A case of familial angiolipomatosis with Lisch nodules

Familial angiolipomatosis is a rare syndrome that may be confused clinicall y with neurofibromatosis type 1. This condition is most often inherited in an autosomal recessive manner; however, several reports have been published suggesting an autosomal dominant mode of inheritance. Angiolipomatosis, al though somewhat disfiguring, is a benign condition with no known associatio n with malignant neoplasms. This is in contradistinction to neurofibromatos is, an autosomal dominant syndrome associated with a myriad of benign and m alignant neoplasms. It is, therefore, important to discriminate this entity from neurofibromatosis when a patient presents with multiple subcutaneous tumors and a family history of similar lesions. Described is a case of a pr ison inmate with a history of seizures and "neurofibromatosis" without clin ical documentation. Lisch nodules were noted on the irides. Postmortem exam ination showed multiple subcutaneous yellow tumors on the chest and arms. F ine-needle aspiration of 1 mass yielded adipose tissue with prominent vesse ls; histologic sections of another mass showed angiolipoma. The remainder o f the autopsy showed significant coronary artery disease and a remote cereb ral infarction of the temporal lobe but no signs of neurofibromatosis. We f eel that the presence of multiple angiolipomas in combination with Lisch no dules lends credence to the proposed relationship between fatty tumors and neurofibromatosis suggested by other authors.