Fibrodisplasia óssea e síndrome de Mccune-albright: Descrição de um caso clínico e diagnóstico diferencial com doença óssea de paget

• Autor(a) principal: Silva, I
• Data de Publicação: 2010
• Outros Autores: Mateus, M, Branco, Jaime
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: http://hdl.handle.net/10362/23189
• topic: Fibrous; Dysplasia; Disease; lesions; spine; Syndrome; Biphosphonates; Paget; Osteosarcoma; McCune-Albright; of; cyst; Bone; Fibrodisplasia óssea; Síndrome McCune-Albright; Bifosfonatos; Osteosarcoma; Doença Óssea de Paget; Fi brous Dyspla sia of Bone; McCu ne-Al - bright Syndro me; Bip hospho na tes; Os te o sar co ma; Pa get Bone Di se a se

Fibrous dysplasia of bone or Jaffe Lischtenstein's disease is a genetic, non-inheritable disease of bone development, characterized by bone pain, deformities and fracture, mainly observed in young adults. The frequency is equal between sexes. Mutations in the gene coding the Gs alpha, GNAS complex, results in osteoblastic differentiation defects, and bone resorption. The disease can have a monostotic or polyostotic form, or be associated with cafe-au-lait skin spots and precocious puberty (McCune-Albright syndrome). The normal bone and bone marrow is replaced with abnormal benign intramedullary fibro-osseous tissue, and can involve any bone in the body. The vertebral involvement is rare. Radiological and pathological findings can be diagnostic. Biphosphonates and calcium, vitamin D and phosphorus supplements have been used in fibrous dysplasia treatment. Osteosarcoma is a rare, but serious malignant complication. We report the case of a 68 year old woman with a history of hypofisectomy, with a progressive low back pain, without systemic or neurological symptons. The bone scan, the radiographs and the computed tomography findings revealed polyostotic fibrous dysplasia, with vertebral and mandibular involvement. In this paper we compared fibrous dysplasia of bone with Paget bone disease.