PMID- 23845449 OWN - NLM STAT- MEDLINE DCOM- 20140303 LR - 20220419 IS - 1479-6821 (Electronic) IS - 1351-0088 (Linking) VI - 20 IP - 5 DP - 2013 Oct TI - Are G3 ENETS neuroendocrine neoplasms heterogeneous? PG - 649-57 LID - 10.1530/ERC-13-0027 [doi] AB - The new WHO classification of gastroenteropancreatic (GEP) neuroendocrine tumors (NET) implies that G3 neoplasms with mitotic index >20 and/or Ki67 index >20% are neuroendocrine carcinomas (NEC), described as poorly differentiated, small or large cell types, by analogy with lung NEC. To characterize the subgroup of non-small-cell-type GEP and thoracic NET with mitotic index >20 and/or Ki67 >20% according to their pathological features, response to cisplatin and overall survival (OS). We reviewed pathological and clinical presentation of G3 non-small-cell-type NET referred to our institution for 5 years. Data from 166 patients with metastatic thoracic and GEP-NET were collected. Twenty-eight patients (17%) fulfill the inclusion criteria. Tumors were classified as well-differentiated NET (G3-WDNET) in 42.8% of cases and poorly differentiated, large-cell NEC (G3-LCNEC) in 57.2% of cases. Plasma chromogranin A or neuron-specific enolase were elevated in 42 and 25% respectively of G3-WDNET and 31 and 50% of G3-LCNEC. Somatostatin receptor scintigraphy was positive in 88 and 50% of G3-WDNET or G3-LCNEC respectively. Complete or partial response to cisplatin was observed in 31% of cases, all classified as G3-LCNEC. The median OS was 41 months for G3-WDNET but 17 months for G3-LCNEC (P=0.34). Short survival was observed in 25% of G3-WDNET but 62.5% of G3-LCNEC patients (P=0.049). G3 ENETS GEP and thoracic neuroendocrine neoplasms (NEN) could constitute a heterogeneous subgroup of NEN as regards diagnosis, prognosis, and treatment. If confirmed, future classifications may consider splitting them into two groups according to their morphological differentiation. FAU - Velayoudom-Cephise, Fritz-Line AU - Velayoudom-Cephise FL AD - Department of Nuclear Medicine and Endocrine Oncology, Villejuif, France. FAU - Duvillard, Pierre AU - Duvillard P FAU - Foucan, Lydia AU - Foucan L FAU - Hadoux, Julien AU - Hadoux J FAU - Chougnet, Cecile N AU - Chougnet CN FAU - Leboulleux, Sophie AU - Leboulleux S FAU - Malka, David AU - Malka D FAU - Guigay, Joel AU - Guigay J FAU - Goere, Diane AU - Goere D FAU - Debaere, Thierry AU - Debaere T FAU - Caramella, Caroline AU - Caramella C FAU - Schlumberger, Martin AU - Schlumberger M FAU - Planchard, David AU - Planchard D FAU - Elias, Dominique AU - Elias D FAU - Ducreux, Michel AU - Ducreux M FAU - Scoazec, Jean-Yves AU - Scoazec JY FAU - Baudin, Eric AU - Baudin E LA - eng PT - Journal Article DEP - 20130819 PL - England TA - Endocr Relat Cancer JT - Endocrine-related cancer JID - 9436481 RN - 0 (Antineoplastic Agents) RN - 0 (Chromogranin A) RN - 0 (SYP protein, human) RN - 0 (Synaptophysin) RN - EC 4.2.1.11 (Phosphopyruvate Hydratase) RN - Q20Q21Q62J (Cisplatin) RN - Gastro-enteropancreatic neuroendocrine tumor SB - IM MH - Adult MH - Aged MH - Antineoplastic Agents/therapeutic use MH - Chromogranin A/metabolism MH - Cisplatin/therapeutic use MH - Female MH - Humans MH - Intestinal Neoplasms/drug therapy/metabolism/*pathology MH - Male MH - Middle Aged MH - Neoplasm Grading MH - Neuroendocrine Tumors/drug therapy/metabolism/*pathology MH - Pancreatic Neoplasms/drug therapy/metabolism/*pathology MH - Phosphopyruvate Hydratase/metabolism MH - Stomach Neoplasms/drug therapy/metabolism/*pathology MH - Synaptophysin/metabolism OTO - NOTNLM OT - Ki67 index OT - WHO classification OT - grade 3 neuroendocrine carcinoma OT - mitotic count EDAT- 2013/07/13 06:00 MHDA- 2014/03/04 06:00 CRDT- 2013/07/13 06:00 PHST- 2013/07/13 06:00 [entrez] PHST- 2013/07/13 06:00 [pubmed] PHST- 2014/03/04 06:00 [medline] AID - ERC-13-0027 [pii] AID - 10.1530/ERC-13-0027 [doi] PST - epublish SO - Endocr Relat Cancer. 2013 Aug 19;20(5):649-57. doi: 10.1530/ERC-13-0027. Print 2013 Oct.