Abstract
Myxoinflammatory fibroblastic sarcoma is a neoplasm of intermediate biological aggressiveness and is classified among fibroblastic-myofibroblastic tumors.
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Notes
- 1.
SMA: smooth muscle actin.
- 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.
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
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
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
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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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