Displasia fibrosa: relato de três casos

Abstract

A displasia fibrosa é lesão pseudo-neoplásica de etiologia desconhecida, caráter benigno e recidivante, caracterizada pelo desenvolvimento de tecido fibroso e traves osteóides que substituem gradualmente o osso normal. O principal diagnóstico diferencial da forma monostótica, no envolvimento dos ossos da cabeça e pescoço, é o fibroma ossificante, que alguns consideram variante desta entidade. Esse trabalho tem como objetivo revisar as principais características clínicas, radiológicas e histopatológicas que auxiliam no diagnóstico diferencial da displasia fibrosa com o fibroma ossificante e discutir o comportamento recidivante da displasia fibrosa, essencial ao planejamento cirúrgico. Relatamos três casos provenientes do ambulatório de Otorrinolaringologia da Santa Casa de Misericórdia de São Paulo, avaliados quanto às queixas, exame físico, achados de imagem e tratamentos. Nos três casos o diagnóstico foi confirmado pelo exame anatomopatológico. Todos apresentaram recidiva após a ressecção cirúrgica, diagnosticada entre o primeiro e o oitavo ano de seguimento. Devido ao comportamento clínico semelhante da displasia fibrosa e do fibroma ossificante, o exame anatomopatológico é essencial para o diagnóstico diferencial. O acompanhamento clínico permanente desses pacientes é necessário para que as possíveis recidivas sejam diagnosticadas precocemente.

Case Report of Fibrous Dysplasia of Maxillary Region

“…Lee e col. proposed that the abnormalities in the AMPc or kinase protein intracellular regulation etiologic factors are possible in the development of the FD. Other researchers have identified mutations in the GNAS gen, which result in the GTPase activity alterations, with a consequent increase of the AMPc intracellular concentrations (8).It manifests more frequently in the childhood and, however, is not exclusive of this age range (4,9). It has an usually slow evolution, a tendency to stabilize after adolescence and a high recurrence rate (2, 9, 10).…”

“…The simple presence of the lesion does not justify surgical intervention. The main indications for surgical treatment of FD are the presence of significant clinical symptoms and the control of large aesthetic deformities (2,9). Because of the lesion benign nature and its recurrence character (10-20%), the surgery must be relatively conservative with the main objective of preserving the function (9).…”

“…The main indications for surgical treatment of FD are the presence of significant clinical symptoms and the control of large aesthetic deformities (2,9). Because of the lesion benign nature and its recurrence character (10-20%), the surgery must be relatively conservative with the main objective of preserving the function (9). In this case, we chose an expectant procedure, not only for the absence of organic and functional alterations, in spite of the aesthetic involvement, but also because of the age of the patient.…”

“…In this case, we chose an expectant procedure, not only for the absence of organic and functional alterations, in spite of the aesthetic involvement, but also because of the age of the patient. He keeps on quarterly service follow-up until his growth and adolescent development is complete.Radiotherapy is counter-indicated not only because the tumor is radioresistant but also because of the probable increase of the capacity for the dysplasia sarcomatous transformation, whose estimates range from less than 0.5% of its natural evolution to about 44% after radiotherapy (2,9).…”

“…Lustig reports that, for inhibiting the osteoclasts, the biphosphonates are employed in the treatment of patients with osteodystrophies, but that their effects in DF have been limited (9). Moreno reports the following benefits in patients treated with biphosphonates: improvement of pain and inflammatory symptoms, stabilization and reduction of bone destruction, increase of the osseous density, recalcification of osteolytic lesion in 50% of the patients, improvement of the radiologic aspects and the osseous metabolism (6).Some authors defend the need for a clinical, endocrinologic study and cintilography with Tc 99 in the patients with diagnosis of Monostotic Fibrous Dysplasia in search of other lesions, other regions and extra-skeleton nvolvement (6,11).…”