Proteus Syndrome: Report of Two Cases With Pelvic Lipomatosis

Abstract

Localized growth disturbances are characteristic of a group of congenital hamartomatous disorders, the most common of which is neurofibromatosis. The literature contains many reports of individual cases that have been difficult to place into well-delineated syndromes, although a few had been recognized as new entities. Some of these1-6 were clearly established as a new syndrome when wiedmann and colleagues7 described four patients with an unusual constellation of findings, including partial gigantism of the hands and/or feet, asymmetry, cutaneous abnormalities, macrocrania, and osseous protuberances of the skull. They called this the Proteus syndrome. Eleven possible cases were recently reviewed.8,9 We report two other patients with the Proteus syndrome who, in addition, were found to have abdominal and pelvic lipomatosis.