Primary hepatic neuroendocrine tumor: A case report

Abstract

CA 125 and alpha fetoprotein were normal. CT scan: liver mass of 18 cm of maximum diameter encapsulated depending on segments 5 and 6, exophytic, that could correspond to angiosarcoma. Encoscopy and colonoscopy were normal. Surgery was decided, performing anatomic segmentectomy of segments 5e6. The postoperative course was uneventful and the patient was discharged on the sixth postoperative day. Final histology: firm tan-white mass of 3.674 kg, well-defined borders, fleshy cut-surface with foci of cystic degeneration, 19 cm in mean diameter, strong and diffuse cytoplasmic expression of c-Kit (CD-117). Strong expression of DOG-1 and bcl-2 antibodies. No expression of CD-34. Ki-67 expression in a 10% of tumour cells. 6 months after surgery the patient was asymptomatic and without signs of recurrence. Discussion: Although rare, we suggested that GISTs should be considered in the differential diagnosis of hepatic nodules, and that not all hepatic GISTs should automatically be considered to be metastases from a primary gastrointestinal site. Background: Undifferentiated embryonal sarcoma is a rare entity in adulthood, it more typically occurs in children. We report a case of undifferentiated embryonal sarcoma of liver in an adult patient. Case report: A 28 year old female, presented with upper abdominal pain, associated with evening rise of temperature, loss of weight and appetite. No history of jaundice. On examination hepatomegaly was present. Ultrasound abdomen showed a lesion with solid and cystic components in right lobe of liver. On contrast enhanced computed tomography of the abdomen a large cystic lesion was seen in the right lobe of liver with a solid component within it which showed enhancement in delayed phase which was reported as cystic neoplasm of liver. With a preoperative diagnosis of biliary cystadenoma of liver patient was planned for right hepatectomy. Intraoperatively a Cystic lesion with solid component involving the segments VI, VII & VIII of liver was noted with haemorrhagic fluid within the cyst, left lobe was normal. A right hepatectomy was done. Patient had an uneventful postoperative period and was discharged by 7 th postoperative day. The histopathological examination of the specimen was reported as undifferentiated embryonal sarcoma of the liver. Conclusion: Undifferentiated embryonal sarcoma of liver in the adult population is a rare entity. Patients present with nonspecific symptoms and the radiological features are inconclusive. Surgical resection isnecessary for histological assessment and complete tumourremoval. Embryonal sarcomas have generally poor prognosis;however surgical resection and adjuvant chemoradiationoffer the best chance for longterm survival.