Angiolipomatosis, familialD17.9
Synonym(s)
Angiolipoma microthromboticum
DefinitionThis section has been translated automatically.
Familial occurrence of multiple cutaneous angiolipomas in symmetrical arrangement, predominantly in the extremities.
EtiopathogenesisThis section has been translated automatically.
Autosomal recessive mode of inheritance is discussed.
ManifestationThis section has been translated automatically.
Start in early childhood.
LocalizationThis section has been translated automatically.
Predominantly in the area of the extremities, mostly near the joints.
Clinical featuresThis section has been translated automatically.
Up to chestnut-sized, blurred, subcutaneously located, skin-coloured, soft tumours in symmetrical arrangement. Expansion of tumor tissue between muscles, tendons and joint capsules possible.
HistologyThis section has been translated automatically.
Benign, non-encapsulated, mesenchymal tumor consisting of mature fatty tissue and well-formed arterioles, venules and capillaries; numerous fibrin thrombi. In comparison to ordinary lipomas, the proportion of the vascular component is increased to 15-50%.
Differential diagnosisThis section has been translated automatically.
Angiomyolipomas in Pringle-Bourneville phacomatosis, multiple lipomas in Gardner syndrome, familial forms of lipomatosis.
TherapyThis section has been translated automatically.
Excision, since no spontaneous regression tendency.
Progression/forecastThis section has been translated automatically.
Slow growth, recurrence after incomplete removal.
LiteratureThis section has been translated automatically.
- Cina SJ et al (1999) A case of familial angiolipomatosis with Lisch nodules. Arch Catholic Lab Med 123: 946-948
- Hapnes SA, Boman H, Skeie SO (1980) Familial angiolipomatosis. Clin Genet 17: 202-208
- Klem KK (1949) Multiple lipoma - angiolipomas. Acta Surgeon Scand 97: 527-532