A case report of Gorham–Stout syndrome remission

Abstract

Gorham–Stout syndrome (GSS) is a rare disorder characterized by spontaneous bone resorption with rapid progression, occasionally after minor trauma. It is also known as massive osteolysis or vanishing bone disease [1–3]. Some patients present with relatively abrupt pain and have swelling in the affected extremity, whereas others present with a history of insidious onset of pain, limitation of motion, and progressive weakness in the involved limb. The affected bone disappears completely [4]. The degree of osseous deformity in patients with GSS becomes severe. Paraplegia occurs in patients with spinal cord compression caused by vertebral involvement. Spinal involvement increases mortality to over 30%, because of complications such as spinal cord compression [5]. Thoracic cage, pulmonary, or pleural involvement leads to compromise of respiratory function and consequent death [3, 6]. Visceral complication can occur when the disease invades the surrounding tissues [7]. Recently, interferon and bisphosphonates have been reported to have remissive effects, although the mechanisms are unclear [8, 9]. Understanding the mechanisms that contribute to spontaneous or drug-induced remission is important in the control of this severe condition.