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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
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
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
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

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
Tyrphostin 4-nitrobenzylidene malononitrile reduces chemotherapy toxicity without impairing efficacy.
Novogrodsky A, Weisspapir M, Patya M, Meshorer A, Vanichkin A
Cancer Res 1998 Jun 1;58(11):2397-403. PMID: 9622080
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
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
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

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
Ampulla of Vater Carcinoma: Sequencing Analysis Identifies TP53 Status as a Novel Independent Prognostic Factor and Potentially Actionable ERBB, PI3K, and WNT Pathways Gene Mutations.
Mafficini A, Amato E, Cataldo I, Rusev BC, Bertoncello L, Corbo V, Simbolo M, Luchini C, Fassan M, Cantù C, Salvia R, Marchegiani G, Tortora G, Lawlor RT, Bassi C, Scarpa A
Ann Surg 2018 Jan;267(1):149-156. doi: 10.1097/SLA.0000000000001999. PMID: 27611608
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

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