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Gastrointestinal stromal tumor of small intestine

MedGen UID:
234790
•Concept ID:
C1335996
•
Neoplastic Process
Synonym: Small Intestinal Gastrointestinal Stromal Tumor
SNOMED CT: Gastrointestinal stromal tumor of small intestine (445737002)

Definition

A gastrointestinal stromal tumor that arises from the small intestine. It usually affects adults over fifty years of age. The majority of cases have spindle cell morphology. The prognosis depends on the tumor size and the mitotic activity. [from NCI]

Professional guidelines

PubMed

Updates in the diagnosis and management of small-bowel tumors.
Vlachou E, Koffas A, Toumpanakis C, Keuchel M
Best Pract Res Clin Gastroenterol 2023 Jun-Aug;64-65:101860. Epub 2023 Aug 12 doi: 10.1016/j.bpg.2023.101860. PMID: 37652650
Progress in the Treatment of Small Intestine Cancer.
Symons R, Daly D, Gandy R, Goldstein D, Aghmesheh M
Curr Treat Options Oncol 2023 Apr;24(4):241-261. Epub 2023 Feb 24 doi: 10.1007/s11864-023-01058-3. PMID: 36826686
Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis.
Miettinen M, Lasota J
Arch Pathol Lab Med 2006 Oct;130(10):1466-78. doi: 10.5858/2006-130-1466-GSTROM. PMID: 17090188

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Gastrointestinal Stromal Tumors, 2023

Recent clinical studies

Etiology

Incidence and Survival Outcomes of Gastrointestinal Stromal Tumors.
Alvarez CS, Piazuelo MB, Fleitas-Kanonnikoff T, Ruhl J, Pérez-Fidalgo JA, Camargo MC
JAMA Netw Open 2024 Aug 1;7(8):e2428828. doi: 10.1001/jamanetworkopen.2024.28828. PMID: 39158910Free PMC Article
Updates in the diagnosis and management of small-bowel tumors.
Vlachou E, Koffas A, Toumpanakis C, Keuchel M
Best Pract Res Clin Gastroenterol 2023 Jun-Aug;64-65:101860. Epub 2023 Aug 12 doi: 10.1016/j.bpg.2023.101860. PMID: 37652650
Gastrointestinal Stromal Tumours.
Sunamak O, Gul MO, Subasi IE, Donmez T, Ozkara S
J Coll Physicians Surg Pak 2021 Sep;31(9):1089-1093. doi: 10.29271/jcpsp.2021.09.1089. PMID: 34500527
Gastrointestinal stromal tumour.
Joensuu H, Hohenberger P, Corless CL
Lancet 2013 Sep 14;382(9896):973-83. Epub 2013 Apr 24 doi: 10.1016/S0140-6736(13)60106-3. PMID: 23623056
Risk stratification of patients diagnosed with gastrointestinal stromal tumor.
Joensuu H
Hum Pathol 2008 Oct;39(10):1411-9. doi: 10.1016/j.humpath.2008.06.025. PMID: 18774375

Diagnosis

Updates in the diagnosis and management of small-bowel tumors.
Vlachou E, Koffas A, Toumpanakis C, Keuchel M
Best Pract Res Clin Gastroenterol 2023 Jun-Aug;64-65:101860. Epub 2023 Aug 12 doi: 10.1016/j.bpg.2023.101860. PMID: 37652650
Gastrointestinal Stromal Tumours.
Sunamak O, Gul MO, Subasi IE, Donmez T, Ozkara S
J Coll Physicians Surg Pak 2021 Sep;31(9):1089-1093. doi: 10.29271/jcpsp.2021.09.1089. PMID: 34500527
Jejunal Gastrointestinal Stromal Tumor.
Yousef AJA
Mayo Clin Proc 2017 Oct;92(10):1602. doi: 10.1016/j.mayocp.2017.08.011. PMID: 28982490
Risk stratification of patients diagnosed with gastrointestinal stromal tumor.
Joensuu H
Hum Pathol 2008 Oct;39(10):1411-9. doi: 10.1016/j.humpath.2008.06.025. PMID: 18774375
Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis.
Miettinen M, Lasota J
Arch Pathol Lab Med 2006 Oct;130(10):1466-78. doi: 10.5858/2006-130-1466-GSTROM. PMID: 17090188

