unusual case of hand-wrist synovial sarcoma in a 69-year-old Caucasian woman, and characterizes this condition’s demographic, pathologic, and clinical features with a literature review.A 69-year-old Caucasian woman with a past medical history of low back pain, hypertension, and hyperlipidemia presented with a chief complaint of a dorsal ulnar-sided left hand-wrist mass that had been growing slowly over the previous 10 years. The patient stated that the mass had become progressively more painful over time, being particularly tender when she wore a watch. There had been no history of preceding trauma nor constitutional symptoms. On physical exam, the skin on the hands and wrist was intact with normal musculature. In the left dorsal ulnar wrist, near the extensor carpi ulnaris tendon, there was a small palpable mass that was semi-firm and not mobile; quite tender to palpation. A hand-wrist X-ray showed marked osteoarthritic changes. An initial clinical diagnosis of a probable ganglion cyst was made, and the patient underwent surgical excision of the mass.The pathology of the soft tissue fragments revealed a biphasic neoplasm composed of spindle cells admixed with neoplastic glands Fig. . No necrCT (computed tomography) of the thorax/abdomen and pelvis were without evidence of metastatic sarcoma. The tumor was classified as AJCC (American Joint Commission on Cancer) Stage IIA. A wide re-excision of the tumor was performed with en-bloc resection of the distal ulna. The resected tissue showed an ill-defined 1.0 × 0.5 × 0.5 cm firm mass involving the soft tissue without involving the bone. The histopathologic exam confirmed the prior diagnosis. The patient received adjuvant radiotherapy and had regular follow-ups for 5.5 years with no evidence of any local recurrence of the tumor or distant metastases. The timeline of the episode of care is summarized in Fig. Synovial sarcomas are rarely encountered in routine clinical practice. Nevertheless, they are a highly malignant type of soft tissue sarcoma. Therefore, clinicians and pathologists should always maintain a high level of suspicion to ensure these cases are not unrecognized.Synovial sarcomas in the hand-wrist area are an uncommon occurrence. Their frequency is 4-8.5% of all synovial sarcomas in different studies –12. WhenThe synovial sarcoma case herein described is particularly unusual considering the patient’s age and sex (a woman in her late sixties), and the infrequent location (the hand-wrist region). Despite its name, synovial sarcomas do not arise from synovium and rarely occur in the joints. A microscopic similarity to the primitive synovium was observed early in the literature, but its histogenesis is still unclear , 14. Nevmonophasic variant is characterized by fascicles of atypical spindled cells with scant cytoplasm and monotonous vesicular nuclei, often overlapping. There is a variable mitotic rate. Collagenous stroma can be more or less abundant, and thickened bundles of “wiry collagen” can be seen. Vasculature can be prominent, and hyalinization or myxoid changes are rarely seen. Mast cells are frequently observed, and necrosis is uncommon. Biphasic synovial sarcoma contains the aforementioned spindle cells, and in addition there is an epithelial component showing glandular or nested growth. The epithelial cells have amphophilic cytoplasm and round to ovoid nuclei. The glandular lumina may contain eosinophilic amorphous material or mucin. Rarely, squamous metaplasia can be seen. Poorly differentiated synovial sarcoma is characterized by hypercellularity, hyperchromatic nuclei, polymorphism, and increased mitoses. Necrosis is common. Poorly differentiated areas may be present adjacent to more typical monophasic or biphasic areas. In some cases, the entire tumor is poorly differentiated.Histologically, there are three variants of synovial sarcoma : monophaSS18 (SYT) in chromosome 18 and SSX1, SSX2, or SSX4 in chromosome X [A characteristic cytogenetic translocation tX;18)  is present in almost all synovial sarcomas; helping provide diagnostic confirmation. This translocation fuses mosome X . Immunoh , which  [et al. , 22–67 det al. and in keeping with the literature [The demographic features of the cases reported in the literature are summarized in Table terature . Therefohand was the most commonly involved location . Nearly all cases involved the soft tissue with the palmar surface of the hand reported in 18 cases, the dorsal surface in four, the area between metacarpals in five, the fingers in eight, the anatomical snuffbox in one. A digital nerve was involved in one case, while an unspecified hand area was reported in eight cases. The wrist was involved in 14 cases (24.5%), and two cases concomitantly involved the hand. Among these, in 13 cases affected the soft tissue, while only one case involved the median nerve. These findings confirm that the incidence of finger involvement by this type of sarcoma is less common than that of the carpus [The In 20 cases collected, the duration of symptoms before diagnosis was clearly stated in the studies and is reported in Table The greatest dimensions of the tumors at diagnosis were clearly reported in only 34 cases. Size ranged between 0.5 and 10 cm, with an average of 2.8 cm. In eight additional cases, the size reported was “5 cm or less”, while in two it was “more than 5 cm”. In total, 37 cases out of 44 were diagnosed when the tumor size was 5 cm or less. Therefore, this review and the presented case report show that in a majority of cases, this tumor is 5 cm or less in greatest dimension. This is in disagreement with few articles stating that larger tumor sizes are usually present