The lesions of fibrous dysplasia usually stabilize after adolescence, and the surgical treatment of adult patient remains the mainstay. However, the surgical treatment alone may be not enough for patient with polyostotic fibrous dysplasia. We present the case of a surgically treated 36-year-old man with a diagnosis of progressive polyostotic fibrous dysplasia in the craniomaxillofacial region. ...

The orbitofrontal fibrous dysplasia is a rare, non-malignant disease that involves the development and thickening of craniofacial bones, causing facial asymmetry, visual, neurological and dental complications. Decreased visual acuity in orbitofrontal and sphenoidal fibrous dysplasia appears as a result of compressive lesions on the optic nerve. The aim of this article is to present the ophthalm...

INTRODUCTION McCune-Albright syndrome (MAS) is a rare disorder characterized by the classic triad of precocious puberty, polyostotic fibrous dysplasia and café-au-lait pigmented skin lesions. Cystic change is rare in fibrous dysplasia (FD), especially in McCune-Albright syndrome. There were no reports about cyst degeneration in MAS which resulted in abnormal visual acuity and visual fields. Her...

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 ...

INTRODUCTION Mazabraud's syndrome is a rare but well-described disorder characterized by fibrous dysplasia in single or multiple bones associated with one or more soft-tissue myxomas. In this report, we describe what is, to the best of our knowledge, the first case involving an intra-osseous myxoma. This finding supports, and could provide new insight into, the pathological association between ...

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...

BACKGROUND Interpretation of small biopsy fragments from suspected lesions of fibrous dysplasia with unusual clinical and/or radiological features may be challenging due to wide histomorphological spectrum of stromal appearances. Awareness of these variations should improve diagnostic confidence. METHODS We retrospectively studied 26 cases of fibrous dysplasia (F- 19, M- 7; Ages ranged from 1...

McCune-Albright syndrome is a rare sporadic disease characterized by bone fibrous dysplasia, café-au-lait skin spots and a variable association of hyperfunctional endocrine disorders. Fibrous dysplasia (FD), which can involve the craniofacial, axial, and appendicular skeleton, may range from an isolated, asymptomatic monostotic lesion to a severe disabling polyostotic disease involving the enti...

The syndrome of McCune-Albright syndrome (MAS) is clasically defined as a triad presentation with the findings of polyostotic fibrous dysplasia, café-au-lait spots, and sexual precocity. However, not all patients present with complete symptoms. A 52-year-old man was diagnosed as having a variant of McCune-Albright syndrome with the following findings: polyostotic fibrous dysplasia, acromegaly d...

A NUMBER of bone diseases may affect the orbital bones decreasing the orbital volume and narrowing the optic canal, giving rise to unilateral exophthalmos and usually optic atrophy, simulating clinically an orbital tumour. Among such diseases are osteitis deformans (Paget, 1876); leontiasis ossea (Virchow, 1896); osteopetrosis (Albers-Schonberg, 1904), and the generalized and localized osteitis...