Introdouction: Fibrous dysplasia is a benign fibro-osseous lesion that affects craniofacial bones including the maxilla and mandible. In the most cases of fibrous dysplasia of facial bones, radiographic findings represent lucent or mixed radiolucent-radiopaque lesion with ill-defined borders and gradual blending of its border with adjacent bone . In this presentation, a patient suffering ...

An elderly woman presented with a history of slow and progressive facial asymmetry since childhood. Physical examination revealed painless right facial enlargement involving the soft tissue and bony structures of the maxilla and mandible (image A). A plain radiograph revealed hazy radiopacities with a ground glass appearance (image B), suggestive of polyostotic fibrous dysplasia (FD). Transoral...

McCune-Albright syndrome (MAS) is a rare, post-zygotic (non-germline) disorder, characterized by hypersecretory endocrinopathies, fibrous dysplasia of the bone and café-au-lait macules. The most common endocrine dysfunction is gonadal hyperfunction; thus, hypersecretion of growth hormones (GHs) as a manifestation of endocrine hyperfunction in MAS is rarely reported. MAS affects both genders, al...

Gorham's disease is a rare disorder involving the proliferation of endothelial channels resulting in resorption and disappearance of bone. An unusual case of polyostotic Gorham's disease affecting the scapula, humerus, radius, and ulna in a 39-year-old woman is described. The patient had extensive disease spreading across both the glenohumeral and humeroulnar joints. This is the first report of...

McCune-Albright syndrome (MAS) is characterized by café-au-lait spot, multiple endocrine hyperfunction, and polyostotic fibrous dysplasia. A somatic point mutation of Gsalpha protein was reported to decrease GTPase activity, leading to increase in the GSalpha-associated hormone actions via cAMP. IL-6 is known to stimulate osteoclast formation and in the IL-6 promoter, a cAMP responsive element ...

Our aim is to provide review of available studies on Langerhans cell histiocytosis (LCH) and discuss treatment for polyostotic monosystem form of disease based on our clinical experience. LCH is an enigmatic disease with insufficiently understood etiology, pathophysiology, and variety of clinical presentations ranging from solitary eosinophilic granuloma to severe multisystem disease. It is mar...

Intravenous infusions with the bisphosphonate compound pamidronate decrease bone pain and reportedly can lead to refilling of dysplastic lesions in adults with fibrous dysplasia (FD) of bone. Here we describe the effects of this treatment approach in 18 children and adolescents (age at start of therapy, 6.2-17.5 yr; eight girls) with polyostotic FD, who received pamidronate for 1.2-9.1 yr (medi...

INTRODUCTION McCune-Albright syndrome is a complex inborn disorder due to early embryonal postzygotic somatic activating mutations in the GNAS1 gene. The phenotype is very heterogeneous and includes polyostotic fibrous dysplasia, typically involving the facial skull, numerous café-au-lait spots and autonomous hyperfunctions of several endocrine systems, leading to hyperthyroidism, hypercortisol...

Fibrous Dysplasia (FD) is a focal bone lesion occurring sporadically and in hereditary syndromes caused by activating mutations of the G protein alpha subunit. It rarely undergoes malignant transformation. Malignant Fibrous Histiocytoma (MFH) is a sarcoma of soft tissues and may be found as primary sarcoma of the bone or multifocally. Until now only a few cases of malignant transformation of FD...

OBJECTIVES/HYPOTHESIS Fibrous dysplasia is a condition of nonmalignant osseous change and may occur in a monostotic or polyostotic pattern, the latter potentially being associated with McCune-Albright syndrome. Symptoms are highly variable and dependent upon lesion location and size. STUDY DESIGN Retrospective review. METHODS Consecutive subjects with fibrous dysplasia of the temporal bone ...