at diagnosis . The rat et al., who found that 30% of patients had radiotherapy and 5% chemotherapy. This demonstrates more widespread use of therapies like radiation and chemotherapy in recent decades to treat synovial sarcomas.Radical surgical excision was the treatment of choice . Adjuvant radiotherapy was performed in 29 cases (51%), while chemotherapy was used in 12 cases (21%), either before or after surgery. These percentages are higher than those observed by Dreyfussp > 0.05) when comparing tumor size , histology subtype (biphasic versus monophasic), and age . It is uncertain why the recurrence rate in the present literature review is relatively lower compared to the review by Dreyfuss et al. and also considering that Dreyfuss did not provide the tumors’ size at diagnosis in their analysis.The follow-up period is summarized in Table  et al. in their review focused on hand synovial sarcomas found that 18% of the patients survived five years after beginning treatment and 9% survived ten years. Therefore, the present literature review showed an improved 5-year and 10-year survival in hand-wrist sarcomas in the recent decades. Various factors seem to have a poor survival prognostic value in subjects affected by synovial sarcomas [In the literature analyzed, the mortality rate was 10.2% (5 out of the 49 followed-up subjects). Sixteen patients (28%) survived at least 5 years after beginning treatment. Twelve (21%) survived at least 10 years, with one surviving more than 32 years. The overall five-year survival rate for synovial sarcomas of any location is 27–85% in the literature , 16. Dresarcomas : advanceIn conclusion, this literature review confirms that synovial sarcomas of hand-wrist are more frequent among males and subjects 10 to 40 years old. These findings corroborate that the case report presented is a rare occurrence. In the literature analyzed, the incidence of finger involvement by this type of sarcoma was less common than that of the remaining areas of the hand and wrist. A delay in diagnosis was not common, probably because a high level of suspicion for this tumor has been achieved. Moreover, in most cases, this tumor was diagnosed when 5 cm or less in greatest dimension. The five-year survival rate in the cases analyzed was higher than that reported in the previous literature review on hand synovial sarcomas; suggesting an improved survival in the recent decades.


Primary dural lymphoma (PDL) is an extranodal non-Hodgkin lymphoma that accounts for less than 1% of all central nervous system lymphomas. Primary dural lymphoma grows from the dura mater, and it is often diagnosed as meningioma or acute subdural hematoma due to its radiological characteristics. Surgery is the gold standard of therapy; in many patients, PDL is relatively benign with good outcome. Authors report their experience in three patients affected by extranodal non-Hodgkin lymphoma (PDL) mimicking a meningioma. Primary dural lymphoma (PDL) is an extranodal non-Hodgkin lymphoma, a subtype of primary central nervous system lymphoma (PCNSL). This lymphoma develops primarily from brain parenchyma, eyes, meninges, or spinal cord in the absence of systemic disease. This lymphoma subtype accounts less than 1% of all central nervous system lymphomas and less than 0 1% of all non-Hodgkin's lymphomas . The cenFrom 2012 to 2018, in the Clinic of Neurosurgery of Ancona three patients affected by primary dural lymphoma have been admitted. The first patient was a 49-year-old man with clinical history of personality and mood change and the second a 64-year-old woman with onset of right-handed and lateral hemianopsia. An MRI study with contrast documented the presence of expansive lesions with surrounding edema and isointense on T1-weighted and hyperintense on T2-weighted images, characterized by an intense post-contrastographic enhancement, with dural implant, located in the first case in the right frontal region, and in the second case, in the left occipito-parietal region . Due to th–6th decade of life predominantly in women. The incidence of this tumor varies from 0.6 to 3% of all brain neoplasms [Primary dural lymphoma (PDL) is a very rare disease, and most commonly it occurs in the 4eoplasms . Iwamotoeoplasms . MeningePrimary dural lymphoma (PDL) should be considered in the differential diagnosis of meningiomas. In O'Neil series of 15 patients with PDL, a clinical and radiographic diagnosis of meningioma was made in 14 out of 15 patients prior histopathological diagnosis . The PDLPrimary dural lymphoma is a rare disease, and no standard treatment is described in literature. Surgical total excision remains the gold standard of treatment. Because complete resection of PDL may be technically difficult due to multiple tumors and infiltrative and en plaque presentation, many adjuvant drugs were indicated: systemic or intrathecal chemotherapy with R-CHOP, high dose of methotrexate, cytarabione, and vincristine –10. As rPrimary dural lymphoma is a very rare disease, and it should be considered in the differential diagnosis of other tumors of dura mater and/or cranial bone. Our cases illustrate that intracranial lymphoma may rarely present as a wide dural-based mass mimicking a meningioma clinically, radiologically, and intraoperatively. So it must be considered in the differential diagnosis for all extra-axial enhancing lesions. Since PDL is very rare, there is no standard treatment for this tumor, so the decision should be tailored on each patient, based on the immunological and histopathological features of lesion. However, surgical excision and radiotherapy are the gold standard of therapy which grant good outcome.


