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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]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVSmall intestinal sarcoma

Disorder of digestive system
- Abnormality of the digestive system
  - Digestive system neoplasm
    - Neoplasm of the gastrointestinal tract
      - Digestive System Soft Tissue Neoplasm
        Digestive System Sarcoma
        Small intestinal sarcoma
        Duodenal Extraskeletal Osteosarcoma
        Small Intestinal Angiosarcoma
        Small intestinal fibrosarcoma
        Small Intestinal Kaposi Sarcoma
        Small intestine leiomyosarcoma
        Childhood Small Intestinal Leiomyosarcoma

Professional guidelines

PubMed

Assessment of Gender-Specific COVID-19 Case Fatality Risk per Malignant Neoplasm Type.

Matsuo K, Mandelbaum RS, Vallejo A, Klar M, Roman LD, Wright JD
JAMA Oncol 2023 Aug 1;9(8):1113-1118. doi: 10.1001/jamaoncol.2023.0768. PMID: 37103920 Free PMC Article

Challenging differential diagnoses in small biopsies from the sinonasal tract.

Israel AK, Cracolici V, Griffith CC
Semin Diagn Pathol 2023 Sep;40(5):321-332. Epub 2023 Apr 14 doi: 10.1053/j.semdp.2023.04.011. PMID: 37085435

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: 30397729 Free PMC Article

See all (38)

Recent clinical studies

Etiology

Challenging differential diagnoses in small biopsies from the sinonasal tract.

Israel AK, Cracolici V, Griffith CC
Semin Diagn Pathol 2023 Sep;40(5):321-332. Epub 2023 Apr 14 doi: 10.1053/j.semdp.2023.04.011. PMID: 37085435

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

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

See all (224)

Diagnosis

Challenging differential diagnoses in small biopsies from the sinonasal tract.

Israel AK, Cracolici V, Griffith CC
Semin Diagn Pathol 2023 Sep;40(5):321-332. Epub 2023 Apr 14 doi: 10.1053/j.semdp.2023.04.011. PMID: 37085435

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: 27478963 Free 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

See all (568)

Therapy

FDA Approval Summary: Selpercatinib for the Treatment of Advanced RET Fusion-Positive Solid Tumors.

Duke ES, Bradford D, Marcovitz M, Amatya AK, Mishra-Kalyani PS, Nguyen E, Price LSL, Fourie Zirkelbach J, Li Y, Bi Y, Kraft J, Dorff SE, Scepura B, Stephenson M, Ojofeitimi I, Nair A, Han Y, Tezak Z, Lemery SJ, Pazdur R, Larkins E, Singh H
Clin Cancer Res 2023 Sep 15;29(18):3573-3578. doi: 10.1158/1078-0432.CCR-23-0459. PMID: 37265412 Free PMC Article

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: 37059834 Free 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

See all (146)

Prognosis

Small intestinal angiosarcoma on clinical presentation, diagnosis, management and prognosis: A case report and review of the literature.

Ma XM, Yang BS, Yang Y, Wu GZ, Li YW, Yu X, Ma XL, Wang YP, Hou XD, Guo QH
World J Gastroenterol 2023 Jan 21;29(3):561-578. doi: 10.3748/wjg.v29.i3.561. PMID: 36688020 Free 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: 27478963 Free PMC Article

Acute complications of fibroids.

Gupta S, Manyonda IT
Best Pract Res Clin Obstet Gynaecol 2009 Oct;23(5):609-17. Epub 2009 Mar 4 doi: 10.1016/j.bpobgyn.2009.01.012. PMID: 19264555

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

See all (316)

Clinical prediction guides

Exposure to nonanoic acid alters small intestinal neuroendocrine tumor phenotype.

Almobarak B, Amlani V, Inge L, Hofving T, Muth A, Nilsson O, Johansson M, Arvidsson Y, Elias E
BMC Cancer 2023 Mar 23;23(1):267. doi: 10.1186/s12885-023-10722-8. PMID: 36959559 Free PMC Article

Extraenteric Malignant Gastrointestinal Neuroectodermal Tumor-A Clinicopathologic and Molecular Genetic Study of 11 Cases.

Ulici V, Hornick JL, Davis JL, Mehrotra S, Meis JM, Halling KC, Fletcher CDM, Kao E, Folpe AL
Mod Pathol 2023 Jul;36(7):100160. Epub 2023 Mar 17 doi: 10.1016/j.modpat.2023.100160. PMID: 36934861

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: 31322481 Free 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

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

See all (126)

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

See all (4)

MedGen

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Small intestinal sarcoma

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Consumer resources

Reviews

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