PATHOLOGY OF LIPOMATOUS LESIONS IN PROTEUS-SYNDROME

Proteus syndrome is an extremely rare, complex hamartomatous disorder with markedly variable clinical expression. We present a case of Prote us syndrome with multiple disfiguring soft tissue masses that were pre sent since early childhood. The lesions involved predominantly the rig ht side of the body and included scoliosis, macrodactyly, and limited hyperostosis in the right foot. There was no evidence of cranial or sk in lesions. The patient underwent multiple resections of soft tissue m asses, including an amputation of the right foot because of severe gai t disturbance. All specimens exhibited lipomatous lesions that were pr obably hamartomatous rather than neoplastic. She carried a diagnosis o f neurofibromatosis for more than a decade, but a re-evaluation of cli nical features and pathological findings prompted the diagnosis of Pro teus syndrome. We believe that a more informed evaluation of the patho logy material may help to identify this rare entity.