Hydrocystomas are cystic proliferations of the sweat glands with apocrine or eccrine differentiation . ApocrinWritten informed consent was obtained from the legal guardian of the patient to publish this case and accompanying images in scientific journals for research and educational purposes.In January 2015, a healthy 6-yr-old boy presented to the Pediatric Surgery Department  with a painless, cystic mass on the right breast for 2 years. The mass had recently been increasing in size until it was 7×6 cm at presentation .He had no past medical history and was otherwise asymptomatic. Ultrasound examination of the right breast mass revealed a 6.5×5 cm hypoechoeic lesion. Differential diagnoses were tending towards cyst lymphangioma rather than a hidrocystoma, clinical suspicion of malignancy was low. The patient proceeded to excision of the lesion and the gross specimen showed characteristics of a multiloculated cyst . HistopaApocrine hidrocystoma is a benign neoplastic lesion of the skin. This tumor is very rare in childhood and generally present as small cutaneous nodules, which are mainly solitary . The difTreatment of single apocrine hidrocystomas should be focussed on excision with narrow margins due to the benign nature of the lesion , 9. No f


An important complication of pyogenic spondylitis is aneurysms in the adjacent arteries. There are reports of abdominal aortic or iliac aneurysms, but there are few reports describing infected aneurysms of the vertebral artery. Furthermore, there are no reports describing infected aneurysms of the vertebral arteries following cervical pyogenic spondylitis. We report a rare case of an infected aneurysm of the vertebral artery as a complication of cervical pyogenic spondylitis, which was successfully treated by endovascular treatment.Cervical magnetic resonance imaging (MRI) of a 59-year-old man who complained of severe neck pain showed pyogenic spondylitis. Although he was treated extensively by antibiotic therapy, his neck pain did not improve. Follow-up MRI showed the presence of a cyst, which was initially considered an abscess, and therefore, treatment initially included guided tapping and suction under ultrasonography. However, under ultrasonographic examination an aneurysm was detected. The contrast-enhanced computed tomography (CT) scan showed an aneurysm of the vertebral artery. Following endovascular treatment (parent artery occlusion: PAO), the patient’s neck pain disappeared completely.Although there are several reports of infected aneurysms of the vertebral arteries, this is the first report describing an infected aneurysm of the vertebral artery as a result of cervical pyogenic spondylitis.Whenever a paraspinal cyst exist at the site of infection, we recommend that clinicians use not only X-ray, conventional CT, and MRI to examine the cyst, but ultrasonography and contrast-enhanced CT as well because of the possibility of an aneurysms in neighboring blood vessels. It is necessary to evaluate the morphology of the aneurysm to determine the treatment required. The number of patients being hospitalized because of pyogenic spondylitis is increasing annually . AbscessA 59-year-old man with a medical history of dyslipidemia was suffering from continuous neck pain. Three days after the appearance of neck pain, he visited a nearby clinic and was treated conservatively using analgesics only. Two weeks later, he was referred to another hospital because of a high fever of 38.6° C and increased inflammatory markers on a blood examination . He was hospitalized on the same day (day 0) because magnetic resonance imaging (MRI) showed cervical pyogenic spondylitis and an extradural abscess at C6 and C7  score of 8.0. Physical examination revealed muscle weakness at the bilateral finger extension. There was no evidence of pyramidal signs or sensory disturbance.An ultrasonographic examination of the neck was performed in order to evaluate the properties of the neck cyst (which was thought to be an abscess). Ultrasonographic examination showed pulsatile turbulence in the cyst Fig. a. Color He was transferred back to the referring hospital on day 47 without complications resulting from the treatment. Cefazolin was administered to the patient until day 58, after which cefaclor was orally administered until day 91 (as an outpatient). CRP was 0.02 mg/dl and WBC count was 4600/µl in a blood examination performed on day 91. When the antibiotic therapy was finished, his muscle weakness recovered and his VAS for neck pain remained 0. The C5-C6 vertebral body was completely fused. At the 2-year follow-up, there were no new lesions or recurrence of the pyogenic spondylitis or aneurysm.Most incidences of pyogenic spondylitis are reported as bloodstream infections that often infect intervertebral discs . Our casAlthough there are reports of infected aneurysms, a limited number of reports have described infected aneurysms of the vertebral arteries as indicated in Table Infected aneurysms are broadly classified into those arising from infection of arterial walls and the infection of existing aneurysms. The routes of infections of arterial walls are further classified into four categories: (1) infected endocarditis , (2) adjIn previous reports, the diagnosis of an infected aneurysm of the vertebral artery was confirmed by conventional and contrast-enhanced CT. An aneurysm may be diagnosed by palpation, but this was difficult in our case because the common carotid artery was in close proximity to the aneurysm. Therefore, we confirmed that the cyst  was an aneurysm by ultrasonographic examination, which is a non-invasive and convenient procedure. Furthermore, contrast-enhanced CT scans showed that the cyst was an aneurysm of the vertebral artery. MRI is generally considered the most sensitive method for examination of pyogenic spondylitis . In our The treatment for infected aneurysms of the vertebral arteries, includes surgical treatments for ruptured cases and large aneurysms , 11. In In conclusion, we described the treatment of an infected aneurysm of the vertebral artery following cervical pyogenic spondylitis. This report highlights the need to evaluate paraspinal cysts located around the vertebral artery using not only X-ray, conventional CT, and MRI, but also ultrasonographic examination and contrast-enhanced CT considering the possibility of an aneurysm being present. Moreover, we recommend that the morphology of the aneurysm be evaluated to determine the most effective treatment required. The cervical pain of the patient in the current study disappeared after endovascular treatment.


