A case of aggressive neuroendocrine carcinoma of the stomach

An 18 cm × 16 cm × 10 cm tumor of the stomach, invading the left lobe of the liver, pancreatic body and tail, and transverse colon, with peritoneal deposits on the major omentum, was resected by total gastrectomy plus left hepatic lobectomy, transverse colectomy, distal pancreatectomy, splenectomy, and omentectomy. Histopathologically, the tumor consisted of large uniform cells with significant nuclear atypia, showing solid growth patterns with occasional small nests without adenocarcinoma components. Immunohistochemical investigations of the neoplastic cells confirmed the tumor as a neuroendocrine (NE) carcinoma. Molecular analyses disclosed loss of heterozygosity at the MEN1 gene locus on chromosome 11q13. Recurrence occurred at the hepatic hilus and incurred obstructive jaundice 2 months after surgery. Following percutaneous transhepatic biliary drainage, intensive chemotherapy (20mg/m2 cisplatin on days 1-5 div, 100mg/m2 etoposide on days 1, 3, and 5 div, and 800 mg/m2 5-fluorouracil on days 1-5 bolus iv) was started. The recurrent tumor shrank dramatically, and could not be detected on image modalities after five courses of chemotherapy. The patient was well and free of symptoms without biliary drainage for 5 months. Then he began to present with jaundice again, and died of acute massive dissemination 7 months after surgery. An aggressive form of NE carcinoma has been known to be associated with an extremely poor prognosis. However, it is notable that treatment with extensive surgery and intensive chemotherapy could contribute to an improvement in quality of life even if the beneficial effect lasted for only half a year.