Abstract
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.
Mulazim Hussain Bukhari, Domenico Coppola, Aejaz Nasir. (2020) Clinicopathologic analysis of primary gastroenteropancreatic poorly differentiated neuroendocrine carcinoma; A ten year retrospective study of 68 cases at Moffit Cancer Center, Pakistan Journal of Medical Sciences, Volume -36, Issue 2.
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