LipofibromatosisM72.8
Synonym(s)
DefinitionThis section has been translated automatically.
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).
Occurrence/EpidemiologyThis section has been translated automatically.
m:w=3:1;
ManifestationThis section has been translated automatically.
Either congenital or acquired during the first year of life. Less frequently later.
LocalizationThis section has been translated automatically.
Predominantly hands/feet, more rarely arms or legs. Occasionally on the trunk or head region.
Clinical featuresThis section has been translated automatically.
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.
Note(s)This section has been translated automatically.
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.
LiteratureThis section has been translated automatically.
- 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
- J Surg Pathol 24:1491-1500. Teo HE et al (2005) Infantile lipofibromatosis of the upper limb. Skeletal radiol 34:799-802.