acral myxoinflammatory fibroblastic sarcoma
Wednesday 14 July 2004
Definition: Acral myxoinflammatory fibroblastic sarcoma is a rare tumor of the distal extremities. It is a rare, low-grade sarcoma characterized by distinctive, large, and bizarre Reed—Sternberg—like cells associated with an intense inflammatory infiltrate. The biology of MIFS is still poorly understood.
Myxoinflammatory fibroblastic sarcoma (which has also been known as inflammatory myxohyaline tumour) is a distinctive lesion occurring most often in the distal extremities of young adults.
It is characterized by an admixture of fibrovascular areas with prominent chronic inflammation and looser, more myxoid areas with mucin-containing pseudolipoblasts.
In the more solid areas, there are characteristically large Reed–Sternberg-like cells, often having mummified nuclei.
These tumours have a high rate of local recurrence but only a very low risk of metastasis.
myxoid and inflammatory background
loss of chromosomes 3 and 13 (11407481)
myxoinflammatory fibroblastic sarcoma
Acral myxoinflammatory fibroblastic sarcoma: case series and immunohistochemical analysis. Kovarik CL, Barrett T, Auerbach A, Cassarino DS. J Cutan Pathol. 2008 Feb;35(2):192-6. PMID: 18190444
Two genetic pathways, t(1;10) and amplification of 3p11-12, in myxoinflammatory fibroblastic sarcoma, haemosiderotic fibrolipomatous tumour, and morphologically similar lesions. Hallor KH, Sciot R, Staaf J, Heidenblad M, Rydholm A, Bauer HC, Aström K, Domanski HA, Meis JM, Kindblom LG, Panagopoulos I, Mandahl N, Mertens F. J Pathol. 2009 Apr;217(5):716-27. PMID: 19199331
Ida CM, Rolig KA, Hulshizer RL, Van Dyke DL, Randolph JL, Jenkins RB, Nascimento AG, Oliveira AM. Myxoinflammatory fibroblastic sarcoma showing t(2;6)(q31;p21.3) as a sole cytogenetic abnormality. Cancer Genet Cytogenet. 2007 Sep;177(2):139-42. PMID: 17854670