Authors: Alessandro Corsi Cristina Remoli Mara Riminucci Ernesto Ippolito John Dimitriou
Publish Date: 2016/11/08
Volume: 46, Issue: 2, Pages: 233-236
Abstract
Multiple nonossifying fibromas MNOFs occur either isolated or in association with other anomalies are usually localized in the long bones of the lower limbs may be radiographically confused with other skeletal lesions and tend to heal spontaneously with the completion of the skeletal growth Segmental distribution either monomelic or polymelic and ipsilateral is rare and commonly observed in the context of developmental diseases known as “RASopathies” which are caused by mutations in genes that encode components or regulators within the Ras/mitogenactivated protein kinase signaling pathway We describe here the radiographic and pathologic features of an 18yearold Caucasian boy whose clinical history started at the age of 3 when the diagnosis of aneurysmal bone cyst was made on a lytic lesion of his left clavicle Over the following 2 years the patient developed polyostotic and monomelic lesions within the left humerus radius and ulna No other skeletal and extraskeletal anomalies were clinically detected The lesions were interpreted as consistent with polyostotic fibrous dysplasia and MNOFs and showed an unusually aggressive clinical course with progressive increase in size and coalescence The definitive diagnosis of MNOFs was made after the exclusion of fibrous dysplasia by molecular analysis The polyostotic and monomelic distribution of the lesions and the unusually aggressive clinical course contribute to make this case of MNOFs unique
Keywords: