Gorham's disease that gave response to radiotherapy

摘要

Background: Gorham's disease is a disorder that results as destruction and resorption of osseous matrix and is characterized by proliferation of vascular channels. We here reported a rare case of Gorham's disease that gave response to radiotherapy. Our aim is to remind the clinicians about this rare entity, and to discuss the etiopathology, clinical presentation and treatment options for patients with Gorham's disease. Case Report: 32 years old male patient had admitted to neurosurgery department for pain, weakness and limitation of motion at the head and neck. Osteolysis was detected at the bodies of 1, 2 and 3. vertebrae. After a number of examinations and excisional biopsy, the patient was diagnosed as Gorham's disease. Cervical stabilization was made but after 3 years, the patient's complaints had occurred again and a restabilization was made. 2 years after; with increased complaints, the patient was send to our clinic for consultation and radiotherapy (RT) was applied to the destructive parts of the bones. 2 months after RT, the patients' complaints had a significant decrease both clinically and radiologically. Conclusion: Gorham's disease can be seen in both men, women and at any age group, although most cases are diagnosed before the age of 40 years. No familial predisposition has been found. The disease may affect the appendicular or the axial skeleton. The exact nature of the disease process is unknown. The medical treatment for Gorham's disease includes radiation therapy, anti-osteoclastic medications (bisphosphonates), and alpha-2b interferon. As laboratory studies are usually within normal limits in most cases, diagnosis can often be delayed. By the light of clinical findings with characteristic radiographic and histopathological findings, an early diagnosis can be possible.