title: Autosomal-dominant familial angiolipomatosis.
abstract: Angiolipomas are among the most common benign soft-tissue tumors and usually present as solitary nodules; however, angiolipomas also may present as multiple subcutaneous nodules, typically on the arms and trunk of young men. Although multiple angiolipomas most often occur sporadically, a family history can be identified in a minority of cases. Familial angiolipomatosis is a rare condition with an autosomal-recessive transmission pattern that is characterized by multiple subcutaneous tumors and a family history of similar lesions, which are not associated with malignant neoplasms. We report a case of familial angiolipomatosis with an unusual autosomal-dominant transmission pattern. Our patient presented with multiple angiolipomas that were highly suggestive of familial angiolipomatosis transmitted in an autosomal-dominant fashion, as he had several family members with a history of similar fatty tumors. Autosomal-dominant familial angiolipomatosis may be misdiagnosed as neurofibromatosis type I. Therefore, in cases of multiple subcutaneous tumors and a family history of similar lesions, histologic examination is important to establish the correct diagnosis.
authors: George
authors: G
authors: Garib
authors: Mowry Professor and Director, Division of Anatomic Pathology, University of Alabama at Birmingham, 619 19th St S, HSB 149K, Birmingham, AL 35249, USA. gsiegal@uab.edu.
authors: Gene P
authors: GP
authors: Siegal
authors: Aleodor A
authors: AA
authors: Andea
mesh_terms: D000328:Adult; D018206:Angiolipoma / Q000175:diagnosis* / Q000235:genetics; D003937:Diagnosis, Differential; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D010375:Pedigree; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000235:genetics; D050151:Subcutaneous Fat