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Digestive System Neuroendocrine Carcinoma

MedGen UID:
459354
•Concept ID:
C2987128
•
Neoplastic Process
Synonyms: Gastrointestinal NEC; Gastrointestinal NEC G3; Gastrointestinal Neuroendocrine Carcinoma; Gastrointestinal System Neuroendocrine Carcinoma

Definition

A high grade poorly differentiated carcinoma arising from the digestive system. It is composed of small or large neoplastic cells that express immunohistochemical evidence of neuroendocrine differentiation. There is marked nuclear atypia, necrosis, and more than 20 mitoses per 10 HPF. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDigestive System Neuroendocrine Carcinoma

Professional guidelines

PubMed

Management Strategies and Outcomes for Small Intestinal Neuroendocrine Tumours with Involvement of the Superior Mesenteric Vessels: A Systematic Review.
Kmiotek E, Lakda S, Borakati A, Ogunbiyi O, Mandair D, Caplin M, Toumpanakis C, Mirnezami R
Curr Oncol 2023 Oct 18;30(10):9192-9204. doi: 10.3390/curroncol30100664. PMID: 37887564Free PMC Article
Diagnosis and Management of Gastrointestinal Neuroendocrine Neoplasms.
Gonzalez RS
Surg Pathol Clin 2020 Sep;13(3):377-397. doi: 10.1016/j.path.2020.04.002. PMID: 32773190
The role of endoscopy in the evaluation and management of patients with solid pancreatic neoplasia.
ASGE Standards of Practice Committee, Eloubeidi MA, Decker GA, Chandrasekhara V, Chathadi KV, Early DS, Evans JA, Fanelli RD, Fisher DA, Foley K, Hwang JH, Jue TL, Lightdale JR, Pasha SF, Saltzman JR, Sharaf R, Shergill AK, Cash BD, DeWitt JM
Gastrointest Endosc 2016 Jan;83(1):17-28. doi: 10.1016/j.gie.2015.09.009. PMID: 26706297

Recent clinical studies

Etiology

Management of Gastric Neuroendocrine Tumors: A Review.
Sok C, Ajay PS, Tsagkalidis V, Kooby DA, Shah MM
Ann Surg Oncol 2024 Mar;31(3):1509-1518. Epub 2023 Dec 7 doi: 10.1245/s10434-023-14712-9. PMID: 38062290Free PMC Article
Neoplasms of the Appendix.
Köhler F, Matthes N, Rosenfeldt M, Kunzmann V, Germer CT, Wiegering A
Dtsch Arztebl Int 2023 Aug 7;120(31-32):519-525. doi: 10.3238/arztebl.m2023.0136. PMID: 37282595Free PMC Article
Neuroendocrine neoplasms of the pancreas: diagnosis and pitfalls.
Konukiewitz B, Jesinghaus M, Kasajima A, Klöppel G
Virchows Arch 2022 Feb;480(2):247-257. Epub 2021 Oct 13 doi: 10.1007/s00428-021-03211-5. PMID: 34647171Free PMC Article
Neuroendocrine neoplasms of the esophagus and stomach.
Mastracci L, Rindi G, Grillo F, Solcia E, Campora M, Fassan M, Parente P, Vanoli A, La Rosa S
Pathologica 2021 Feb;113(1):5-11. doi: 10.32074/1591-951X-229. PMID: 33686305Free PMC Article
Types of Gastric Carcinomas.
Waldum HL, Fossmark R
Int J Mol Sci 2018 Dec 18;19(12) doi: 10.3390/ijms19124109. PMID: 30567376Free PMC Article