Peutz–Jeghers syndrome (PJS) is a very rare autosomal dominant genetic disorder characterized by hamartomatous polyps in the gastrointestinal tract and hyperpigmentation of the lips, hands, and feet. The hamartomatous polyps in the small intestine often cause intussusception and bleeding.A 62-year-old male was hospitalized for treatment of deep vein thrombosis and pulmonary embolism. In the small intestine, computed tomography showed three small polyps with intussusceptions. Since the patient had gastrointestinal polyposis and pigmentation of his lips, fingers, and toes, he was diagnosed with PJS. After an inferior vena cava filter was placed, he underwent laparoscopic-assisted surgery. The polyps causing intussusception were resected as far as possible without intestinal resection, since they had caused progressive anemia and might cause intestinal obstruction in the future. The patient was discharged from the hospital on postoperative day 9 without complications.Laparoscopic-assisted disinvagination and polypectomy is a useful, minimally invasive treatment for multiple intussusceptions caused by small intestinal polyps in patients with PJS.The online version contains supplementary material available at 10.1186/s12957-021-02133-5. Peutz–Jeghers syndrome (PJS) is an autosomal dominant genetic disorder characterized by gastrointestinal polyposis and mucocutaneous pigmentation . It was STK11), also known as liver kinase B1 [STK11 is localized on chromosome 19p13.3 and functions as a tumor suppressor gene [PJS is characterized by germline mutations in the serine–threonine kinase 11 gene  and pulmonary embolism (PE) with heparin and factor Xa inhibitor. Magnetic resonance imaging showed that DVT extended from both the left external iliac vein and the right femoral vein to the periphery  was made at the navel and a wound retractor was placed. The first port was inserted through the wound retractor. Four additional ports were inserted as follows: at the wound retractor, in the left upper quadrant, and in the right and left lower quadrants.We examined the entire small intestine laparoscopically and identified five intussusceptions, located 110, 140, 180, 200, and 225 cm from the ligament of Treitz Fig. a. After The patient resumed oral ingestion of water on postoperative day (POD) 1, liquid food on POD5, and porridge on POD6. He received a transfusion of red cell concentrate (280 ml) on POD2 since his hemoglobin was 6.9 g/dl. There were no complications and he was discharged from the hospital on POD9.All polyps were pathologically diagnosed as hamartomatous polyps compatible with PJS Fig. a, b.FigSTK11, which is a tumor suppressor gene [It has been reported that patients with PJS have a high risk of malignant tumors of the gastrointestinal tract, pancreas, lung, uterus, ovary, breast, and testis due to germline mutations in sor gene , 10. BoaPolyps in PJS may be found throughout the gastrointestinal tract, excluding the esophagus, and are most common in the small intestine . Since pAlthough PJS polyps are considered to be hamartomas, some studies have shown that they may have an adenomatous component , 16. A hDouble-balloon enteroscopy (DBE) and capsule endoscopy devices are able to directly visualize the inside of the small intestine, and DBE can also be used to resect polyps. Reported complications of DBE include bleeding (1.4–2.7%), perforation (1.4–2.9%), and acute pancreatitis (2.7%) –20. If pSurgery for small intestinal intussusception requires not only intestinal repositioning but also resection of polyps, since these are a risk factor for multiple conditions. If an irreversible disorder is present, especially intestinal necrosis, bowel resection is necessary. However, multiple enterotomies should be avoided whenever possible, as these may result in short bowel syndrome. In the present case, since IOE was not available at our hospital, we made a small incision near each small intestinal intussusception and successfully resected the nearby polyps without the need for bowel resection. Since all of the polyps with a dimension of over 1 cm were pedunculated and were obviously located within the mucosa on both visual inspection and palpation during surgery, we decided to resect the nearby polyps without bowel resection. Pathological findings revealed that all polyps were benign tumors situated within the mucosa. Moreover, since laparoscopic-assisted resection is a minimally invasive operation, we were able to minimize the patient’s suffering.After surgery, the digestive tract should be evaluated by CT and/or gastrointestinal tract examination to identify sizeable gastroenterological polyps that may cause intussusception or bleeding. Moreover, patients with PJS are at increased risk of cancers of various organs, including the esophagus, stomach, small bowel, colon, pancreas, lung, testis, breast, uterus, ovary, and cervix , 22. HowAdditional file 1: Figure S1 a. Magnetic resonance imaging reveals DVT extending from both the left external iliac vein and right femoral vein to the periphery. Figure S1b. Echocardiography reveals PE involving an artery of the right lower lung, with no pulmonary hypertension (arrowhead).Additional file 2: Table S1. Results of hematological examination.


