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At least 649 records · Page 36Linked to original sources
PubMed2005

Fibrous dysplasia in combination with aneurysmal bone cyst presenting as a subarachnoid haemorrhage.

Fibrous dysplasia (FD) is a rare tumour, representing 2.5% of all bone tumours and 7% of benign tumours. Aneurysmal bone cyst (ABC) is also an uncommon pathology, usually associated with a secondary vascular lesion consisting of an arteriovenous malformation. In this article, we relate a case report of a young female with a rare combination of FD with aneurysmal bone cyst presenting as a subarachnoid haemorrhage (SAH). Despite the possibility of clinical treatment of these lesions, this report demonstrates that symptomatic lesions may be successfully managed by surgical resection. The authors' opinion is that this treatment should be individualised depending on particularities of each case, such as localisation of the lesion, biopsy results and image exams features. We also present a critical literature review of diagnostic methods and therapeutical options for both ABC and FD, with emphasis on controversial topics surrounding these issues.

Adult↗
PubMed2004

Monostotic fibrous dysplasia originating from ethmoid bone: treatment with endoscopic approach.

We present a case of monostotic fibrous dysplasia of the ethmoidal sinus in an 11-year-old boy. This condition is of interest to the otorhinolaryngologist because of the difficulty of differential diagnosis and treatment. This tumorlike growth was not restricted to the right ethmoidal sinus, but also compressed the orbit and the globe. Endoscopic and transnasal removal of the mass with a drill was performed under general anesthesia. No residual tumor was observed 6 months later.

Child↗
PubMed2006

Pegvisomant for the treatment of gsp-mediated growth hormone excess in patients with McCune-Albright syndrome.

CONTEXT: GH excess affects approximately 20% of the patients with McCune-Albright syndrome (MAS). MAS is caused by sporadic, postzygotic, activating mutations in the GNAS gene, which codes for the cAMP-regulating protein, G(s)alpha (gsp oncogene). These same mutations are found in approximately one third of the sporadic cases of acromegaly. OBJECTIVE: We examined efficacy of the GH receptor antagonist, pegvisomant, in controlling gsp oncogene-mediated GH excess and skeletal disease (fibrous dysplasia of bone) associated with MAS. SETTING AND PATIENTS: Five MAS patients with GH excess were treated with 20 mg/d sc injection of pegvisomant for 12 wk in a randomized, double-blind, placebo-controlled crossover study at the National Institutes of Health. MAIN OUTCOME MEASURES: The primary measure of efficacy was normalization of IGF-I. Secondary outcome measures were reduction in serum IGF binding protein-3 (IGFBP-3), improvement of fatigue and sweating, and reduction in markers of bone metabolism and bone pain. RESULTS: Combined mean changes in serum IGF-I at 6 and 12 wk were -236.4 ng/ml (53%, P < 0.005) and -329.8 ng/ml (62%, P < 0.001), respectively. IGFBP-3 decreased by 0.8 mg/liter (24%, P < 0.01) and 2.9 mg/liter (37%, P < 0.005), respectively. There were no significant changes in signs and symptoms of acromegaly or markers of bone metabolism and bone pain, nor was there a significant change in pituitary size. Retrospective comparison of the degree of control achieved with pegvisomant vs. other medications (long-acting octreotide +/- dopamine agonist) in the same group showed that the two regimens were similarly effective. CONCLUSIONS: Pegvisomant effectively reduced IGF-I and IGFBP-3 levels in gsp-mediated GH excess but had no effect on fibrous dysplasia.

Bone and Bones↗
PubMed1977

The radiologic features of fibrous dysplasia of the craniofacial bones.

The radiographs of twenty-five patients (eight males, seventeen females) with histologically proven craniofacial fibrous dysplasia were analyzed. The lesions could be classified into six radiologic types: (1) peau d'orange, (2) whorled plaquelike, (3) diffuse sclerotic, (4) cystlike (multilocular or unilocular), (5) pagetoid, and (6) chalky types. The peau d'orange type was by far the most common (40 per cent) followed by the plaquelike (20 per cent, cystlike (16 per cent), and sclerotic (12 per cent). Most of the patients were young, with 60 per cent being underthe age of 20 years and 96 per cent under the age of 40 years.

Adolescent↗
PubMedDate not supplied

[Monostotic fibrous dysplasia of the temporal bone: description of a case and review of the literature].

Only a few cases of fibrous dysplasia of the temporal bone (monostotic form) have been described in the literature. The recent observation of a 15-year-old child presenting this pathology led the authors to review the literature available and to analyze the most common aspects of this disease. The clinical and radiological aspects, the differential diagnosis, histopathological features and therapeutical approaches are discussed.

Adolescent↗
PubMed2001

Positivity of extrapulmonary Ga-67 uptake in sarcoidosis: thyroid uptake due to chronic thyroiditis and bone uptake due to fibrous dysplasia.

Ga-67 citrate scintigraphy was performed on a 29-year-old man who had been diagnosed as having pulmonary sarcoidosis by a transbronchial lung biopsy. A Ga-67 citrate scintigram showed increased uptake not only in the pulmonary hilum and mediastinum, but also in the thyroid gland and the right ilium. Chronic thyroiditis was confirmed by aspiration biopsy of the thyroid gland, and fibrous dysplasia was confirmed by CT guided biopsy of the right ilium. Extrapulmonary Ga-67 uptake in patients with sarcoidosis does not necessarily indicate the involvement of other tissues and organs.

Adult↗
PubMedDate not supplied

Cranial sclerosis with striated bone disease (osteopathia striata).

This report concerns another case of striped bone disease (Osteopathia striata) together with sclerosis of the base of the skull. The mode of inheritance of this rare genetic disorder is still unknown. The clinical characteristics of 20 cases reported in the international literature are summarized and compared with our own findings.

Adolescent↗