Diagnosis

Neuroendocrine neoplasms of the pancreas: diagnosis and pitfalls.
Konukiewitz B, Jesinghaus M, Kasajima A, Klöppel G
Virchows Arch 2022 Feb;480(2):247-257. Epub 2021 Oct 13 doi: 10.1007/s00428-021-03211-5. PMID: 34647171Free PMC Article
Neuroendocrine neoplasms of the appendix, colon and rectum.
Volante M, Grillo F, Massa F, Maletta F, Mastracci L, Campora M, Ferro J, Vanoli A, Papotti M
Pathologica 2021 Feb;113(1):19-27. doi: 10.32074/1591-951X-230. PMID: 33686307Free PMC Article
Neuroendocrine neoplasms of the esophagus and stomach.
Mastracci L, Rindi G, Grillo F, Solcia E, Campora M, Fassan M, Parente P, Vanoli A, La Rosa S
Pathologica 2021 Feb;113(1):5-11. doi: 10.32074/1591-951X-229. PMID: 33686305Free PMC Article
Pancreatic Ductal Adenocarcinoma.
Dhillon J, Betancourt M
Monogr Clin Cytol 2020;26:74-91. Epub 2020 Sep 28 doi: 10.1159/000455736. PMID: 32987385
Diagnosis and Management of Gastrointestinal Neuroendocrine Neoplasms.
Gonzalez RS
Surg Pathol Clin 2020 Sep;13(3):377-397. doi: 10.1016/j.path.2020.04.002. PMID: 32773190

Therapy

Aspirin for Metabolic Dysfunction-Associated Steatotic Liver Disease Without Cirrhosis: A Randomized Clinical Trial.
Simon TG, Wilechansky RM, Stoyanova S, Grossman A, Dichtel LE, Lauer GM, Miller KK, Hoshida Y, Corey KE, Loomba R, Chung RT, Chan AT
JAMA 2024 Mar 19;331(11):920-929. doi: 10.1001/jama.2024.1215. PMID: 38502074Free PMC Article
Neoplasms of the Appendix.
Köhler F, Matthes N, Rosenfeldt M, Kunzmann V, Germer CT, Wiegering A
Dtsch Arztebl Int 2023 Aug 7;120(31-32):519-525. doi: 10.3238/arztebl.m2023.0136. PMID: 37282595Free PMC Article
Effectiveness of Etoposide and Cisplatin vs Irinotecan and Cisplatin Therapy for Patients With Advanced Neuroendocrine Carcinoma of the Digestive System: The TOPIC-NEC Phase 3 Randomized Clinical Trial.
Morizane C, Machida N, Honma Y, Okusaka T, Boku N, Kato K, Nomura S, Hiraoka N, Sekine S, Taniguchi H, Okano N, Yamaguchi K, Sato T, Ikeda M, Mizuno N, Ozaka M, Kataoka T, Ueno M, Kitagawa Y, Terashima M, Furuse J; Japan Clinical Oncology Group (JCOG)
JAMA Oncol 2022 Oct 1;8(10):1447-1455. doi: 10.1001/jamaoncol.2022.3395. PMID: 35980649Free PMC Article
Casting a Wide Net.
Cohn A, Nehs M, Chan J, Vaidya A
N Engl J Med 2021 Apr 1;384(13):e50. doi: 10.1056/NEJMimc2028852. PMID: 33789015
Liver ablation therapy.
Gillams AR
Br J Radiol 2004 Sep;77(921):713-23. doi: 10.1259/bjr/86761907. PMID: 15447955

Prognosis

Management of Gastric Neuroendocrine Tumors: A Review.
Sok C, Ajay PS, Tsagkalidis V, Kooby DA, Shah MM
Ann Surg Oncol 2024 Mar;31(3):1509-1518. Epub 2023 Dec 7 doi: 10.1245/s10434-023-14712-9. PMID: 38062290Free PMC Article
Neuroendocrine neoplasms of the appendix, colon and rectum.
Volante M, Grillo F, Massa F, Maletta F, Mastracci L, Campora M, Ferro J, Vanoli A, Papotti M
Pathologica 2021 Feb;113(1):19-27. doi: 10.32074/1591-951X-230. PMID: 33686307Free PMC Article
Neuroendocrine neoplasms of the esophagus and stomach.
Mastracci L, Rindi G, Grillo F, Solcia E, Campora M, Fassan M, Parente P, Vanoli A, La Rosa S
Pathologica 2021 Feb;113(1):5-11. doi: 10.32074/1591-951X-229. PMID: 33686305Free PMC Article
Appendix Tumor Microenvironment.
Roncati L, Gasparri P, Gallo G, Bernardelli G, Zanelli G, Manenti A
Adv Exp Med Biol 2020;1226:87-95. doi: 10.1007/978-3-030-36214-0_7. PMID: 32030678
Types of Gastric Carcinomas.
Waldum HL, Fossmark R
Int J Mol Sci 2018 Dec 18;19(12) doi: 10.3390/ijms19124109. PMID: 30567376Free PMC Article

