Susumu Hijioka, Waki Hosoda, Chigusa Morizane, Nobumasa Mizuno, Kazuo Hara, Takuji Okusaka
Pancreatic neuroendocrine carcinoma (panNEC; pancreatic neuroendocrine neoplasm NEN-G3) is expected to be divided into neuroendocrine tumor-G3 and neuroendocrine carcinoma-G3 in the World Health Organization 2017 classification of tumors of endocrine organs. The distinction is clinically important?£????because the response to chemotherapy differs between the two. Evidence is accumulating on the molecular features of NET-G3, which supports the view that NET-G3 is more closely related to well-differentiated NET. Several molecular markers can be useful in predicting the effectiveness of platinum-based chemotherapies, including high Ki67 labeling index, loss of Rb expression, or KRAS mutation. However, appropriate chemotherapy for NET-G3 remains unclear. Appropriate classification of NET-G3/NEC-G3 and the adequacy of standard treatments depending on this classification are expected in the future with the accumulation of further cases.