Angiolipomatosis, familial D17.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Angiolipoma microthromboticum

Definition
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Familial occurrence of multiple cutaneous angiolipomas in symmetrical arrangement, predominantly in the extremities.

Etiopathogenesis
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Autosomal recessive mode of inheritance is discussed.

Manifestation
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Start in early childhood.

Localization
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Predominantly in the area of the extremities, mostly near the joints.

Clinical features
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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.

Histology
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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 diagnosis
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Angiomyolipomas in Pringle-Bourneville phacomatosis, multiple lipomas in Gardner syndrome, familial forms of lipomatosis.

Therapy
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Excision, since no spontaneous regression tendency.

Progression/forecast
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Slow growth, recurrence after incomplete removal.

Literature
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  1. Cina SJ et al (1999) A case of familial angiolipomatosis with Lisch nodules. Arch Catholic Lab Med 123: 946-948
  2. Hapnes SA, Boman H, Skeie SO (1980) Familial angiolipomatosis. Clin Genet 17: 202-208
  3. Klem KK (1949) Multiple lipoma - angiolipomas. Acta Surgeon Scand 97: 527-532

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Last updated on: 29.10.2020