Massive osteolysis ( Gorham,s disease) and report a case

Gotham's disease or vanishing bone is a rare disease with unknown etiology. Frequently, children and young adults are affected. It is characterized by proliferation of vascular or lymphatic tissue which results in destruction and resorption of bone. In this disease, the destructed bone is replaced either by hemangiomatous or lymphangiomatous tissue ( s) and in a later stage, fibrosis occurs. It is postulated that the vascular replacement is an attempt to repair the lesions rather a cause. The first radiograph may show only an irregular area of osteolysis one bone, but the destructive process slowly extends over a period of several years in pathology reports, and the affected Bone irrespective of the intervening joints. The bone is described as soft and spongy in pathology reports, and the affected Bone is replaced by loose or loosing angiomatous tissue, consisting of capillary or sinusoidal vessels. Lymphangiomatous elements may predominate. A patient with Gotham's is reported, whose main complaint was pain in left side of the chest, and in initial chest X-RAY several ribs were vanished on the left side. In spite of radiation therapy, the patient died because of progression of lesion and development of bilateral pleural effusion.

کلیدواژه ها:

Massive osteolysis ، Gotham's disease