Ultrasound (US) is a useful tool in diagnosis and follow‐up of portal vein aneurysms (PVA). In the absence of international surgical guidelines on PVAs, US can be effectively used in follow‐up of asymptomatic patients not suitable for surgery. Ultrasound (US) is a useful tool in diagnosis and follow‐up of portal vein aneurysms (PVA). In the absence of international surgical guidelines on PVAs, US can be effectively used in follow‐up of asymptomatic patients not suitable for surgery. In 1976, Doust and Pearce reported a maximum diameter of the portal vein of 15 mm in healthy subjects and 19 mm in cirrhotic patients. Therefore, the portal vein diameters exceeding such measures need to be considered as pathological.2An 86‐year‐old Caucasian man was admitted to the emergency room (ER) for dyspnea. The patient's past medical history included ischemic heart disease with congestive heart failure, permanent atrial fibrillation on anticoagulant prophylaxis with warfarin, chronic obstructive pulmonary disease (COPD), and chronic kidney disease (stage IV K‐DOQI) and was unremarkable for diseases affecting the digestive system. The patient also denied any surgical history, trauma, and malignancy. Routine laboratory tests including complete blood count, liver function, and coagulation tests were within the normal range. In the suspicion of acute exacerbation of COPD, the ER work‐up included a chest x‐ray that showed right basal parenchymal thickening and pleural effusion. Hence, during the observation period in ER, because of the worsening of respiratory failure, a computed tomography (CT) scan (without contrast medium) was required. The latter showed lung architectural distortion, reticular opacities in the immediate subpleural lung, honeycombing, and traction bronchiectasis. The thoracic radiological findings were consistent with the diagnosis of usual interstitial pneumonia (UIP). Furthermore, the scans passing through the upper abdominal quadrants showed, as an incidental finding, a space‐occupying lesion in the hepatic hilum Figure . It was An additional evaluation through abdominal US was carried out during the following stay of the patient in the medical ward. The liver hilum lesion appeared at US as a hypo‐anechoic formation, with a maximum diameter of 5.5 cm, with thickened wall, and apparently in continuity with the right branch of portal vein. The pulsed‐wave Doppler evaluation described the presence of a nonpulsatile, monophasic and slow flow inside the lesion Figure , with a Except for fatty liver disease, the US examination did not show any evidence of cirrhosis, ascites, splenomegaly, bowel distension, or any other abdominal abnormality. The aforementioned findings led to the diagnosis of portal vein aneurysm. Despite the lesion's notable dimension, no signs of portal hypertension and thrombosis were found. In fact, the spleno‐porto‐mesenteric axis was characterized by normal velocity curves throughout its whole course. Pulsed‐wave Doppler demonstrated the presence of a monophasic, nonpulsatile venous flow pattern inside the aneurysm Figure . Hepatic3Portal vein aneurysms is a rare visceral venous aneurysm, representing 3% of the venous aneurysm in the human body,The etiology of PVAs is generally considered to be either congenital or acquired. The former variant may be related to the incomplete regression of the right primitive distal vitelline vein or to the presence of other abnormalities of vitelline veins . During the development of the portal vein taking place in the embryonic phase, such abnormalities may promote the formation of a diverticulum which can grow into a venous aneurysm.Given the notable dimensions of the PVA, we presumed that our patient would have had a relevant risk to develop intra‐aneurysmal thrombosis. The fact that he was taking an anticoagulant might have prevented such PVA complication. However, eighteen cases of nonthrombosed PVAs exceeding 5 cm in their largest diameter are reported in the literature, with no anticoagulation taken before their diagnosis. With regard to possible underlying factors in the risk of PVA thrombosis, in their paper Koc et al reported that four out of seven patients with thrombosed PVAs were carrying a thrombophilic defect, proposing thrombophilia screening in patients with this complication.US and CT are the methods of choice in the evaluation of a PVA.The clinical and therapeutic management of PVAs remains controversial. In 2012, Ma et al proposed an algorithm to help clinicians make diagnosis and therapeutic decisions in this setting.This case highlights the importance of ultrasonography in the diagnosis and follow‐up of PVAs. Furthermore, this case represents an additional report of efficacious clinical management of a large PVA. In the absence of complications, a conservative approach to PVAs may guarantee a good clinical outcome, especially in elderly patients.The authors have no conflicts of interest to disclose.Andrea Schilardi and Gianfranco Antonica: performed the ultrasound examination. Mariangela Carbone: performed the CT scan. AS, GA, Alessandro Ciavarella, Elsa Berardi, and Carlo Sabbà: participated at the case management. AC, AS, and MC: wrote the manuscript. GA, EB, and CS: read and approved the manuscript.All procedures were under the ethical standards of the local research committee.


