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Gastrointestinal Stromal Tumor of the Gastrointestinal Tract

MedGen UID:
232555
Concept ID:
C1333802
Neoplastic Process
Synonyms: Digestive Stromal Neoplasm; Digestive Stromal Tumor; Digestive Tract Stromal Neoplasm; Digestive Tract Stromal Tumor; Gastrointestinal Stromal Tumor of Gastrointestinal Tract; Gastrointestinal Tract Stromal Neoplasm; Gastrointestinal Tract Stromal Tumor; Stromal Neoplasm of Digestive Tract; Stromal Neoplasm of Gastrointestinal Tract; Stromal Neoplasm of the Digestive Tract; Stromal Neoplasm of the Gastrointestinal Tract; Stromal Tumor of Digestive Tract; Stromal Tumor of Gastrointestinal Tract; Stromal Tumor of the Digestive Tract; Stromal Tumor of the Gastrointestinal Tract

Definition

A stromal tumor that originates from the interstitial cells of Cajal. It may involve any part of the gastrointestinal tract. The majority of cases occur in the stomach. Morphologic variants include the epithelioid cell type, spindle cell type, and mixed cell type. Most cases contain KIT- or PDGFRA-activating mutations. Lesions with a diameter greater than 10cm and more than 5 mitotic figures per 50HPF tend to have a more aggressive clinical course. Until recently, surgery has been the only effective therapy for this tumor. However, many patients still experience recurrence. A KIT tyrosine kinase inhibitor, imatinib mesylate (also known as STI-571 or Gleevec), is now effective in the treatment of relapsed and unresectable cases. [from NCI]

Professional guidelines

PubMed

Bamboat ZM, Dematteo RP
Surg Oncol Clin N Am 2012 Apr;21(2):301-16. doi: 10.1016/j.soc.2011.12.004. PMID: 22365521Free PMC Article

Recent clinical studies

Etiology

Deprez PH, Moons LMG, OʼToole D, Gincul R, Seicean A, Pimentel-Nunes P, Fernández-Esparrach G, Polkowski M, Vieth M, Borbath I, Moreels TG, Nieveen van Dijkum E, Blay JY, van Hooft JE
Endoscopy 2022 Apr;54(4):412-429. Epub 2022 Feb 18 doi: 10.1055/a-1751-5742. PMID: 35180797
Sbaraglia M, Businello G, Bellan E, Fassan M, Dei Tos AP
Pathologica 2021 Jun;113(3):230-251. doi: 10.32074/1591-951X-309. PMID: 34294940Free PMC Article
Costa F, Casaca R, Monteiro C, Ramos P
BMJ Case Rep 2020 Oct 31;13(10) doi: 10.1136/bcr-2020-238058. PMID: 33130586Free PMC Article
Keung EZ, Raut CP
Surg Clin North Am 2017 Apr;97(2):437-452. doi: 10.1016/j.suc.2016.12.001. PMID: 28325196
Miettinen M, Lasota J
Semin Diagn Pathol 2006 May;23(2):70-83. doi: 10.1053/j.semdp.2006.09.001. PMID: 17193820

Diagnosis

Deprez PH, Moons LMG, OʼToole D, Gincul R, Seicean A, Pimentel-Nunes P, Fernández-Esparrach G, Polkowski M, Vieth M, Borbath I, Moreels TG, Nieveen van Dijkum E, Blay JY, van Hooft JE
Endoscopy 2022 Apr;54(4):412-429. Epub 2022 Feb 18 doi: 10.1055/a-1751-5742. PMID: 35180797
Kelly CM, Gutierrez Sainz L, Chi P
J Hematol Oncol 2021 Jan 5;14(1):2. doi: 10.1186/s13045-020-01026-6. PMID: 33402214Free PMC Article
Akahoshi K, Oya M, Koga T, Shiratsuchi Y
World J Gastroenterol 2018 Jul 14;24(26):2806-2817. doi: 10.3748/wjg.v24.i26.2806. PMID: 30018476Free PMC Article
Stamatakos M, Douzinas E, Stefanaki C, Safioleas P, Polyzou E, Levidou G, Safioleas M
World J Surg Oncol 2009 Aug 1;7:61. doi: 10.1186/1477-7819-7-61. PMID: 19646278Free PMC Article
Miettinen M, Lasota J
Arch Pathol Lab Med 2006 Oct;130(10):1466-78. doi: 10.5858/2006-130-1466-GSTROM. PMID: 17090188

