PMID- 33305819 OWN - NLM STAT- MEDLINE DCOM- 20211129 LR - 20240102 IS - 1699-5848 (Electronic) IS - 0213-3911 (Linking) VI - 36 IP - 4 DP - 2021 Apr TI - Pancreatic neuroendocrine neoplasms: Clinicopathological features and pathological staging. PG - 367-382 LID - 10.14670/HH-18-288 [doi] AB - The nomenclature and classification of pancreatic neuroendocrine neoplasms has evolved in the last 15 years based on the advances in knowledge of the genomics, clinical behaviour and response to therapies. The current 2019 World Health Organization classification of pancreatic neuroendocrine neoplasms categorises them into three groups; pancreatic neuroendocrine tumours (PanNETs)(grade 1 grade 2, grade 3), pancreatic neuroendocrine carcinomas and mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) based on the mitotic rate, Ki-67 index, morphological differentiation and/or co-existing tissue subtype. PanNETs are also classified into non-functional NET, insulinoma, gastrinoma, VIPoma, glucagonoma, somatostatinoma, ACTH-producing NET and serotonin producing NET based on hormone production and clinical manifestations. A portion of the cases were associated with genetic syndromes such as multiple neuroendocrine neoplasia 1 (MEN 1), neurofibromatosis and Von Hippel-Lindau syndrome. In view of the distinctive pathology and clinical behaviour of PanNENs, the current 8th AJCC/UICC staging system has separated prognostic staging grouping for PanNETs from the pancreatic neuroendocrine carcinomas or MiNENs. Pancreatic neuroendocrine carcinomas and MiNENs are staged according to the prognostic stage grouping for exocrine pancreatic carcinoma. The new stage grouping of PanNETs was validated to have survival curves separated between different prognostic groups. This refined histological and staging would lead to appropriate selections of treatment strategies for the patients with pancreatic neuroendocrine neoplasms. FAU - Lam, Alfred King-Yin AU - Lam AK AD - School of Medicine, Griffith University, Gold Coast, QLD, Australia. a.lam@griffith.edu.au. FAU - Ishida, Hirotaka AU - Ishida H AD - School of Medicine, Griffith University, Gold Coast, QLD, Australia. LA - eng GR - -/-/ PT - Journal Article PT - Review DEP - 20201211 PL - Spain TA - Histol Histopathol JT - Histology and histopathology JID - 8609357 SB - IM MH - Humans MH - Neoplasm Grading MH - Neoplasm Staging MH - *Neuroendocrine Tumors/classification/diagnosis/pathology MH - *Pancreatic Neoplasms/classification/diagnosis/pathology MH - Prognosis EDAT- 2020/12/12 06:00 MHDA- 2021/11/30 06:00 CRDT- 2020/12/11 08:39 PHST- 2020/12/12 06:00 [pubmed] PHST- 2021/11/30 06:00 [medline] PHST- 2020/12/11 08:39 [entrez] AID - HH-18-288 [pii] AID - 10.14670/HH-18-288 [doi] PST - ppublish SO - Histol Histopathol. 2021 Apr;36(4):367-382. doi: 10.14670/HH-18-288. Epub 2020 Dec 11.