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Background: Fibrous dysplasia (FD) is a less common benign bony fibro-osseous lesion, which is divided into two main kinds: monostotic FD and polyostotic FD (PFD). The natural history or aetiopathogenesis of FD is poorly understood, and treatments are still controversial. Methods: A review of the literature was performed to analyse and discuss the diagnosis and management of FD through a case report and literature review. Results: The current case of a PFD patient, which is usually presented in late childhood or early adulthood, is sometimes associated with endocrine disorders such as McCune–Albright syndrome (MCA). Computed tomography-guided biopsy from the lytic bone area is a useful technique for the evaluation and diagnosis of disease, though the accuracy of the histopathology findings has been not conclusive. Exclusion of other lytic bone lesion disease is the mainstay of diagnosis. All medical and surgical treatments are valid; no one has been proven to be better. The present case is reported because it illustrates specific points of differential diagnosis, such as multiple myeloma, hyperparathyroidism, primary bone malignancy, secondary metastasis in bones, sarcoidosis or granulomatous disease of bone. Conclusions: In the current case, surgical repair of the bone is not possible due to extensive involvement in different sites of the bones, and there is no mechanical compression in any neurological structures, hence medical management was chosen. There is no gold standard in medical or surgical treatment, and each treatment should be individualised. Only medical management shows good results in such cases.