Gastroenteropancreatic Well-Differentiated Grade 3 Neuroendocrine Tumors: Review and Position Statement

Author:

Coriat Romain12,Walter Thomas34,Terris Benoît52,Couvelard Anne67,Ruszniewski Philippe87

Affiliation:

1. Department of Gastroenterology, Cochin Teaching Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France

2. Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité, Paris, France

3. Hospices Civils de Lyon, Hôpital Edouard Herriot, Service d’Oncologie Digestive, Lyon, France

4. Université Claude Bernard Lyon 1, Université de Lyon, Lyon, France

5. Department of Pathology, Cochin Teaching Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France

6. Department of Pathology, Bichat Hospital, Assistance Publique-Hôpitaux de Paris, Départements Hospitalo Universitaires, Paris, France

7. Department of Gastroenterology and Pancreatology, Beaujon Hospital, Assistance Publique-Hôpitaux de Paris, Départements Hospitalo Universitaires, Clichy, France

8. Université Paris Diderot, Sorbonne Paris Cité, Paris, France

Abstract

Abstract In 2010, the World Health Organization (WHO) classification of neuroendocrine neoplasms was reviewed and validated the crucial role of the proliferative rate. According to the WHO classification 2010, gastroenteropancreatic neuroendocrine neoplasms are classified as well-differentiated neuroendocrine tumors (NETs) of grade 1 or 2 in up to 84%, or poorly differentiated neuroendocrine carcinomas in 6%–8%. Neuroendocrine carcinomas are of grade G. Recently, a proportion of neuroendocrine tumors presenting a number of mitoses or a Ki-67 index higher than 20% and a well-differentiated morphology have been identified, calling for a new category, well-differentiated grade 3 NET (NET G-3). Studies that have reported the characteristics of neuroendocrine neoplasms have identified more well-differentiated NET G-3 than neuroendocrine carcinomas. The main localizations of NET G-3 are the pancreas, stomach, and colon. Treatment for NET G-3 is not standardized and is balanced between G-1/2 neuroendocrine tumor and neuroendocrine carcinoma treatments. In nonmetastatic neuroendocrine tumors, the European and American guidelines recommended a surgical resection for localized neuroendocrine neoplasm, irrespective of the tumor grading. In NET G-3, chemotherapy is the benchmark if the main treatment goal is reduction of the tumor mass, particularly if it would allow a secondary surgery. In the present work, we review the epidemiology and make recommendations for the management of NET G-3.

Publisher

Oxford University Press (OUP)

Subject

Cancer Research,Oncology

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