Lipofibromatosis M72.8

Rare mesenchymal tumor of fibroblastically differentiated spindle cells with enclosed nests of mature adipocytes, congenital or acquired in the first year of life (rarely later).

m:w=3:1;

Either congenital or acquired during the first year of life. Less frequently later.

Predominantly hands/feet, more rarely arms or legs. Occasionally on the trunk or head region.

Non-coloured, skin-coloured or slightly reddened, deep-set, 1.0-max.7.0 cm large, plaque or nodule-shaped, moderately indurated neoplasms on hands and feet.

The clinical picture is, according to various opinions. The clinical picture is classified as a variant of infantile/juvenile fibromatosis according to various authors. The inclusion of mature fatty tissue in the tumor parenchyma identifies lipofibromatosis as a distinct entity.

1. Fetsch JFet al. (2000) A clinicopathologic study of 45 pediatric soft tissue tumors with an admixture of adipose tissue and fibroblastic elements, and a proposal for classification as lipofibromatosis. At
2. J Surg Pathol 24:1491-1500. Teo HE et al (2005) Infantile lipofibromatosis of the upper limb. Skeletal radiol 34:799-802.