Clinical prediction guides

Circadian dysfunction induces NAFLD-related human liver cancer in a mouse model.
Padilla J, Osman NM, Bissig-Choisat B, Grimm SL, Qin X, Major AM, Yang L, Lopez-Terrada D, Coarfa C, Li F, Bissig KD, Moore DD, Fu L
J Hepatol 2024 Feb;80(2):282-292. Epub 2023 Oct 27 doi: 10.1016/j.jhep.2023.10.018. PMID: 37890720Free PMC Article
Neuroendocrine neoplasms of the esophagus and stomach.
Mastracci L, Rindi G, Grillo F, Solcia E, Campora M, Fassan M, Parente P, Vanoli A, La Rosa S
Pathologica 2021 Feb;113(1):5-11. doi: 10.32074/1591-951X-229. PMID: 33686305Free PMC Article
Histology of Colorectal Carcinoma: Proven and Purported Prognostic Factors.
Johncilla M, Yantiss RK
Surg Pathol Clin 2020 Sep;13(3):503-520. Epub 2020 Jul 9 doi: 10.1016/j.path.2020.05.008. PMID: 32773197
Appendix Tumor Microenvironment.
Roncati L, Gasparri P, Gallo G, Bernardelli G, Zanelli G, Manenti A
Adv Exp Med Biol 2020;1226:87-95. doi: 10.1007/978-3-030-36214-0_7. PMID: 32030678
Imaging of pancreatic neoplasms.
Balachandran A, Bhosale PR, Charnsangavej C, Tamm EP
Surg Oncol Clin N Am 2014 Oct;23(4):751-88. doi: 10.1016/j.soc.2014.07.002. PMID: 25246049

Recent systematic reviews

Management Strategies and Outcomes for Small Intestinal Neuroendocrine Tumours with Involvement of the Superior Mesenteric Vessels: A Systematic Review.
Kmiotek E, Lakda S, Borakati A, Ogunbiyi O, Mandair D, Caplin M, Toumpanakis C, Mirnezami R
Curr Oncol 2023 Oct 18;30(10):9192-9204. doi: 10.3390/curroncol30100664. PMID: 37887564Free PMC Article
A systematic review and meta-analysis of spectral CT to differentiate focal liver lesions.
Bhandari A, Koppen J, Wastney T, Hacking C
Clin Radiol 2023 Jun;78(6):430-436. Epub 2023 Mar 11 doi: 10.1016/j.crad.2023.02.017. PMID: 37019736
Cancer of the Liver, Intrahepatic Bile Ducts, Gallbladder, Exocrine and Neuroendocrine Pancreas: 20-Year Comparative Survival and Mortality Analysis by Age, Sex, Race, Stage, Grade, Cohort Entry Time-Period, Disease Duration & Selected ICD-O-3 Oncologic Phenotypes A Systematic Review of 367,420 Cases for Diagnosis Years 1973-2014: (SEER*Stat 8.3.4).
Milano AF
J Insur Med 2022 Apr 1;49(2):60-96. doi: 10.17849/insm-49-2-1-36.1. PMID: 35051267
Unexpected Prolonged Survival After Extended and Emergent Resection of Pancreatic Metastases from Renal Cell Carcinoma.
Brozzetti S, Sterpetti AV
J Gastrointest Cancer 2019 Dec;50(4):1055-1058. doi: 10.1007/s12029-019-00209-w. PMID: 30767105
Merkel cell carcinoma: a systematic review of ENT presentations.
Gioacchini FM, Postacchini V, Simonetti O, Offidani A, Magliulo G, Re M
Eur Arch Otorhinolaryngol 2013 Aug;270(8):2191-9. Epub 2012 Nov 29 doi: 10.1007/s00405-012-2283-1. PMID: 23192664

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