LYMPHANGIOLIPOMA OF THE THORACIC SPINE IN A PEDIATRIC-PATIENT WITH PROTEUS SYNDROME

Proteus syndrome is a rare hamartomatous disorder involving macrodacty ly, hemihypertrophy, and subcutaneous lymphangiomas; fewer than 25 cas es have been reported worldwide. We report a case of a thoracic epidur al lymphangiolipoma in a 5-year-old boy with Proteus syndrome. Compute rized axial tomography (CT) of the thoracic spine revealed a left post erior mediastinal mass that extended into the spinal canal through adj acent neural foramina. No sign of spinal cord compression was observed despite the extensive volume of tumor within the spinal canal. Surgic al debulking utilizing a T3-10 laminectomy resulted in gross total res ection of the tumor. Microscopic examination of the surgical specimen revealed a lymphangiolipoma. No previous report of spinal cord involve ment has been reported in this syndrome. A detailed discussion of the phenotypic features and probable mode of genetic transmission is inclu ded.