Therapy

Li B, Chen H, Yang S, Chen F, Xu L, Li Y, Li M, Zhu C, Shao F, Zhang X, Deng C, Zeng L, He Y, Zhang C
Mol Cancer 2023 Apr 18;22(1):71. doi: 10.1186/s12943-023-01770-6. PMID: 37072770Free PMC Article
Naito Y, Nishida T, Doi T
Gastric Cancer 2023 May;26(3):339-351. Epub 2023 Mar 13 doi: 10.1007/s10120-023-01381-6. PMID: 36913072Free PMC Article
Kelly CM, Gutierrez Sainz L, Chi P
J Hematol Oncol 2021 Jan 5;14(1):2. doi: 10.1186/s13045-020-01026-6. PMID: 33402214Free PMC Article
Akahoshi K, Oya M, Koga T, Shiratsuchi Y
World J Gastroenterol 2018 Jul 14;24(26):2806-2817. doi: 10.3748/wjg.v24.i26.2806. PMID: 30018476Free PMC Article
von Mehren M, Joensuu H
J Clin Oncol 2018 Jan 10;36(2):136-143. Epub 2017 Dec 8 doi: 10.1200/JCO.2017.74.9705. PMID: 29220298Free PMC Article

Prognosis

Li B, Chen H, Yang S, Chen F, Xu L, Li Y, Li M, Zhu C, Shao F, Zhang X, Deng C, Zeng L, He Y, Zhang C
Mol Cancer 2023 Apr 18;22(1):71. doi: 10.1186/s12943-023-01770-6. PMID: 37072770Free PMC Article
Akahoshi K, Oya M, Koga T, Shiratsuchi Y
World J Gastroenterol 2018 Jul 14;24(26):2806-2817. doi: 10.3748/wjg.v24.i26.2806. PMID: 30018476Free PMC Article
Miettinen M, Lasota J
Gastroenterol Clin North Am 2013 Jun;42(2):399-415. Epub 2013 Mar 13 doi: 10.1016/j.gtc.2013.01.001. PMID: 23639648Free PMC Article
Miettinen M, Lasota J
Semin Diagn Pathol 2006 May;23(2):70-83. doi: 10.1053/j.semdp.2006.09.001. PMID: 17193820
Miettinen M, Lasota J
Arch Pathol Lab Med 2006 Oct;130(10):1466-78. doi: 10.5858/2006-130-1466-GSTROM. PMID: 17090188

Clinical prediction guides

Khan SI, O'Sullivan NJ, Temperley HC, Rausa E, Mehigan BJ, McCormick P, Larkin JO, Kavanagh DO, Kelly ME
Curr Oncol 2022 Dec 28;30(1):416-429. doi: 10.3390/curroncol30010034. PMID: 36661683Free PMC Article
Davila RE
Gastroenterol Clin North Am 2022 Sep;51(3):609-624. Epub 2022 Aug 29 doi: 10.1016/j.gtc.2022.06.009. PMID: 36153113
Cannella R, La Grutta L, Midiri M, Bartolotta TV
World J Gastroenterol 2020 Aug 28;26(32):4729-4738. doi: 10.3748/wjg.v26.i32.4729. PMID: 32921953Free PMC Article
Grothey A, Blay JY, Pavlakis N, Yoshino T, Bruix J
Cancer Treat Rev 2020 Jun;86:101993. Epub 2020 Feb 20 doi: 10.1016/j.ctrv.2020.101993. PMID: 32199197
Miettinen M, Lasota J
Arch Pathol Lab Med 2006 Oct;130(10):1466-78. doi: 10.5858/2006-130-1466-GSTROM. PMID: 17090188

Recent systematic reviews

Khan SI, O'Sullivan NJ, Temperley HC, Rausa E, Mehigan BJ, McCormick P, Larkin JO, Kavanagh DO, Kelly ME
Curr Oncol 2022 Dec 28;30(1):416-429. doi: 10.3390/curroncol30010034. PMID: 36661683Free PMC Article
Raitio A, Salim A, Mullassery D, Losty PD
Pediatr Surg Int 2021 Sep;37(9):1161-1165. Epub 2021 Jun 3 doi: 10.1007/s00383-021-04931-0. PMID: 34081161Free PMC Article
Waidhauser J, Bornemann A, Trepel M, Märkl B
World J Gastroenterol 2019 Aug 14;25(30):4261-4277. doi: 10.3748/wjg.v25.i30.4261. PMID: 31435178Free PMC Article
Mori H, Kobara H, Nishiyama N, Masaki T
Dig Endosc 2018 Apr;30 Suppl 1:25-31. doi: 10.1111/den.13042. PMID: 29658644
Søreide K, Sandvik OM, Søreide JA, Giljaca V, Jureckova A, Bulusu VR
Cancer Epidemiol 2016 Feb;40:39-46. Epub 2015 Nov 24 doi: 10.1016/j.canep.2015.10.031. PMID: 26618334

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