GORHAM-STOUT DISEASE: THE EXPERIENCE OF ISTITUTO RIZZOLI

Abstract

Purpose: GSD, also known as massive osteolysis or disappearing bone disease, is rare, characterized by proliferation of vascular channels of hematic and lymphatic origin resulting in progressive distruction of bone. This study about Gorham-Stout disease is a retrospective review of the Rizzoli files with special attention given to treatment and outcome.

Materials and Methods: This study is based on a retrospective analysis of a single institution experience. In the Rizzoli files we found 15 cases of GSD from 1968 to 2008. Two were excluded for insufficient documentation. For 13 cases clinical data, imaging and histology were analysed. Histopatologically benign vascular proliferation of thin-walled endothelial capillaries surrounded by a fibrous stroma is present. Adipose involution of the bone marrow and extreme thinning of bony trabeculae represent other histopatologic features. A final diagnosis was established based on clinical, radiological and histopathologic features, as recommended in the literature. Imaging included X-rays in 11 cases and CT or MRI in 5. All lesions were lytic, with an associated sclerosis in two cases. There was one lesion in four cases, several lesions in the same bone in one, and multiple bones involved in six patients. Primary sites were proximal femur in 7 cases, pelvis in 2, hip and knee, calcaneus, humerus and cervical spine in 1 case each.

Results: Two patients had no treatment, 2 conservative treatment (cast or brace), 5 surgery, 6 medical treatment (byphosphonates, calcitonin, zoledronic acid, interferon, steroids), 1 radiotherapy, 2 selective arterial embolization. Surgery consisted of internal fixation of 4 pathologic fractures and reconstruction of the entire humerus with a double composite allograf in 1. Overall, surgery only in 2 patients, medical treatment only in 4 (1 also embolization), surgery and medical treatment in 2 (1 also embolization), radiotherapy only in 1, conservative treatment in 2. Four patients were lost at follow up. In the remaining 9 patients mean follow up was 17 ys.(min 2, max 30). These 9 patients had the following results: 2 dead, 3 healed, 3 with stable disease, 1 alive with asymptomatic disease at 24 ys.

Conclusions: No clear treatment recommendations were desumed. Surgery is indicated in pathologic fractures or reconstruction of massively destroyed bones, medical treatment and selective embolization are helpful. In the literature prostheses are mostly recommended for reconstructions due to the risk of allografts resorption.