No abstract

BackgroundClear cell sarcoma (CCS), also known as malignant melanoma of soft parts, is a rare type of soft tissue sarcoma which exhibits morphological, immunohistochemical and ultrastructural similarity with malignant melanoma. It is rarely localized in the intestine and the natural history of this tumor is not yet clear.Case reportA 49-year-old woman presented with diffuse abdominal colicky pain and vomitus over the previous seven days. An X-ray of the abdomen revealed obstruction of the small intestine. The patient underwent contrast enhanced abdominal computerized tomography (CT), which confirmed the obstruction at the jejunum and an associated circumferential wall thickening extending about 3 cm in length, causing concentric narrowing of the lumen. At laparotomy, a mass was recognized at the level of the jejunum in the small intestine, which caused almost complete obstruction of the lumen. At the point of obstruction, adhered loops of small intestine were found. A segmental small bowel resection was performed with 5 cm clear margins and its respective mesenteric lymph nodes.ResultsHistological examination of the specimen revealed a tumor (3×3×2cm) with epithelioid cell characteristics and eosinophilic or clear cytoplasm and focal translucent nuclei. Immunohistochemistry was positive for S100, epithelial membrane antigen (EMA) and synaptophysin. The tumor was pankeratin AE1/AE2, GFAP, HMB45 and MART-1/Melan-A negative. Twelve lymph nodes were retrieved and were free of neoplastic infiltration. Cytogenetic examination revealed translocation of the EWSR1 gene. The patient had an uncomplicated postoperative course and left the hospital seven days after her admission in good general condition. After 20 months of follow-up the patient remains asymptomatic without any clinical or radiological evidence of recurrence.ConclusionCCS sarcoma can be rarely localized in the jejunum. Due to its morphological similarity to malignant melanoma, cytogenetic examination is necessary for its diagnosis. Wide resection of the tumor and its respective lymph nodes was associated with a 20-month disease free survival in this patient.

Multiple myeloma is considered a hypercoagulable state due to several mechanisms such as the increased IL-6 and immunoglobulins production, the defective fibrinolytic mechanism, and the acquired resistance to activated protein C that are involved in the pathogenesis and clinical futures of the disease. We describe a case of a female patient who presented to the hospital with pulmonary embolism as the first manifestation of the hypercoagulability of multiple myeloma.

Bronchobiliary fistula (BBF) is a rare complication following hepatectomy, and consists of an abnormal intercommunication between the biliary tract and bronchial tree. The management of this rare entity is challenging with limited current evidence to date on how to treat this condition. Herein, a case of BBF following a central hepatectomy and the successive steps of the management was presented. Fourteen months postoperatively, the patient presented to the Oncology Department complaining of new onset fever and expectoration. A computerized tomography scan revealed a BBF and the patient was subsequently referred to our department for further treatment. The surgical team decided that a further operation was required. Using the transabdominal approach, a communication between the initial intrahepatic collection and one inferior lobular bronchus was revealed. Transhiatal removal of fistula was performed with closure of the defect through the abdomen. There was no bile leak through the remaining liver parenchyma. Multidisciplinary management should be considered, taking into consideration the underlying pathology leading to this rare complication. Conservative treatment should be considered first, while surgical resection of the BBF remains an option when other therapies have failed. Surgeons should be aware of this rare complication bile duct injuries during hepatic operations can cause.