John Libbey Eurotext

Hépato-Gastro & Oncologie Digestive

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Management of gastroenteropancreatic neuroendocrine carcinoma in 2016 Volume 23, issue 4, Avril 2016

Authors
1 Hôpital Edouard Herriot,
Hospices Civils de Lyon,
service d’anatomopathologie,
69437 Lyon cedex 03,
France
2 Hôpital Edouard Herriot,
Hospices Civils de Lyon,
service d’oncologie médicale,
Pavillon E, UJOMM,
69437 Lyon cedex 03,
France
3 Unité INSERM UMR 1052 CNRS UMR 5286,
Centre de Recherche en Cancérologie de Lyon (CRCL),
Équipe 4, Faculté de Médecine RTH Laennec,
69372 Lyon Cedex 08
4 Unité INSERM UMR 1052 CNRS UMR 5286,
Centre de Recherche en Cancérologie de Lyon (CRCL),
Équipe 12, Faculté de Médecine RTH Laennec,
69372 Lyon Cedex 08
* Tirés à part

Small or large cells gastro-enteropancreatic neuroendocrine carcinomas (also known as GEP NEC) are defined as poorly differentiated tumors with a high proliferative index (grade 3 with a Ki67>20%). More than 80% of GEP-NEC are metastatic at diagnosis and their prognosis is poor, as their median overall survival without treatments is below 6 months. They have to be managed rapidly but treatment options are very limited to date. Surgical removal of localized GEP-NEC is very controversial, as associated with very high rate of recurrence. Chemotherapy ± radiotherapy is preferred. Treatment of metastatic diseases relies on platinium-based chemotherapy (cisplatine or carboplatin) associated with etoposide (6 cycles in total). More than 50% of patients have an objective response but relapse occurs for a large majority of them. There is no consensus about maintenance treatment. Second line chemotherapy relies on Folfiri, Folfox or temozolomide protocols, but with a limited effectiveness (around 4 months of progression-free survival). To better manage these specific types of tumors, more knowledge on carcinogenesis and progression/invasion mechanisms of GEP-NEC are required, as well as more inclusion of patients in clinical trials.