Monostotic fibrous dysplasia of the ribs

Abstract

Fibrous dysplasia (FD) is a sporadic benign skeletal disorder that can affect one bone (monostotic form) or multiple bones (polyostotic bone). Around 6-20% of monostotic FD occurs in the ribs. The objective of this study was to report our experience in the management of the monostotic FD of the ribs. Between January 2004 and December 2009, seven cases of FD of the rib (six men and one woman, mean age 30.4 years, range 17-40 years) were operated on. The patients were evaluated with plain radiographs and computer tomography (CT). All our patients were symptomatic; two patients presented chest pain and swelling and other patients presented only chest pain. One rib was involved in all our patients (monostotic form): the site was fifth rib (four cases), sixth rib (two cases) or second rib (one case). Radiologically, plain films and CT showed an expansible lesion with a ground-glass centre and thinning of the cortex. Rib resection was performed in all patients; there were no postoperative complications and no recurrence in all cases at mean 43 month follow-up. In symptomatic monostotic FD of ribs, the involved segment of bone may be excised to rule out malignancy and for painful lesions.

Monostotic Fibrous Dysplasia of the Rib: A Case Report

“…It was recognized that both monostotic and polyostotic FD are nonneoplastic processes associated with postzygotic-activating mutations of signal-transducing G proteins encoded by GNAS1 on chromosome 20. Osteoblasts carrying this mutation show increased proliferation and inappropriate differentiation which resulted in fibrotic bone matrix [2, 4, 7, 9, 13]. In its polyostotic form, FD may be associated with McCune-Albright syndrome (polyostotic FD, café-au-lait spots, and endocrine dysfunction) and Mazabraud's syndrome (polyostotic FD and soft-tissue myxomas) [2, 5, 14].…”

“…The most common sites of involvement include the ribs (28%), proximal femur (23%), and craniofacial bones (20%). Solitary involvement of other bone is unusual [2, 4, 7]. Monostotic FD in the long bones occurs most frequently in adolescence.…”

“…Any bone may be affected, the long bone, skull, and ribs most often [2, 4–6]. FD of the ribs accounts up to 30% of all benign chest wall tumors and monostotic forms are about four–six times more common than polyostotic forms [2, 4, 7]. It is usually an incidental imaging finding.…”

Thoracoscopic Management of Fibrous Dysplasia: First Experience in Turkey

“…Fibrous dysplasia (FD) is a osseous abnormality and bone structure is replaced by fibro-osseous tissue 1 . This change causes expansion and loss of the bone cortex.…”

Rare case of symptomatic (painful) fibrous dysplasia of 12th Rib

“…Author believe that in ribs location of symptomatic monostotic FD, excision of involved bone must be indicated for both curative and diagnosis intention, to rule out malignancy and provide relief from symptoms. 15 …”