Fibrous Dysplasia of Single Bones (Monostotic Fibrous Dysplasia)

Monostotic Fibrous Dysplasia of the Distal Radius Metaphysis: Case Report

“…The mass was light tan in color, rubbery in texture, and was easily removed as an intact single large mass They are typically said to have a "ground-glass" appearance, but depending on the extent of the dysplastic fibrous tissue and on the amount of bone and degree of calcification, they may range from radiolucent to sclerotic [2,7,17]. Radiographically, the differential diagnosis may include Paget's disease, solitary bone cysts, aneurysmal bone cysts, enchondroma, adamantinoma, low-grade intramedullary osteosarcoma, osteofibrous dysplasia, and giant cell tumor [5,19]. In the presented case, as in all bone tumor cases, a biopsy was required to make a definitive diagnosis.…”

“…This mutation occurs in somatic cells sometime after fertilization and therefore is not inherited [17,19]. Chromosome 12 has also been implicated in the pathogenesis of fibrous dysplasia, but so far, no chromosomal changes have been demonstrated consistently [4,17].…”

“…In 1942, Lichtenstein and Jaffe described two types of fibrous dysplasia: monostotic, occurring at one locus in one bone, and polyostotic, occurring at multiple loci in one or multiple bones [15]. Monostotic fibrous dysplasia is the more common of the two and generally portends a better outcome [11,19,20]. It is commonly found in the tibia, fibula, ribs, and the bones of the jaw and skull but may occur in any bone [1,8,10,15].…”

Displasia fibrosa do osso temporal: relato de dois casos

“…8 Schlumberger sugeriu que a forma monostótica deste distúrbio pode não ser relacionado com ambas as síndrome de Albright e displasia fibrosa poliostótica, e ao contrário, é causado por um distúrbio do processo normal de reparação logo após uma lesão. 9 Estudos radiográficos destas lesões geralmente demonstram uma radiodensidade homogênea envolvendo qualquer combinação da porção petrosa do osso temporal, timpânica, mastóidea, escamosa e petrosa. Três padrões radiográficos na displasia fibrosa do crânio e ossos faciais foram descritos por Fries.…”

“…A DF mostra um padrão ósseo tipo entrelaçado ao invés de lamelar, o que permite uma diferenciação do fibroma ossificante. 9,13 A DFM é um processo benigno de crescimento lento, o qual se torna quiescente após a puberdade. O sintoma otológico mais comum da DFM do osso temporal é uma perda auditiva condutiva secundária à oclusão da tuba de eustaquio ou do conduto auditivo externo ou por fixação da cadeia ossicular.…”