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Small intestinal fibrosarcoma

MedGen UID:
234788
•Concept ID:
C1335994
•
Neoplastic Process
Synonyms: Fibrosarcoma of Small Bowel; fibrosarcoma of small bowel; Fibrosarcoma of Small Intestine; fibrosarcoma of small intestine; Fibrosarcoma of the Small Bowel; fibrosarcoma of the small bowel; Fibrosarcoma of the Small Intestine; fibrosarcoma of the small intestine; Fibrosarcoma, Small Intestine; fibrosarcoma, small intestine; Small Bowel Fibrosarcoma; small bowel fibrosarcoma; Small Intestinal Fibrosarcoma; small intestinal fibrosarcoma; Small Intestine Fibrosarcoma; small intestine fibrosarcoma; small intestine fibrosarcoma (disease)
 
Monarch Initiative: MONDO:0004028

Definition

A usually aggressive malignant neoplasm arising from the small intestine. It is characterized by the presence of spindle-shaped fibroblasts and collagenous stroma formation in a herringbone growth pattern. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSmall intestinal fibrosarcoma

Recent clinical studies

Etiology

Synchronous cutaneous malignant peripheral nerve sheath tumor and jejunal gastrointestinal stromal tumor and submucosal angiomyolipoma in type 1 neurofibromatosis: A case report and literature review.
Kim KJ, Kim MS, Hong R, Lim SC
Medicine (Baltimore) 2023 Jan 20;102(3):e32696. doi: 10.1097/MD.0000000000032696. PMID: 36701730Free PMC Article
Mesenchymal tumors of the gastrointestinal tract with NTRK rearrangements: a clinicopathological, immunophenotypic, and molecular study of eight cases, emphasizing their distinction from gastrointestinal stromal tumor (GIST).
Atiq MA, Davis JL, Hornick JL, Dickson BC, Fletcher CDM, Fletcher JA, Folpe AL, Mariño-Enríquez A
Mod Pathol 2021 Jan;34(1):95-103. Epub 2020 Jul 15 doi: 10.1038/s41379-020-0623-z. PMID: 32669612
Acute toxic effects of single dose dacarbazine: hematological and histological changes in an animal model.
Milijašević B, Stefanović D, Lalić-Popović M, Tomić Z, Kolarović J, Lalošević D, Mikov M
Biotech Histochem 2014 Nov;89(8):583-90. Epub 2014 May 28 doi: 10.3109/10520295.2014.918653. PMID: 24867497
Distribution of neural cell adhesion molecule (NCAM/CD56) in gastrointestinal stromal tumours and their intra-abdominal mesenchymal mimics.
Agaimy A, Wünsch PH
J Clin Pathol 2008 Apr;61(4):499-503. Epub 2007 Oct 8 doi: 10.1136/jcp.2007.052696. PMID: 17923471
Malignant tumors of the small bowel.
Laws HL, Han SY, Aldrete JS
South Med J 1984 Sep;77(9):1087-90. doi: 10.1097/00007611-198409000-00007. PMID: 6484673

Diagnosis

Synchronous cutaneous malignant peripheral nerve sheath tumor and jejunal gastrointestinal stromal tumor and submucosal angiomyolipoma in type 1 neurofibromatosis: A case report and literature review.
Kim KJ, Kim MS, Hong R, Lim SC
Medicine (Baltimore) 2023 Jan 20;102(3):e32696. doi: 10.1097/MD.0000000000032696. PMID: 36701730Free PMC Article
Mesenchymal tumors of the gastrointestinal tract with NTRK rearrangements: a clinicopathological, immunophenotypic, and molecular study of eight cases, emphasizing their distinction from gastrointestinal stromal tumor (GIST).
Atiq MA, Davis JL, Hornick JL, Dickson BC, Fletcher CDM, Fletcher JA, Folpe AL, Mariño-Enríquez A
Mod Pathol 2021 Jan;34(1):95-103. Epub 2020 Jul 15 doi: 10.1038/s41379-020-0623-z. PMID: 32669612
Metastasis of dermatofibrosarcoma diagnosed by capsule endoscopy.
Martínez Sánchez A, Sánchez Ceballos FL, Fernández Aceñero MªJ, Rey Díaz-Rubio E
Rev Esp Enferm Dig 2019 Jan;111(1):71-72. doi: 10.17235/reed.2018.4900/2017. PMID: 30238763
Small intestine and ovarian metastasis in a patient with a history of cardiac fibrosacoma.
Esfehani MH, Mahmoodzadeh H, Omranipour R
J Egypt Natl Canc Inst 2015 Sep;27(3):171-2. Epub 2015 Apr 30 doi: 10.1016/j.jnci.2015.04.001. PMID: 25936897
Intestinal congenital/infantile fibrosarcoma: a new clinico-pathological entity?
Berrebi D, Fournet JC, Boman F, Fabre M, Philippe-Chomette P, Branchereau S, Fréneaux P, Bouron-Dal Soglio D, Michon J, Peuchmaur M
Pediatr Surg Int 2015 Apr;31(4):375-9. Epub 2015 Feb 5 doi: 10.1007/s00383-015-3670-7. PMID: 25652760