Background: Feminizing adrenal tumors are rare and generally malignant tumors usually seen in male adults and children. We report the case of a benign feminizing adrenal tumor in a male patient. To our knowledge, only 2 other cases of benign, estrogen-only–secreting adrenal tumors have been reported.Case Report: A 44-year-old male with a history of hypertension presented to his primary care physician with chest tenderness, fatigue, and erectile dysfunction. Hormonal workup and imaging identified an estrogen-only–secreting adrenal adenoma. The adenoma was removed via laparoscopic adrenalectomy, and the patient had a normal postoperative course. Pathologic findings were an adrenal cortical adenoma with a Weiss score of 0 and a Ki-67 score of 0%. At 6-month follow-up, the patient's symptoms had significantly improved, and his previously abnormal sex hormone levels were within normal limits.Conclusion: Given the ambiguity in distinguishing between malignant and benign feminizing adrenal tumors, we suggest that radiologic (via Hounsfield units), clinical (via trending hormone levels), and histopathologic (via Weiss and Ki-67 scores) findings are sufficient to confirm the benign nature of this commonly malignant tumor. In a study conducted by Moreno et al, feminizing adrenal tumors accounted for 0.37% of 801 adrenalectomies between 1970 and 2003, and all of them were malignant.2 The most common initial presenting symptoms in males are gynecomastia, erectile dysfunction, and fatigue.1 Gynecomastia is usually bilateral and painful, and the size varies significantly among patients. This breast development is related to the high levels of estrogen being secreted from the tumor via increased activity of aromatase and the peripheral conversion of androstenedione to estrone.3 Estrogen production can be solitary but is more commonly accompanied by the production of other adrenocortical hormones.Feminizing adrenal tumors are extremely rare adrenal neoplasms. Chentli et al identified 50 cases reported between 1979 and 2014.4 All of the tumors in these 3 studies showed aggressive pathology and were considered carcinomas according to the consensus guidelines (requiring a Weiss score >2).5The prognosis of patients with malignant tumors varies in the literature, with Chentli et al reporting a survival of 4 years, Moreno et al reporting a survival of 7 years, and Lanigan et al reporting a median survival of 2.9 months.7We report a case of benign feminizing adrenal tumor in a male patient. To our knowledge, only 2 other cases of benign estrogen-only–secreting adrenal tumors have been reported.A 44-year-old male with a history of hypertension presented to his primary care physician in January 2017 for chest tenderness, predominantly on the left, for the prior several months. He had also noticed an increase in the size of both breasts. The patient was an active individual with a regimen of weightlifting and basketball 3 times per week, but he reported weight gain  and feeling fatigued. Additionally, he had had erectile dysfunction symptoms since 2016 for which he had seen a urologist. Mammogram revealed benign bilateral gynecomastia that was managed conservatively.In January 2018, he presented again to his primary care physician with spontaneous improvement of the previously reported chest pain but continued symptoms of fatigue and erectile dysfunction. He was referred to an endocrinologist who determined that his breasts were still enlarged but overall had not changed since his previous mammogram. Hormonal workup showed an elevated estrone level of 192 pg/mL , elevated estradiol of 44 pg/mL , low follicle-stimulating hormone of 0.8 mIU/mL , and low testosterone of 28 ng/dL . Human chorionic gonadotropin and alpha-fetoprotein levels were normal, and testicular ultrasounds to evaluate for testicular and extragonadal germ cell tumors were unremarkable. Pituitary hormonal workup to evaluate secondary hypopituitarism was normal . AdrenalThe patient underwent outpatient laparoscopic left adrenalectomy  in June 8 However, current consensus guidelines consider the components of the Weiss score—mitotic rate, atypical mitoses, high nuclear grade, low percentage of clear cells, necrosis, diffuse architecture of tumor, capsular invasion, sinusoidal invasion, and venous invasion—as essential in distinguishing between benign and malignant.5 Another important aspect to consider is the proliferative indices, such as Ki-67, as these values are significantly higher in the setting of adrenocortical carcinoma vs adenoma. Beuschlein et al suggested a grading system in relation to Ki-67 in which higher Ki-67 scores are associated with higher tumor grades and worse prognoses.9 A Ki-67 score <10% is considered grade 1, scores between 10% and 19% are grade 2, and scores >20% are grade 3. The feminizing adrenal tumors presenting with high mitotic rates (a component of the Weiss score) were associated with a dire prognosis.5 We used the Weiss score and the Ki-67 score to label our case as benign and suggest that if a tumor is radiologically, macroscopically, and histopathologically diagnosed as benign with Hounsfield units, Weiss score, and Ki-67 score, the tumor should be considered benign, regardless of the patient's hormonal status.The paucity of data describing truly benign feminizing adrenal tumors leads to difficulties in distinguishing between benign and malignant tumors. Mongiat-Artus et al postulated that any adrenal tumor secreting estrogens should be considered malignant regardless of presentation and size.7 The outcome of the male patient—reported by Andía Melero et al—was not expected, as 3 years postadrenalectomy, he had a reoccurrence with metastasis and died.7 This patient had cellular proliferation without atypia and no capsular invasion and was diagnosed with adenoma. The histologic finding in the case begs the questions of whether the histologic appearance can be considered accurate in all cases and whether histologic appearance is more appropriate for prognosis of malignant feminizing adrenal tumors than benign feminizing adrenal tumors. Andía Melero et al suggest that even in the setting of a benign histopathologic diagnosis, the clinician should be highly suspicious of a tumor secreting estrogen.7 Andía Melero et al did not report a Weiss score or a Ki-67 value for their patient.As mentioned previously, only 2 other cases of benign estrogen-only–secreting adrenal tumors have been reported, and only 1 was in a male patient with feminizing features as in our case.6 Despite the difference in sex, we can draw parallels in the way the tumors behaved in our patient and in the patient described by Luton et al. After excision of the tumors, both patients’ symptoms improved, and neither patient had further issues at extended follow-up. This outcome suggests that for a truly benign estrogen-only–secreting tumor, excision is the only treatment necessary.Luton et al reported a case of a benign estrogen-only–secreting adrenal tumor in a female who was in her reproductive years. The patient presented with metrorrhagia as her chief complaint, and imaging revealed a mass on her adrenal gland. After resection, the patient's ovulatory cycle returned to normal, and she had no further issues at 2-month, 15-month, and 11-year follow-up. Luton et al described the histologic findings as benign, but they did not include information on special stains or Weiss score.5 Surgical resection generally results in reduced gynecomastia and the other feminizing features associated with feminizing adrenal tumors. Medical therapy with tamoxifen or aromatase inhibitors and mitotane has been reported to be successful in treatment of adrenocortical carcinoma; however, no studies have compared the efficacy between these treatments.10The approach to a benign feminizing adrenal tumor is similar to the approach used for other adrenocortical tumors. Evaluation of these masses should include a workup for hypercortisolism and pheochromocytoma, along with testing for sex hormones and the adrenal intermediaries listed in Given the sentinel event after benign histopathology in the case reported by Andía Melero et al, primary care physician follow-up of these patients is imperative. Follow-up at 6 months postoperatively and yearly thereafter should include an adequate history for identifying reoccurring symptoms, a physical examination, and trending of hormonal levels.Benign feminizing adrenal tumor in a male adult is a rare presentation, with prognosis and management largely unreported. More important, the ability to predict the aggressiveness or malignant potential of these tumors is uncertain. This report substantiates the importance of radiologic and histopathologic findings in the diagnosis of these tumors, as well as the need for regular postoperative follow-up with trending of hormonal levels.


