TY - JOUR
T1 - Management of neuroendocrine carcinomas of the pancreas (WHO G3)
T2 - A tailored approach between proliferation and morphology
AU - Crippa, Stefano
AU - Partelli, Stefano
AU - Belfiori, Giulio
AU - Palucci, Marco
AU - Muffatti, Francesca
AU - Adamenko, Olga
AU - Cardinali, Luca
AU - Doglioni, Claudio
AU - Zamboni, Giuseppe
AU - Falconi, Massimo
PY - 2016/12/7
Y1 - 2016/12/7
N2 - 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.
AB - 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.
KW - Chemotherapy
KW - Metastases
KW - Morphology
KW - Neuroendocrine carcinomas
KW - Pancreatic neuroendocrine tumors
KW - Prognosis
KW - Proliferation
KW - Surgery
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U2 - 10.3748/wjg.v22.i45.9944
DO - 10.3748/wjg.v22.i45.9944
M3 - Review article
AN - SCOPUS:85003031556
VL - 22
SP - 9944
EP - 9953
JO - World Journal of Gastroenterology
JF - World Journal of Gastroenterology
SN - 1007-9327
IS - 45
ER -