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Myxoinflammatory Fibroblastic Sarcoma

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Abstract

Myxoinflammatory fibroblastic sarcoma is a neoplasm of intermediate biological aggressiveness and is classified among fibroblastic-myofibroblastic tumors.

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Notes

  1. 1.

    SMA: smooth muscle actin.

  2. 2.

    TGFBR3-MGEA5 fusion gene: TGFBR3 (transforming growth factor beta receptor 3) encodes a receptor which is a membrane proteoglycan that often functions as a co-receptor with other TGF-beta receptor superfamily members. MGEA5 (also known as OGA, O-GlcNAcase): the modification of cytoplasmic and nuclear proteins by O-linked N-acetylglucosamine (O-GlcNAc) addition and removal on serine and threonine residues is catalyzed by OGT (which adds O-GlcNAc) and MGEA5, a glycosidase that removes O-GlcNAc modifications.

  3. 3.

    TOM1L2-BRAF fusion gene: TOM1L2 (target Of Myb1 like 2 membrane trafficking protein) encodes a protein which belongs to a small family whose members have an N-terminal VHS domain followed by a GAT domain, which typically participate in vesicular trafficking. The BRAF proto-oncogene encodes a protein belonging to the RAF family of serine/threonine protein kinases; this protein plays a key role in the MAPK/ERK signaling pathway, which affects cell division, differentiation, and secretion; mutations in this gene (mainly V600E mutation) are frequently encountered in melanoma as well as non-Hodgkin lymphoma, colorectal cancer, thyroid carcinoma, non-small cell lung carcinoma, hairy cell leukemia, and adenocarcinoma of lung.

Suggested Readings

  • Fletcher et al (2020) WHO classification of tumours of soft tissue and bone, 5th edn

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  • Kao et al (2017) Recurrent BRAF gene rearrangements in myxoinflammatory fibroblastic sarcomas, but not hemosiderotic fibrolipomatous tumors. Am J Surg Pathol 41(11):1456–1465

    Article  Google Scholar 

  • Liu et al (2019) The t(1;10)(p22;q24) TGFBR3/MGEA5 translocation in pleomorphic hyalinizing angiectatic tumor, myxoinflammatory fibroblastic sarcoma, and hemosiderotic fibrolipomatous tumor. Arch Pathol Lab Med 143(2):212–221

    Article  CAS  Google Scholar 

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Correspondence to Simone Mocellin .

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Mocellin, S. (2021). Myxoinflammatory Fibroblastic Sarcoma. In: Soft Tissue Tumors . Springer, Cham. https://doi.org/10.1007/978-3-030-58710-9_183

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  • DOI: https://doi.org/10.1007/978-3-030-58710-9_183

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-58709-3

  • Online ISBN: 978-3-030-58710-9

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