Gorham-Stout disease: the experience of the Rizzoli Institute and review of the literature.
Gorham-Stout disease (also known as "disappearing bone disease") was first described by Jackson in 1838, but was properly defined by Gorham and Stout in a series of 24 patients in 1954-1955. It is a rare disease of unknown etiology (about 200 cases reported in the literature) characterized by spontaneous progressive resorption of bone without malignant proliferation of vascular structures. The diagnosis is one of exclusion and it is based on combined histological, radiological, and clinical features. Benign vascular proliferation with fatty bone marrow and thinning of bony trabeculae is a typical histological feature. Standard radiographs of disappearing bone disease show progressive bony resorption with adjacent soft tissue involvement. Most cases of Gorham-Stout disease resolve spontaneously, but prognosis remains unpredictable. This study reports 13 cases of Gorham-Stout disease treated in our institution from 1968 to 2008. The aim of the work was to review our series and the literature on this rare disease, as well as to evaluate whether or not an optimal treatment can be identified and recommended.
Ruggieri
Pietro
P
Department of Orthopedics, University of Bologna, Istituto Ortopedico Rizzoli, Bologna, Italia. pietro.ruggieri@ior.it
Montalti
Maurizio
M
Angelini
Andrea
A
Alberghini
Marco
M
Mercuri
Mario
M
D000293:Adolescent; D000328:Adult; D001842:Bone and Bones / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D005260:Female; D005598:Fractures, Spontaneous / Q000209:etiology / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D010015:Osteolysis, Essential / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000628:therapy; D011859:Radiography; D055815:Young Adult