Management of neuroendocrine carcinomas of the pancreas (WHO G3): A tailored approach between proliferation and morphology

• Published in: World journal of gastroenterology : WJG Vol. 22; no. 45; pp. 9944 - 9953
• Main Authors: Crippa, Stefano, Partelli, Stefano, Belfiori, Giulio, Palucci, Marco, Muffatti, Francesca, Adamenko, Olga, Cardinali, Luca, Doglioni, Claudio, Zamboni, Giuseppe, Falconi, Massimo
• Format: Journal Article
• Language: English
• Published: United States Baishideng Publishing Group Inc 07.12.2016
• Subjects: Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Carboplatin - administration & dosage; Carcinoma, Neuroendocrine - diagnostic imaging; Carcinoma, Neuroendocrine - pathology; Carcinoma, Neuroendocrine - therapy; Cell Proliferation; Chemotherapy, Adjuvant; Cisplatin - administration & dosage; Etoposide - administration & dosage; Humans; Ki-67 Antigen; Minireviews; Mitotic Index; Neoadjuvant Therapy; Neoplasm Staging; Neuroendocrine Tumors - diagnostic imaging; Neuroendocrine Tumors - pathology; Neuroendocrine Tumors - therapy; Pancreatectomy; Pancreatic Neoplasms - diagnostic imaging; Pancreatic Neoplasms - pathology; Pancreatic Neoplasms - therapy; Prognosis; Chemotherapy; Prognosis; Morphology; Neuroendocrine carcinomas; Surgery; Pancreatic neuroendocrine tumors; Proliferation; Metastases
• ISSN: 1007-9327; 2219-2840
• DOI: 10.3748/wjg.v22.i45.9944

Neuroendocrine carcinomas(NEC) of the pancreas are defined by a mitotic count > 20 mitoses/10 high power fields and/or Ki67 index > 20%, and included all the tumors previously classified as poorly differentiated endocrine carcinomas. These latter are aggressive malignancies with a high propensity for distant metastases and poor prognosis, and they can be further divided into small- and large-cell subtypes. However in the NEC category are included also neuroendocrine tumors with a well differentiated morphology but ki67 index > 20%. This category is associated with better prognosis and does not significantly respond to cisplatin-based chemotherapy, which represents the gold standard therapeutic approach for poorly differentiated NEC. In this review, the differences between well differentiated and poorly differentiated NEC are discussed considering both pathology, imaging features, treatment and prognostic implications. Diagnostic and therapeutic flowcharts are proposed. The need for a revision of current classification system is stressed being well differentiated NEC a more indolent disease compared to poorly differentiated tumors.