Right hepatectomy for combined primary neuroendocrine and hepatocellular carcinoma. A case report
A 51-year-old man presented to our outpatient clinic with a 9-month history of dull aching abdominal pain and a medical history of hepatitis C. Periodic hepatic ultrasound follow-up showed a mass in the right hemiliver. A CT scan of the abdomen revealed a 20 cm × 15 cm mass occupying most of the hemiliver with cystic degeneration and calcification and multiple dilated collaterals related to the mass (). The mass was compressing the right portal vein, yet no invasion detected (). CT volumetry estimated the residual left hemiliver volume including the middle hepatic vein to be 1300 cc. Serum level of alpha-fetoprotein (AFP) was slightly elevated (2.3 ng/ml). The patient had normal liver enzymes, total serum bilirubin of 1.2 mg/dl, serum albumin of 3.3 mg/dl and INR of 1.1. CA19-9 and CA125 were normal. CT of chest and extrahepatic abdomen showed no other lesions.\nThe patient underwent laparotomy through a J shaped incision (). On exploration the mass was found to be capsulated, 20 cm × 15 cm occupying most of the right hemiliver with multiple dilated collaterals extending from the mass to the diaphragm and the hepatic flexure of the colon. No ascites was found. No peritoneal nodules or significant lymph nodes. Right hepatectomy was done. Blood loss was 2500 cc and the patient received 4 units of blood intraoperatively due to presence of collaterals and large size of the mass making mobilization and resection of the right lobe technically challenging. The patient had an eventless post-operative course and was discharged on the 6th post-operative day.\nThe resected specimen measured 20 cm × 15 cm and weighed 4500 g. Cut section revealed a well-defined heterogeneous gray-white to brown friable mass 7.5 cm in diameter (). The surrounding liver was non-cirrhotic.\nMicroscopically, the tumor consisted predominantly of monotonous small- to medium-sized neoplastic cells arranged in trabeculea separated by sinusoidal spaces. Tumor cells had round to oval punctuate nuclei within conspicuous nucleoli and scanty cytoplasm (A). The tumor was accompanied by wide areas of geographic necrosis with high mitotic rate that ranged between 2 and 20/10 HPF. The whole morphological picture was reminiscent of high grade NEC. The surrounding liver tissue revealed chronic hepatitis without cirrhotic changes.\nImmunohistochemically, the tumor cells were strongly positive for synaptophysin (D) and focally positive for chromogranin-A. Interestingly, the tumor cells showed patchy positive coarse granular staining for HerPar-1 in less than 1% of tumor cells (B and C) while Glypican-3staining was negative. The immunohistochemical findings supported the diagnosis of combined high grade NEC and HCC. Follow up period was short but no recurrence detected up to 6 months after surgery.