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Small intestinal sarcoma

MedGen UID:
233455
•Concept ID:
C1336007
•
Neoplastic Process
Synonyms: Sarcoma of Small Bowel; sarcoma of small bowel; Sarcoma of Small Intestine; sarcoma of small intestine; Sarcoma of the Small Bowel; sarcoma of the small bowel; Sarcoma of the Small Intestine; sarcoma of the small intestine; Sarcoma, Small Intestinal; sarcoma, small intestinal; Small Bowel Sarcoma; small bowel sarcoma; Small Intestinal Sarcoma; small intestinal sarcoma; Small Intestine Sarcoma; small intestine sarcoma
 
Monarch Initiative: MONDO:0003361

Definition

A malignant soft tissue neoplasm that arises from the small intestine. Representative examples include leiomyosarcoma, angiosarcoma, and Kaposi sarcoma. [from NCI]

Professional guidelines

PubMed

Management of Advanced Small Bowel Cancer.
Puccini A, Battaglin F, Lenz HJ
Curr Treat Options Oncol 2018 Nov 5;19(12):69. doi: 10.1007/s11864-018-0592-3. PMID: 30397729Free PMC Article
Diagnostic and treatment strategy for small gastrointestinal stromal tumors.
Nishida T, Goto O, Raut CP, Yahagi N
Cancer 2016 Oct 15;122(20):3110-3118. Epub 2016 Aug 1 doi: 10.1002/cncr.30239. PMID: 27478963Free PMC Article
Gastrointestinal (GI) leiomyosarcoma (LMS) case series and review on diagnosis, management, and prognosis.
Hilal L, Barada K, Mukherji D, Temraz S, Shamseddine A
Med Oncol 2016 Feb;33(2):20. Epub 2016 Jan 20 doi: 10.1007/s12032-016-0730-3. PMID: 26786155

Recent clinical studies

Etiology

Coffee and cancer risk: a summary overview.
Alicandro G, Tavani A, La Vecchia C
Eur J Cancer Prev 2017 Sep;26(5):424-432. doi: 10.1097/CEJ.0000000000000341. PMID: 28288025
Diagnostic and treatment strategy for small gastrointestinal stromal tumors.
Nishida T, Goto O, Raut CP, Yahagi N
Cancer 2016 Oct 15;122(20):3110-3118. Epub 2016 Aug 1 doi: 10.1002/cncr.30239. PMID: 27478963Free PMC Article
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
Gastrointestinal stromal tumors: pathology and prognosis at different sites.
Miettinen M, Lasota J
Semin Diagn Pathol 2006 May;23(2):70-83. doi: 10.1053/j.semdp.2006.09.001. PMID: 17193820
Gastrointestinal stromal tumours.
Miettinen M, Sarlomo-Rikala M, Lasota J
Ann Chir Gynaecol 1998;87(4):278-81. PMID: 9891765

Diagnosis

Leiomyosarcoma of the Mesentery.
Arhin MA, DeMatos P
Am Surg 2022 Sep;88(9):2210-2211. Epub 2022 Apr 14 doi: 10.1177/00031348221087921. PMID: 35420902
Diagnostic and treatment strategy for small gastrointestinal stromal tumors.
Nishida T, Goto O, Raut CP, Yahagi N
Cancer 2016 Oct 15;122(20):3110-3118. Epub 2016 Aug 1 doi: 10.1002/cncr.30239. PMID: 27478963Free PMC Article
SMALL INTESTINAL TUMORS.
Bozhkov V, Magjov R, Chernopolsky P, Arnaudov P, Plachkov I, Ivanov T
Khirurgiia (Sofiia) 2015;81(2):4-8. PMID: 26668984
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
Small bowel tumors.
Basson MD
Curr Opin Gen Surg 1993:219-24. PMID: 7583974

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
The Nitrogen Mustards.
Highley MS, Landuyt B, Prenen H, Harper PG, De Bruijn EA
Pharmacol Rev 2022 Jul;74(3):552-599. doi: 10.1124/pharmrev.120.000121. PMID: 35710137
Coffee and cancer risk: a summary overview.
Alicandro G, Tavani A, La Vecchia C
Eur J Cancer Prev 2017 Sep;26(5):424-432. doi: 10.1097/CEJ.0000000000000341. PMID: 28288025
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
Small bowel tumors.
Basson MD
Curr Opin Gen Surg 1993:219-24. PMID: 7583974