Paraovarian/paratubal cysts constitute about 10% of adnexal masses and are usually small and asymptomatic. A huge paratubal cyst complicated by adnexal torsion is a rare cause of acute low abdominal pain.We report the case of an obese 31-year-old nulliparous woman who presented with a large pelvic cyst causing ovarian torsion. The size of the mass (~25 cm) caused pain, and obesity led to explorative laparotomy, which showed a huge central abdominal-pelvic cyst arising from the right adnexa. Cystectomy was technically impossible, so all the adnexa was removed. Pathologic diagnosis revealed a papillary serous cystadenoma with torsion of all structures.A giant paratubal cystadenoma is a rare condition and management is challenging. If there are clinical and imaging signs of torsion, it should be approached like any other adnexal mass and surgery should be urgent in order to avoid irremediable compromise of ovarian function. •Paraovarian/paratubal cysts constitute about 10% of adnexal masses, and are usually small and asymptomatic.•A huge paratubal cyst complicated by adnexal torsion is a rare cause of acute low abdominal pain.•Suspected adnexal torsions require prompt surgical treatment in order to avoid compromises of ovarian function. The terThese cysts represent approximately 10% of all adnexal masses, are mostly benign and usually arise from paramesonephric and mesonephric remnants .They are typically between 2 and 20 mm and noted as incidental findings during other pelvic surgeries ,4. Due tWe report a case of a giant paratubal cyst which presented as acute tubo-ovarian torsion.22 ; she was taking no medication and had no other surgical or medical history to disclose.An obese 31-year-old nulliparous woman was admitted to the general emergency service with back pain that had started one week earlier and gradually become constant and increased in intensity. She was also vomiting but denied fever, dysuria, constipation, diarrhoea or anorexia. She had never been pregnant and had had amenorrhea for the past 7 years, due to polycystic ovary syndrome. Her body mass index was 39 kg/m3, 72% neutrophils), normal renal function and were negative for C-reactive protein. Urine analysis and pregnancy test were also negative.During her physical exam, her vital signs were normal and abdominal examination revealed only tenderness in right quadrants, without guarding or rebound. Blood tests were performed and revealed a high white blood cell count  scan, which showed a huge central abdominal-pelvic cyst arising from the right adnexa . The cysShe was then referred to the gynaecology department. Her gynaecologic exam was apparently normal but limited due to obesity. A pelvic ultrasound revealed a 20 cm × 20 cm thin-walled mass of the right adnexa, not clearly separate from it. Doppler examination showed that the right ovary was visible and had a polycystic ovarian morphology and apparent presence of vascular flow. The left ovary had the same polycystic pattern.Her pain became acutely worse, which reinforced the hypothesis of adnexal torsion. Based on these findings and the increased pain, we decided to perform exploratory laparotomy. Intraoperatively, a giant (~25 cm) cystic median mass of apparent right adnexal origin was seen, occupying the entire pelvis up to the diaphragm. The cyst wall was intact, adhesion-free and swollen. Necrotic ovarian tissue was identified within the mass, but no cleavage planes were identified. The pedicle, which included the infundibulopelvic and utero-ovarian ligaments, was twisted twice around its stalk . The lefThe patient underwent resection of the mass without rupture. Frozen-section analysis was not available since the surgery was performed during the night. The final pathology report noted a paratubal serous papillary cystadenoma, an ovary with polycystic morphology and a normal fallopian tube. All fragments had congestion, oedema and haemorrhage, consistent with obstruction of the arteriovenous flow (torsion). The postoperative course was uneventful.3Paratubal or paraovarian cysts are more commonly found in the third and fourth decades of life, but there are reports of cases in children, adolescents and postmenopausal women . These cA paraovarian cyst is clinically similar to an ovarian cyst and imaging techniques can help with the diagnosis. Ultrasound is useful for detecting a round/oval cyst close to but separate from the ipsilateral ovary ,11. The Paraovarian/paratubal cysts are symptomatic when they become larger, but, even then, complications such as haemorrhage, torsion or rupture are rare . These cCysts larger than 10 cm and clinical suspicion of torsion require surgery . DiagnosLaparoscopy is the preferred approach for the diagnosis and treatment of adnexal torsion, but experience is required and size of the lesion is a limiting factor . This nu4Paraovarian/paratubal cysts are frequent but only in a few cases do they reach clinically relevant dimensions. Complications such as torsion and rupture are rare and are rarely diagnosed preoperatively. Differential diagnosis of acute abdominal pain must consider this condition and imaging can clarify the diagnosis and detect signs of acute complications, including torsion.In the case of suspected adnexal torsion in young women, prompt surgical treatment is required in order to avoid irremediable compromise of ovarian function and fertility.


