The role of chemotherapy and/or octreotide in patients with metastatic gastroenteropancreatic and hepatobiliary neuroendocrine carcinoma

Background: Neuroendocrine carcinomas (NECs) of the gastro-entero-pancreatic (GEP) and hepatobiliary (HB) tract are rare and a heterogenous group of malignancies. Octreotide showed the anti-tumor activity in functional and nonfunctional well differentiated metastatic midgut neuroendocrine tumors (NETs). However, the effect of octreotide on survival has not been evaluated. Patients and methods: We analyzed 17 patients (6 HB- and 11 GEP- tract) with metastatic NEC diagnosed between January 2009 and June 2012. All patients had one or more cytotoxic chemotherapy and nine patients had received octreotide as single agent (n=3) or combination of cytotoxic chemotherapy (n=6). Results: The median age was 68 years (range, 23-79 years) and median Eastern Cooperative Oncology Group performance status (ECOG PS) was 1. Sixteen of all patients (n=17) received cytotoxic chemotherapy with or without octreotide as the first line therapy and 10 of 16 patients who experienced disease progression to the first line therapy received the second line therapy. Overall response rates (RR) and disease control rates (DCR) to the 1^st line therapy were 41.2% and 76.5%, respectively. The median overall survival (OS) was 16 months [95% confidence interval (CI), 12.8-19.2] and the median OS in patients receiving octreotide during treatment was 40.2 months. In univariate analysis, any clinico-pathologic features including sex, the location of primary tumor, the number of metastatic sites, the debulking operation and the liver metastasis did not have prognostic value regarding OS. However, the use of octreotide offered favorable trend for OS (P=0.091). Conclusions: The use of octreotide may benefit for patients with GEP- and HB- NECs as a single agent or a combination therapy.