Therapy

A rare case report of malignant peripheral nerve sheath tumor involving both the small bowel and large bowel.
Khan N, Hashmi I, Atallah L, Shaaban H, Guron G, Fedida A
Ann Afr Med 2021 Jul-Sep;20(3):228-231. doi: 10.4103/aam.aam_10_20. PMID: 34558453Free PMC Article
Congenital intestinal fibrosarcoma with rapid recurrence requiring adjuvant chemotherapy.
See WSQ, Cheuk DKL, To KF, Ip PP, Chiang AKS, Ha SY, Chan GCF
Pediatr Int 2017 Jun;59(6):733-736. Epub 2017 Apr 24 doi: 10.1111/ped.13252. PMID: 28436622
Acute toxic effects of single dose dacarbazine: hematological and histological changes in an animal model.
Milijašević B, Stefanović D, Lalić-Popović M, Tomić Z, Kolarović J, Lalošević D, Mikov M
Biotech Histochem 2014 Nov;89(8):583-90. Epub 2014 May 28 doi: 10.3109/10520295.2014.918653. PMID: 24867497
Investigation of targeting mechanism of new dextran-peptide-methotrexate conjugates using biodistribution study in matrix-metalloproteinase-overexpressing tumor xenograft model.
Chau Y, Dang NM, Tan FE, Langer R
J Pharm Sci 2006 Mar;95(3):542-51. doi: 10.1002/jps.20548. PMID: 16419048
Verapamil potentiation of melphalan cytotoxicity and cellular uptake in murine fibrosarcoma and bone marrow.
Robinson BA, Clutterbuck RD, Millar JL, McElwain TJ
Br J Cancer 1985 Dec;52(6):813-22. doi: 10.1038/bjc.1985.264. PMID: 4074636Free PMC Article

Prognosis

Recurrent RET fusions in fibrosarcoma-like neoplasms in adult viscera: expanding the clinicopathological and genetic spectrum.
Zhao M, Yin X, He H, Xia Q, Ru G
Histopathology 2023 Mar;82(4):633-645. Epub 2022 Dec 13 doi: 10.1111/his.14847. PMID: 36443894
Mesenchymal tumors of the gastrointestinal tract with NTRK rearrangements: a clinicopathological, immunophenotypic, and molecular study of eight cases, emphasizing their distinction from gastrointestinal stromal tumor (GIST).
Atiq MA, Davis JL, Hornick JL, Dickson BC, Fletcher CDM, Fletcher JA, Folpe AL, Mariño-Enríquez A
Mod Pathol 2021 Jan;34(1):95-103. Epub 2020 Jul 15 doi: 10.1038/s41379-020-0623-z. PMID: 32669612
Carcinosarcoma of the upper urinary tract with an aggressive angiosarcoma component.
Cuadra-Urteaga JL, Font A, Tapia G, Areal J, Taron M
Cancer Biol Ther 2016;17(3):233-6. Epub 2016 Feb 18 doi: 10.1080/15384047.2016.1139237. PMID: 26891233Free PMC Article
Small intestine and ovarian metastasis in a patient with a history of cardiac fibrosacoma.
Esfehani MH, Mahmoodzadeh H, Omranipour R
J Egypt Natl Canc Inst 2015 Sep;27(3):171-2. Epub 2015 Apr 30 doi: 10.1016/j.jnci.2015.04.001. PMID: 25936897
Malignant tumors of the small bowel.
Laws HL, Han SY, Aldrete JS
South Med J 1984 Sep;77(9):1087-90. doi: 10.1097/00007611-198409000-00007. PMID: 6484673

Clinical prediction guides

Synchronous cutaneous malignant peripheral nerve sheath tumor and jejunal gastrointestinal stromal tumor and submucosal angiomyolipoma in type 1 neurofibromatosis: A case report and literature review.
Kim KJ, Kim MS, Hong R, Lim SC
Medicine (Baltimore) 2023 Jan 20;102(3):e32696. doi: 10.1097/MD.0000000000032696. PMID: 36701730Free PMC Article
Recurrent RET fusions in fibrosarcoma-like neoplasms in adult viscera: expanding the clinicopathological and genetic spectrum.
Zhao M, Yin X, He H, Xia Q, Ru G
Histopathology 2023 Mar;82(4):633-645. Epub 2022 Dec 13 doi: 10.1111/his.14847. PMID: 36443894
Mesenchymal tumors of the gastrointestinal tract with NTRK rearrangements: a clinicopathological, immunophenotypic, and molecular study of eight cases, emphasizing their distinction from gastrointestinal stromal tumor (GIST).
Atiq MA, Davis JL, Hornick JL, Dickson BC, Fletcher CDM, Fletcher JA, Folpe AL, Mariño-Enríquez A
Mod Pathol 2021 Jan;34(1):95-103. Epub 2020 Jul 15 doi: 10.1038/s41379-020-0623-z. PMID: 32669612
Acute toxic effects of single dose dacarbazine: hematological and histological changes in an animal model.
Milijašević B, Stefanović D, Lalić-Popović M, Tomić Z, Kolarović J, Lalošević D, Mikov M
Biotech Histochem 2014 Nov;89(8):583-90. Epub 2014 May 28 doi: 10.3109/10520295.2014.918653. PMID: 24867497
Systemic in vitro and in vivo evaluation for determining the feasibility of making an amorphous solid dispersion of a B-Raf (rapidly accelerated fibrosarcoma) inhibitor.
Cui Y, Chiang PC, Choo EF, Boggs J, Rudolph J, Grina J, Wenglowsky S, Ran Y
Int J Pharm 2013 Sep 15;454(1):241-8. Epub 2013 Jul 5 doi: 10.1016/j.ijpharm.2013.06.064. PMID: 23834830

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