Clinicopathologic analysis of primary gastroenteropancreatic poorly differentiated neuroendocrine carcinoma; A ten year retrospective study of 68 cases at Moffit Cancer Center
Authors: Bukhari Mulazim Hussain , Coppoula Domenico , Nasir Aejaz 
Published: December 2019

Objective: To assess clinicopathological characteristics of primary gastro-entero-pancreatic poorly differentiated neuroendocrine carcinomas (GEP-PDNECAs) and evaluate overall survival in patients treated with systemic platinum and etoposide therapy.

Methods: A detailed retrospective review of clinico-pathologic data (1999-2009) on 68 consecutive adult patients with primary GEP-PDNECAs was carried out, from H Lee Moffit Cancer Center and Research Institute, Tampa, Florida; USA, based on electronic patient records, specialty consultation files, tumor registry, social security index and pathology archives. All available tumor slides were reviewed and subtyped by neuro-endocrine pathologists. Clinicopathologic data and patient survival were analyzed.

Results: Of 68 patients 41 were males and 27 females with a mean age of 42 years (range: 25-76 years). Regarding the site of origin, 39 patients were of the colorectal location, 19 from the pancreas, 04 from small intestines, 03 from stomach and 03 were multi-focal from colon, small intestine and pancreas. Sixty three of 68 (93%) patients presented with lymph node/distant metastases. Of 68 tumors 37 (54%) were classified as small cell carcinoma (SCCA), 16 (24%) large cell carcinoma (LCCA), 5 (7%) mixed small and large cell (MSLCCA) and 10 (15%) poorly differentiated carcinoma with neuroendocrine features (PDCA-NEF). Neuroendocrine differentiation was confirmed by positivity for chromogranin in 38/65 (55%), synaptophysin in 62/67 (92%) and CD56 in 17/21 (81%) cases. One neuroendocrine marker was positive in 22/68 (32%), 2 in 40/68 (59%) and all 3 were positive in 9/68 (13%) cases. Fifty-eight of 68 (85%) patients were treated with platinum and etoposide. Overall patient survival at 1, 3 5 and 10 years was 85%, 40%, 16% and 1.5% respectively. Patient survival was independent of age (r= 0.1022), sex (r= -0.909) and histologic tumor subtype (r=0.1028) (p= 0.128) but was related to distant metastases (r=0.306; p=0.0383).

Conclusions: GEP-PDNECA occurred in many part of the GI tract, most commonly in the colorectal region. Positivity of neuroendocrine markers was variable, which helped to confirm neuro-endocrine differentiation and to avoid under-diagnosis of GEP-PDNECA, especially in metastatic setting. Overall prognosis of GEP-PDNECA patients following platinum and etoposide therapy in our series was relatively favorable but remained poor in the presence of distant metastases.

Note: Abstract of this manuscript was earlier published in “Role of immunohistochemistry in the diagnosis of poorly differentiated neuroendocrine carcinoma”. J Clin Oncol. 2010;28(Suppl-15):e21086-e21086. doi: 10.1200/jco.2010.28.15_suppl.e21086”

doi: https://doi.org/10.12669/pjms.36.2.1336

How to cite this:Bukhari MH, Coppola D, Nasir A. Clinicopathologic analysis of primary gastroenteropancreatic poorly differentiated neuroendocrine carcinoma; A ten year retrospective study of 68 cases at Moffit Cancer Center. Pak J Med Sci. 2020;36(2):265-270.  doi: https://doi.org/10.12669/pjms.36.2.1336

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.