J Cancer 2019; 10(14):3140-3144. doi:10.7150/jca.30355 This issue

Research Paper

The impact of primary tumor site on outcomes of treatment with etoposide and cisplatin in grade 3 gastroenteropancreatic neuroendocrine carcinoma

Sang Eun Yoon1*, Jung Hoon Kim2*, Su Jin Lee1, Jeeyun Lee1, Se Hoon Park1, Joon Oh Park1, Ho Yeong Lim1, Won Ki Kang1, Young Suk Park1, Seung Tae Kim1✉

1. Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
2. Division of Hematology/Oncology, Department of Internal Medicine, School of Medicine Gyeongsang National University, Jinju, Korea
*; Equally contributed to this study.

This is an open access article distributed under the terms of the Creative Commons Attribution (CC BY-NC) license (https://creativecommons.org/licenses/by-nc/4.0/). See http://ivyspring.com/terms for full terms and conditions.
Yoon SE, Kim JH, Lee SJ, Lee J, Park SH, Park JO, Lim HY, Kang WK, Park YS, Kim ST. The impact of primary tumor site on outcomes of treatment with etoposide and cisplatin in grade 3 gastroenteropancreatic neuroendocrine carcinoma. J Cancer 2019; 10(14):3140-3144. doi:10.7150/jca.30355. Available from https://www.jcancer.org/v10p3140.htm

File import instruction


Background: Gastroenteropancreatic neuroendocrine carcinoma (GEP-NEC) is a heterogeneous disease in terms of embryonic origin, aggressiveness, prognosis, and genomic profiling. Data regarding the efficacy of etoposide and cisplatin (EP) as a standard treatment of the primary tumor site in GEP-NEC are limited.

Materials and Methods: We analyzed 64 patients with histopathologically confirmed metastatic GEP-NEC who received EP at Samsung Medical Center, Seoul, Korea, between January 2010 and January 2018. Based on primary tumor site, outcome of treatment with EP was evaluated.

Results: Primary sites included 22 foregut-derived GEP-NECs (stomach, n = 6; duodenum, n = 4; pancreas, n = 12), 4 midgut-derived GEP-NECs, 5 hindgut-derived GEP-NECs of the rectum, 25 GEP-NECs originating from the hepatobiliary (HB) tract, and 12 GEP-NECs involving only intra-abdominal lymph nodes. No patient had a complete response (CR) and 17 had a partial response (PR), resulting in a 27.9% response rate (RR). When evaluating the efficacy of EP based on primary tumor site, the RR was most favorable in GEP-NECs involving only intra-abdominal lymph nodes, followed by GEP-NECs originating from foregut, midgut, HB, and hindgut. However, no statistically significant difference was observed for RR based on primary tumor site (P = 0.821). Similarly, no significant differences were found for progression-free survival (PFS) among patients with GEP-NECs arising from various primary tumor sites.

Conclusion: Results from this study showed that RR and PFS associated with EP treatment were not different based on the primary tumor site in patients with advanced or metastatic GEP-NEC.

Keywords: Gastroenteropancreatic neuroendocrine carcinoma, GEP-NEC, primary tumor site, etoposide, cisplatin