Extragonadal teratomas in adults are an extremely rare entity. Teratomas may be mature or immature and are characterized by their midline presentation. We are presenting here a case report of immature teratoma of the palm in a 35-year-old female. The patient developed a recurrent swelling of the palm from which a wedge biopsy was taken and sent for histopathological examination. A diagnosis of “immature teratoma, grade 2” was made, whose margins were compromised. Later on ultrasonography (USG) abdomen and pelvis and whole-body magnetic resonance imaging (MRI) showed no significant findings ruling out the possibility of an occult primary. MRI palm showed a well-defined cystic lesion with loculations suggestive of teratoma. Re-exploration was advised to achieve free margins. A teratoma, a type of germ cell tumor, maybe gonadal or extragonadal. Primary extragonadal germ cell tumors as a whole are extremely rare in incidence. Primary extragonadal germ cell tumors are characterized by their midline location in the body . The patA 35-year-old female presented to our hospital with a complaint of a recurrent ill-defined nodular swelling measuring 3×2 cm over the palm for the past one month Figure .The overlying skin was bluish in coloration and showed the scar of the previous surgery. The patient gave a history of recent surgical excision of a soft tissue mass at the same site measuring around 4X3 cm three months ago. Outside the suggestion of the possibility of a germ cell tumor, no immunohistochemistry was performed. We received all the paraffin blocks for review. On histopathology, there were predominantly mature glial cells Figure , immaturOne of the resected margins was involved by the tumor. On immunohistochemistry, mature neural tissue was positive for glial fibrillary acidic protein (GFAP) and immature neural tissue was positive for neuron-specific enolase (NSE) Figure .Grading was done on the basis of the content of immature neuroepithelium. Hormonal investigations were done. Alpha-fetoprotein (7.1 ng/ml)  and beta-human chorionic gonadotropin (1.4 mIU/ml)  levels were normal. Ultrasonography abdomen and pelvis showed bilateral normal adnexa with no significant lymphadenopathy. Uterus showed multiple fibroids. Whole-body MRI showed no significant abnormality except MRI hand. MRI left hand Figure  showed aA wedge biopsy was done from the present swelling, which showed similar morphological features, including immature neural tissue, which was positive for NSE. After clinico-radiological correlation, a final diagnosis of a primary immature teratoma, grade 2, of the palm was given. A repeat surgical excision of the swelling with the restoration of clear margins was advised.Broadly, teratomas are classified as mature and immature. Mature teratomas are considered benign tumors. They may be cystic or solid and consist of well-differentiated adult tissues from one or more germ cell layers . EndoderGrading of immature teratomas is based on World Health Organization (WHO) 2014 recommendations. Previously, a three-tier grading system was used, which has been modified to “low grade” (Grade 1) and “high grade” (Grade 2 and Grade 3) immature teratoma .Prognosis depends on certain factors like histological type and grade, patient’s age, anatomic location, the status of surgical resection, and metastatic spread . In the We could not find any paper published on an immature teratoma in the palm so our case is extremely rare and unique in presentation. In conclusion, an extragonadal teratoma may develop in sites other than the midline so it should be considered in the differential diagnosis by clinicians. More cases like this presentation need to be reported after proper workup to understand the etiopathogenesis of extragonadal teratomas well.


A 9-month-old patient developed a small, skin-colored lesion in the conchal bowl of her left ear . There wWhat is nodular fasciitis, and why is this case unique?What other lesions can mimic nodular fasciitis on physical examination?What are distinguishing histologic features of nodular fasciitis on pathologic examination?What is the best way to treat nodular fasciitis to prevent recurrence?Nodular fasciitis is a benign, reactive, tumor-like proliferation of myofibroblastic and fibroblastic cells that most often affects subcutaneous tissue, muscle tissue, and fascia.It can be difficult to differentiate nodular fasciitis from malignancy, both clinically and by imaging. Any rapidly growing solitary mass could suggest a variety of malignant lesions and lead to severe patient anxiety. Nodular fasciitis has been misdiagnosed by contributing pathologists in 75% of the cases studied by Thompson et alThe recommended treatment of nodular fasciitis is complete local excision.In conclusion, nodular fasciitis of the external auditory canal is an exceedingly rare mass that can present both diagnostic and reconstructive challenges for the pathologist and the surgeon, respectively. Appropriate diagnosis can often be difficult to ascertain, considering its features often mimic much more serious malignant conditions. Recurrence is known to be highest in auricular nodular fasciitis; thus, complete local excision must be performed carefully to prevent recurrence.