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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.

Synopsis

- virocyte-like
- lipoblast-like cells
- myxoid and inflammatory background

Cytogenetics

- t(1;10)(p22;q24) (11407481)
- t(2;6)(q31;p21.3) (17854670)

- loss of chromosomes 3 and 13 (11407481)

See also

- myxoinflammatory fibroblastic sarcoma

References

- 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

- Acral myxoinflammatory fibroblastic sarcoma: a low-grade tumor of the hands and feet. Meis-Kindblom JM, Kindblom LG. Am J Surg Pathol. 1998 Aug;22(8):911-24. PMID: 9706971