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Myxoinflammatory Fibroblastic Sarcoma: Morphologic and Genetic Updates
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Myxoinflammatory Fibroblastic Sarcoma: Morphologic and Genetic Updates
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Journal Article
Myxoinflammatory Fibroblastic Sarcoma: Morphologic and Genetic Updates
2014
Overview
Myxoinflammatory fibroblastic sarcoma (MIFS) is a malignant mesenchymal neoplasm most frequently arising in the distal extremities of adults, which usually behaves in a low-grade manner but is capable of metastasizing to local and distant sites, rarely leading to death. It is a rare tumor whose unusual morphology can lead to erroneous histologic diagnosis, either as a nonneoplastic (infectious or inflammatory) process or as a variety of neoplastic diseases. While its exact origin is uncertain, ultrastructural studies have shown at least some of the constituent cells to be modified fibroblasts. Distinct and reproducible genetic abnormalities identified in MIFS are translocation t(1;10)(p22:q24), with rearrangements of the TGFBR3 and MGEA5 genes associated with increased levels of FGF8, and formation of marker/ring chromosome 3, with amplification of the VGLL3 locus. Because these genetic abnormalities are shared by both MIFS and hemosiderotic fibrohistiocytic lipomatous tumor, it is thought that these 2 morphologically distinct neoplasms may comprise a spectrum of disease defined by these genetics. We review the literature on MIFS and discuss morphology (including that of MIFS/hemosiderotic fibrohistiocytic lipomatous tumor hybrid lesions), immunohistochemistry, the differential diagnosis, and recent molecular genetic developments.
Publisher
College of American Pathologists
Subject
/ Disease
/ Genes
/ Histone Acetyltransferases - genetics
/ Humans
/ Hyaluronoglucosaminidase - genetics
/ Liposarcoma, Myxoid - diagnosis
/ Liposarcoma, Myxoid - genetics
/ Liposarcoma, Myxoid - pathology
/ Liposarcoma, Myxoid - therapy
/ Receptors, Transforming Growth Factor beta - genetics
/ Sarcoma
/ Soft Tissue Neoplasms - diagnosis
/ Soft Tissue Neoplasms - genetics
/ Soft Tissue Neoplasms - pathology
/ Soft Tissue Neoplasms - therapy
/ Transcription Factors - genetics
/ Tumors