Prognosis

Diagnostic and treatment strategy for small gastrointestinal stromal tumors.
Nishida T, Goto O, Raut CP, Yahagi N
Cancer 2016 Oct 15;122(20):3110-3118. Epub 2016 Aug 1 doi: 10.1002/cncr.30239. PMID: 27478963Free PMC Article
Gastrointestinal (GI) leiomyosarcoma (LMS) case series and review on diagnosis, management, and prognosis.
Hilal L, Barada K, Mukherji D, Temraz S, Shamseddine A
Med Oncol 2016 Feb;33(2):20. Epub 2016 Jan 20 doi: 10.1007/s12032-016-0730-3. PMID: 26786155
Gastrointestinal stromal tumors: pathology and prognosis at different sites.
Miettinen M, Lasota J
Semin Diagn Pathol 2006 May;23(2):70-83. doi: 10.1053/j.semdp.2006.09.001. PMID: 17193820
Gastrointestinal cancer.
Thomas RM, Sobin LH
Cancer 1995 Jan 1;75(1 Suppl):154-70. doi: 10.1002/1097-0142(19950101)75:1+<154::aid-cncr2820751305>3.0.co;2-z. PMID: 8000994
Small bowel tumors.
Basson MD
Curr Opin Gen Surg 1993:219-24. PMID: 7583974

Clinical prediction guides

Telomerase activation in small intestinal neuroendocrine tumours is associated with aberrant TERT promoter methylation, but not hot-spot mutations.
Fotouhi O, Ghaderi M, Wang N, Zedenius J, Kjellman M, Xu D, Juhlin CC, Larsson C
Epigenetics 2019 Dec;14(12):1224-1233. Epub 2019 Jul 19 doi: 10.1080/15592294.2019.1634987. PMID: 31322481Free PMC Article
Unravelling the pharmacologic opportunities and future directions for targeted therapies in gastro-intestinal cancers part 2: Neuroendocrine tumours, hepatocellular carcinoma, and gastro-intestinal stromal tumours.
Neuzillet C, de Mestier L, Rousseau B, Mir O, Hebbar M, Kocher HM, Ruszniewski P, Tournigand C
Pharmacol Ther 2018 Jan;181:49-75. Epub 2017 Jul 16 doi: 10.1016/j.pharmthera.2017.07.006. PMID: 28723416
SMALL INTESTINAL TUMORS.
Bozhkov V, Magjov R, Chernopolsky P, Arnaudov P, Plachkov I, Ivanov T
Khirurgiia (Sofiia) 2015;81(2):4-8. PMID: 26668984
Retroperitoneal Sarcoma Target Volume and Organ at Risk Contour Delineation Agreement Among NRG Sarcoma Radiation Oncologists.
Baldini EH, Abrams RA, Bosch W, Roberge D, Haas RLM, Catton CN, Indelicato DJ, Olsen JR, Deville C, Chen YL, Finkelstein SE, DeLaney TF, Wang D
Int J Radiat Oncol Biol Phys 2015 Aug 1;92(5):1053-1059. Epub 2015 May 1 doi: 10.1016/j.ijrobp.2015.04.039. PMID: 26194680Free PMC Article
Pediatric intestinal foregut and small bowel solid tumors: a review of 105 cases.
Zhuge Y, Cheung MC, Yang R, Eldick D, Koniaris LG, Sola JE
J Surg Res 2009 Sep;156(1):95-102. Epub 2009 May 3 doi: 10.1016/j.jss.2009.03.054. PMID: 19560163

Recent systematic reviews

Special features of sarcomas developed in patients with Lynch syndrome: A systematic review.
Poumeaud F, Valentin T, Vande Perre P, Jaffrelot M, Bonnet D, Chibon F, Chevreau C, Selves J, Guimbaud R, Fares N
Crit Rev Oncol Hematol 2023 Aug;188:104055. Epub 2023 Jun 8 doi: 10.1016/j.critrevonc.2023.104055. PMID: 37301271
Primary Angiosarcoma of the Gastrointestinal Tract: A Systematic Review of the Literature.
Schizas D, Mastoraki A, Giannakodimos I, Giannakodimos A, Ziogou A, Katsaros I, Frountzas M, Koutelidakis I, Vassiliu P, Pikoulis E
J Invest Surg 2022 Feb;35(2):400-408. Epub 2020 Dec 21 doi: 10.1080/08941939.2020.1853283. PMID: 33342318
Role of palliative resection of the primary tumour in advanced pancreatic and small intestinal neuroendocrine tumours: A systematic review and meta-analysis.
Almond LM, Hodson J, Ford SJ, Gourevitch D, Roberts KJ, Shah T, Isaac J, Desai A
Eur J Surg Oncol 2017 Oct;43(10):1808-1815. Epub 2017 May 25 doi: 10.1016/j.ejso.2017.05.016. PMID: 28583792
Second cancers in patients with male breast cancer: a literature review.
Grenader T, Goldberg A, Shavit L
J Cancer Surviv 2008 Jun;2(2):73-8. Epub 2008 Apr 1 doi: 10.1007/s11764-008-0042-5. PMID: 18648975

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