Therapy

Dabrafenib plus trametinib in BRAFV600E-mutated rare cancers: the phase 2 ROAR trial.
Subbiah V, Kreitman RJ, Wainberg ZA, Gazzah A, Lassen U, Stein A, Wen PY, Dietrich S, de Jonge MJA, Blay JY, Italiano A, Yonemori K, Cho DC, de Vos FYFL, Moreau P, Fernandez EE, Schellens JHM, Zielinski CC, Redhu S, Boran A, Passos VQ, Ilankumaran P, Bang YJ
Nat Med 2023 May;29(5):1103-1112. Epub 2023 Apr 14 doi: 10.1038/s41591-023-02321-8. PMID: 37059834Free PMC Article
Progress in the Treatment of Small Intestine Cancer.
Symons R, Daly D, Gandy R, Goldstein D, Aghmesheh M
Curr Treat Options Oncol 2023 Apr;24(4):241-261. Epub 2023 Feb 24 doi: 10.1007/s11864-023-01058-3. PMID: 36826686
Jejunal Gastrointestinal Stromal Tumor.
Yousef AJA
Mayo Clin Proc 2017 Oct;92(10):1602. doi: 10.1016/j.mayocp.2017.08.011. PMID: 28982490
Beyond cytology: why and when does the oncologist require core tissue?
de la Fuente SG, Arnoletti JP
Gastrointest Endosc Clin N Am 2014 Jan;24(1):9-17. Epub 2013 Sep 21 doi: 10.1016/j.giec.2013.08.001. PMID: 24215757
Gastrointestinal stromal tumour.
Joensuu H, Hohenberger P, Corless CL
Lancet 2013 Sep 14;382(9896):973-83. Epub 2013 Apr 24 doi: 10.1016/S0140-6736(13)60106-3. PMID: 23623056

Prognosis

Incidence and Survival Outcomes of Gastrointestinal Stromal Tumors.
Alvarez CS, Piazuelo MB, Fleitas-Kanonnikoff T, Ruhl J, Pérez-Fidalgo JA, Camargo MC
JAMA Netw Open 2024 Aug 1;7(8):e2428828. doi: 10.1001/jamanetworkopen.2024.28828. PMID: 39158910Free PMC Article
Clinical and Morphological Characteristics of Gastrointestinal Stromal Tumor.
Munteanu A, Patrascu S, Bordu S, Laskou S, Surlin V, Radu P
Chirurgia (Bucur) 2023 Dec;118(6):618-623. doi: 10.21614/chirurgia.2023.v.118.i.6.p.618. PMID: 38228594
Gastrointestinal Stromal Tumours.
Sunamak O, Gul MO, Subasi IE, Donmez T, Ozkara S
J Coll Physicians Surg Pak 2021 Sep;31(9):1089-1093. doi: 10.29271/jcpsp.2021.09.1089. PMID: 34500527
Risk stratification of patients diagnosed with gastrointestinal stromal tumor.
Joensuu H
Hum Pathol 2008 Oct;39(10):1411-9. doi: 10.1016/j.humpath.2008.06.025. PMID: 18774375
Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis.
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

Clinical features and prognosis of malignant small bowel tumors: Experience from a university hospital in Chile.
Silva F, Bustamante M, Latorre G, Flandez J, Montero I, Dukes E, Gandara V, Robles C, Uribe J, Iglesias A, Bellolio F, Molina ME, Migueles R, Urrejola G, Larach T, Besser N, Sharp A, Agüero C, Riquelme A, Vargas JI, Candia R, Monrroy H, De Simone F, Espino A
Gastroenterol Hepatol 2024 May;47(5):491-499. Epub 2024 Feb 6 doi: 10.1016/j.gastrohep.2024.01.013. PMID: 38331316
Gastrointestinal Stromal Tumours of the Small Intestine.
Uzunoglu H, Tosun Y, Akinci O, Baris B
J Coll Physicians Surg Pak 2021 Dec;31(12):1487-1493. doi: 10.29271/jcpsp.2021.12.1487. PMID: 34794293
KIT gene mutations in gastrointestinal stromal tumor.
Kang W, Zhu C, Yu J, Ye X, Ma Z
Front Biosci (Landmark Ed) 2015 Jun 1;20(6):919-26. doi: 10.2741/4346. PMID: 25961532
Small intestine gastrointestinal stromal tumors.
Grover S, Ashley SW, Raut CP
Curr Opin Gastroenterol 2012 Mar;28(2):113-23. doi: 10.1097/MOG.0b013e32834ec154. PMID: 22157511
Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis.
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

Current treatment and outcomes of pediatric gastrointestinal stromal tumors (GIST): a systematic review of published studies.
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
Gastrointestinal Stromal Tumours (GIST): A Review of Cases from Nigeria.
Ogun GO, Adegoke OO, Rahman A, Egbo OH
J Gastrointest Cancer 2020 Sep;51(3):729-737. doi: 10.1007/s12029-019-00318-6. PMID: 31659675
Pancreaticoduodenectomy versus limited resection for duodenal gastrointestinal stromal tumors: a systematic review and meta-analysis.
Shen Z, Chen P, Du N, Khadaroo PA, Mao D, Gu L
BMC Surg 2019 Aug 28;19(1):121. doi: 10.1186/s12893-019-0587-4. PMID: 31455328Free PMC Article
Stromal tumor of the ampulla of vater: report of a case and systematic review of reported cases.
Jerraya H, Bel Haj Salah R, Ben Mena K, Haouet K, Zaouche A
Tunis Med 2009 Sep;87(9):556-9. PMID: 20180372

Supplemental Content

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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Gastrointestinal Stromal Tumors, 2023

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    Reviews

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