Radiation therapy in the treatment of Gorham disease.

ABSTRACT

An adolescent male with Noonan syndrome presented with a nonhealing fracture of the proximal right humerus. Over a 7-month period there was progressive loss of bone in this region, resulting in a flail arm at 9 months. Radiographic review was consistent with Gorham disease. In addition, there was significant bleeding in the soft tissues and pain. Radiation was delivered. Seven years passed, until the patient re-presented with right hemithorax near collapse secondary to chylothorax. A chest tube was placed with temporary relief, but significant effusion remained. Radiation was again administered, and by the end of therapy the chest tube was removed. The effusion has not recollected at last follow-up, which is now 6 months. The use of radiation in the treatment of Gorham disease has been demonstrated to have excellent palliative ability.