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liposclerosing myxofibrous tumor

Friday 16 April 2004

Definition: Liposclerosing myxofibrous tumor (LSMFT) is a benign fibro-osseous lesion that is characterized by mixture of histologic elements including lipoma, fibroxanthoma, myxoma, ischemic ossification, and fibrous dysplasia (FD)-like features.

Liposclerosing myxofibrous tumor (LSMFT) is benign fibro-osseous lesion that has been reported in the intertrochanteric region of the proximal femur.

On imaging studies, the lesion is seen as a centrally located mixed lytic and sclerosing lesion.

The histology shows pagetoid bone with admixed fat, xanthomatous tissue, and fibromyxoid tissue, much of which may be considered reactive.

It has been suggested that this lesion represents the end point of a traumatized focus of fibrous dysplasia or other pre-existing benign lesion.

Synopsis

- irregular lytic and sclerosing intertrochanteric lesion
- disordered sclerosing and calcified lesion composed of bone, fibrous tissue, fat, and myxoid areas.

Etiology

- activating Gs alpha (GNAS1) mutation at the Arg201 codon (14652823)

References

- Gilkey FW. Liposclerosing myxofibrous tumor of bone. Hum Pathol. 1993 Nov;24(11):1264. PMID: 8244329