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

Purpose: This paper reviews the natural history and management of pati ents with Gorham's disease and presents four cases treated at The Mass achusetts General Hospital since 1965. Gorham's disease is characteriz ed by localized endothelial proliferation which results in destruction and resorption of bone. The etiology is undefined. There is no eviden ce of a malignant, neuropathic, or infectious component. This disease is progressive in most patients, but in occasional instances the proce ss has been noted to be self-limited. The principal treatment modaliti es are surgery and radiation therapy. Methods and Material: Since 1965 , four patients with Gorham's Disease have been treated at the Massach usetts Gene al Hospital. Three received definitive radiation therapy i n doses ranging from 31.5 to 45 Gy. The fourth patient underwent surge ry primarily. Results: Three patients are currently alive and fully fu nctional with no evidence of disease at last follow-up. The fourth pat ient died of progressive disease despite treatment with both radiation therapy and surgery. Conclusion: The prognosis for patients with Gorh am's disease is generally good unless vital structures are involved. d ue to the rarity of this entity, there is no standard therapy. Definit ive radiation, therapy in moderate doses (40-45 Gy in 2 Gy fractions) appears to result in a good outcome and few long-term complications.