Gorham's disease is a very rare disorder characterized by bone loss (osteolysis) often associated with uncontrolled, destructive proliferation of vascular or lymphatic capillaries within bone and surrounding soft tissue [1, 3]. Most cases occur in children and young adults (usually less than 40 years of age) and no definite inheritance pattern has been reported. The natural history of Gorham's disease is unpredictable. It may spontaneously arrest or progress relentlessly until all osseous tissue disappears. Bone loss can occur in just one bone or spread to soft tissue and adjacent bones. In the case, our patient complained of dull aching pain and insidious onset of progressive weakness. The progressive bony destruction of left upper limb occurred over a period of eight months. Typical characteristics of acute spontaneous absorption of bone were noted.
Diagnosis of Gorham's disease is based on clinical and radiological features of loss of bones with histological evidence of angiomatous tumor. In most cases laboratory tests are usually within normal limits. The clinical presentation is variable, largely depending on the site of skeletal involvement. The characteristic radiographic and histopathological findings are helpful for making an early accurate diagnosis. The bones most commonly affected are the clavicle, scapula, humerus, ribs, and pelvis [4]. Radiographically, the destructive changes seen in the left upper limb mimicked malignant neoplasm due to aggressive osteolytic destruction with ill-defined lesion margins in this case. The resorption of left carpal bone was a distinguishing feature that could be differentiated from malignant neoplasm. CT scans showed extensive progressive osteolysis was involved in the whole left forearm. Soft tissue involvement was limited to the region around the bony abnormality. There does not appear to be a male or female predominance in patients with Gorham's disease. Most patients have been less than 40 years of age at the time of diagnosis [5]. The diagnosis should be made only after carefully excluding the complicated cause of osteolysis. Different forms of osteolysis such as, skeletal angiosarcoma, essential osteolysis and hereditary osteolysis must be ruled out by radiographic studies and appropriate blood tests [6]. A clinical suspicious case must be performed biopsy of the lesion.
The etiology of Gorham's disease is still speculative. There is substantial documentation that the disease has vascular or lymphatic proliferation with or without fibrosis [2, 7]. Hemangiomatosis has been considered to be the characteristic feature related to the pathogenesis [1]. The progressive hemangiomatosis or lymphangiomatosis may extend to adjacent soft tissue or organs, such as the scapula, ribs or thoracic vertebra. A high morbidity and mortality is seen in patients with spinal or visceral involvement. The mechanism of bone resorption is unclear. There was no evidence of a malignant, neuropathic, or visceral component involved in our case. A biopsy confirmed extensive osteolysis was caused by progressive hemangiomatosis.
Several therapeutic modalities include radiation therapy, anti-osteoclastic medications (bisphosphonates) and alpha-2b interferon have been used in the management of Gorham's disease [8]-[10]. No single treatment modality has proven effective in arresting the disease. Surgical treatment options include resection of the lesion and reconstruction using bone grafts or prostheses [11, 12]. In recent years, most patients have been treated with surgery or radiation therapy. Massive resection and radiation therapy was treated in our patient. Two years after surgery, there was no evidence of tumor recurrence.