GORHAM MASSIVE OSTEOLYSIS - THE ROLE OF RADIATION-THERAPY AND A REVIEW OF THE LITERATURE

Purpose: This paper reviews the natural history and management of patients with Gorham's disease and presents four cases treated at The Massachusetts General Hospital since 1965. Gorham's disease is characterized by localized endothelial proliferation which results in destruction and resorption of bone. The etiology is undefined. There is no evidence of a malignant, neuropathic, or infectious component. This disease is progressive in most patients, but in occasional instances the process has been noted to be self-limited. The principal treatment modalities are surgery and radiation therapy. Methods and Material: Since 1965, four patients with Gorham's Disease have been treated at the Massachusetts Gene al Hospital. Three received definitive radiation therapy in doses ranging from 31.5 to 45 Gy. The fourth patient underwent surgery primarily. Results: Three patients are currently alive and fully functional with no evidence of disease at last follow-up. The fourth patient died of progressive disease despite treatment with both radiation therapy and surgery. Conclusion: The prognosis for patients with Gorham's disease is generally good unless vital structures are involved. due to the rarity of this entity, there is no standard therapy. Definitive radiation, therapy in moderate doses (40-45 Gy in 2 Gy fractions) appears to result in a good outcome and few long-term complications.