PMID- 33686307 OWN - NLM STAT- MEDLINE DCOM- 20211028 LR - 20211028 IS - 1591-951X (Electronic) IS - 0031-2983 (Print) IS - 0031-2983 (Linking) VI - 113 IP - 1 DP - 2021 Feb TI - Neuroendocrine neoplasms of the appendix, colon and rectum. PG - 19-27 LID - 10.32074/1591-951X-230 [doi] AB - Neuroendocrine neoplasms of the appendix, colon and rectum are classified according to the most recent WHO classification as neuroendocrine tumors (NET), neuroendocrine carcinomas (NEC) and mixed neuroendocrine-non neuroendocrine neoplasms (MiNENs). NECs and MiNENs are aggressive neoplasms requiring multimodal treatment strategies. By contrast, NETs are, in most cases, indolent lesions occurring as incidental findings in the appendix or as polyps in the rectum. While most appendiceal and rectal NETs are considered relatively non-aggressive neoplasms, a few cases, may show a more aggressive clinical course. Unfortunately, clinical/pathological characteristics to select patients at high risk of recurrence/metastases are poorly consolidated. Diagnosis is generally easy and supported by the combination of morphology and immunohistochemistry. Differential diagnostic problems are for NECs/MiNENs with poorly differentiated adenocarcinomas, when immunohistochemical neuroendocrine markers are not obviously positive, whereas for NETs they are represented by the rare appendiceal tubular and clear cell variants (which may be confused with non-neuroendocrine cancers) and rectal L-cell tumors which may be chromogranin negative and prostatic marker positive. CI - Copyright (c) 2021 Societa Italiana di Anatomia Patologica e Citopatologia Diagnostica, Divisione Italiana della International Academy of Pathology. FAU - Volante, Marco AU - Volante M AD - Department of Oncology, University of Turin, Orbassano, Turin, Italy. AD - Pathology Unit, AOU San Luigi Gonzaga, Orbassano, Turin, Italy. FAU - Grillo, Federica AU - Grillo F AD - Anatomic Pathology, Department of Surgical Sciences and Integrated Diagnostics, University of Genova, Italy. AD - Ospedale Policlinico San Martino IRCCS, Genova, Italy. FAU - Massa, Federica AU - Massa F AD - Pathology Unit, AOU San Luigi Gonzaga, Orbassano, Turin, Italy. FAU - Maletta, Francesca AU - Maletta F AD - Pathology Unit, "Citta della Salute e della Scienza" Hospital, Turin, Italy. FAU - Mastracci, Luca AU - Mastracci L AD - Anatomic Pathology, Department of Surgical Sciences and Integrated Diagnostics, University of Genova, Italy. AD - Ospedale Policlinico San Martino IRCCS, Genova, Italy. FAU - Campora, Michela AU - Campora M AD - Anatomic Pathology, Department of Surgical Sciences and Integrated Diagnostics, University of Genova, Italy. FAU - Ferro, Jacopo AU - Ferro J AD - Anatomic Pathology, Department of Surgical Sciences and Integrated Diagnostics, University of Genova, Italy. FAU - Vanoli, Alessandro AU - Vanoli A AD - Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS San Matteo Hospital, Pavia, Italy. FAU - Papotti, Mauro AU - Papotti M AD - Department of Oncology, University of Turin, Orbassano, Turin, Italy. AD - Pathology Unit, "Citta della Salute e della Scienza" Hospital, Turin, Italy. LA - eng PT - Journal Article PT - Review PL - Italy TA - Pathologica JT - Pathologica JID - 0401123 SB - IM MH - *Appendix MH - *Carcinoma, Neuroendocrine/diagnosis MH - Colon MH - Humans MH - Infant, Newborn MH - Neoplasm Recurrence, Local MH - *Neuroendocrine Tumors/diagnosis MH - Rectum PMC - PMC8138694 OTO - NOTNLM OT - appendix OT - colon OT - neuroendocrine OT - rectum OT - tumor COIS- Conflict of interest The Authors declare no conflict of interest. EDAT- 2021/03/10 06:00 MHDA- 2021/10/29 06:00 PMCR- 2021/02/01 CRDT- 2021/03/09 06:12 PHST- 2020/11/21 00:00 [received] PHST- 2020/11/23 00:00 [accepted] PHST- 2021/03/09 06:12 [entrez] PHST- 2021/03/10 06:00 [pubmed] PHST- 2021/10/29 06:00 [medline] PHST- 2021/02/01 00:00 [pmc-release] AID - 10.32074/1591-951X-230 [doi] PST - ppublish SO - Pathologica. 2021 Feb;113(1):19-27. doi: 10.32074/1591-951X-230.