FaCD Online Syndrome Fact Sheet

Last updated: 06 Feb 2008

Name: Angiolipomatosis, Familial

Mode of Inheritance: AD?/ AR?

Tumor features

angiolipoma

Non-tumor features (possible)

iris, Lisch nodules

Comment

Familial clustering of multiple (non-infiltrating) angiolipomas has been reported a few times, the most extensive family being that published by Kumar et al.[1]. In that family the angiolipomas were well encapsulated and located on the upper part of the body. Hapnes et al.[2] reported on a case with angiolipomas located close to joints and extended deeply between muscle, tendons and joint capsules. Abbasi et al reported an 80-year-old man with a 50-year history of asymptomatic, subcutaneous masses on the arms, trunk, and legs. His father and maternal grandmother had a history of similar lesions[3]. The distinction from familial multiple lipomatosis is not completely certain. Familial angiolipomatosis may sometimes be clinically confused with neurofibromatosis type 1 (NF1), interestingly, Cina et al.[4] reported the occurrence of Lisch nodules (a typical feature of NF1) in familial angiolipomatosis.

References

[1] Kumar R, Pereira BJ, Sakhuja V, Chugh KS. Autosomal dominant inheritance in familial angiolipomatosis. Clin Genet 1989; 35(3):202-204.
[2] Hapnes SA, Boman H, Skeie SO. Familial angiolipomatosis. Clin Genet 1980; 17:202-208.
[3] Abbasi NR, Brownell I, Fangman W. Familial multiple angiolipomatosis. Dermatology online journal 2007; 13(1):3.
[4] Cina SJ, Radentz SS, Smialek JE. A case of familial angiolipomatosis with Lisch nodules. Arch Pathol Lab Med 1999; 123(10):946-948.