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At least 307 records · Page 17Linked to original sources
PubMed2002

Impact of endocrine hyperfunction and phosphate wasting on bone in McCune-Albright syndrome.

Skin dysplasia, as café-au-lait spots, bone fibrous dysplasia and peripheral endocrinopathies are the main clinical features of McCune-Albright syndrome (MAS). This illness is due to activating mutations of the Gsalpha protein and is spread with a mosaic pattern in affected tissues that consist of intermixed areas of normal and mutated cells. Peripheral endocrine secretion, free of hypothalamic pituitary control, is the hallmark of the endocrine syndromes: precocious puberty, Cushing's syndrome, hyperthyroidism and gigantism/acromegaly. In addition, phosphate wasting as hyperphosphaturia is often present. The impact of hormonal hypersecretion and phosphate loss on the bones of patients with MAS is poorly understood both in normal and fibrous bone tissue. As hypercortisolism and hyperthyroidism increase bone resorption, hyperestrogenism and growth hormone hypersecretion stimulate bone growth and mineralization, and phosphate wasting reduces bone mineral content. All these actions can be exerted at varying times and degrees in a single patient on lesional and non-lesional bones. Sonographic evidence of multiple diffused hyperechogenic spots in the testes of patients with MAS do not seem to be related to alterations in calcium-phosphate metabolism but rather to zonal dysplasia/hyperplasia of testicular tissue.

Acromegaly↗
PubMed1998

Concomitant fibrous dysplasia and aneurysmal bone cyst of the skull base. Case report and review of the literature.

The combination of fibrous dysplasia and aneurysmal bone cyst of the skull base is extremely rare yet it should be recognized, lest the rapid growth of the lesion and the radiological picture lead to the wrong diagnosis of a malignant process. We report on a 6(1/2)-year-old patient who suffered from concomitant fibrous dysplasia and aneurysmal bone cyst of the skull base. He is still disease-free 4 years postoperatively. The treatment of choice remains early surgical removal of the maximal amount of involved bone that can safely be achieved. There is no role for radiotherapy in the treatment of these lesions. A review of the literature is included.

Bone Cysts, Aneurysmal↗
PubMed1985

[Monostotic fibrous dysplasia in the parietal bone: a case report].

In this paper a case of monostotic fibrous dysplasia in the left parietal bone is described. This 51-year-old female was admitted to our hospital for a head injury on January 12, 1983, and a flat, painless hump (7 X 7cm) was incidentally found in the left parietal region. Plain x-ray film of the skull showed a multicystic lesion with slightly sclerotic margin in the left parietal bone, and outward bulging of the outer table without destruction of the inner table in tangential view. CT scan at the level of bone window clearly demonstrated the same abnormality and intradiploic mass separated by some bony septums. Angiography revealed no positive findings, but RI bone scan (99m Tc) showed an abnormal uptake in this lesion. On February 1, 1983, operation was performed to verify the nature of the lesion and for relief of the left-sided headache. Post operative course was uneventful. Histological findings of this mass were of inactive fibrous dysplasia with fibrous tissue replacing the normal bone and surrounded by island or scorlled edge of bones. Monostotic fibrous dysplasia in the cranial vault is rare. It is often possible to make diagnosis in this disease on the clinical course, plain x-ray film and CT scan showing cystic findings and outer table bulging, and RI scan demonstrating abnormal uptake.(ABSTRACT TRUNCATED AT 250 WORDS)

Contusions↗
PubMed1987

Fibrous dysplasia involving the temporal bone: report of three new cases.

Monostotic fibrous dysplasia, an unusual disease that can involve the temporal bone, will frequently cause an acquired stenosis of the external auditory canal. Three patients with this disorder who presented with occluded canals are described. Two individuals developed external canal cholesteatomas medial to the obstruction, one of whom eventually developed a postauricular abscess and infected draining sinus. The third patient presented with a restenosis nine months following canalplasty. The cases reported illustrate three surgical criteria necessary to manage these unusual cases successfully: removal of sufficient diseased bone to create a patulous canal; resurfacing denuded bony areas with thin split-thickness skin grafts to prevent soft tissue contractions; an adequate meatoplasty. Postoperatively, the reconstructed canals have remained patent and stable during follow-up periods ranging from one to four years. Clinical, radiographic, and pathologic features of fibrous dysplasia are discussed, the differential diagnosis is presented, and the relevant literature is reviewed.

Adolescent↗
PubMed2000

Vascularised bone grafting for fibrous dysplasia of the upper limb.

We describe our experience with vascularised bone grafting for the treatment of fibrous dysplasia of the upper limb in eight patients, five men and three women, aged between 17 and 36 years. The site was in the humerus in six and the radius in two. Persistent pain, progression of the lesion and pathological fracture with delayed union were the indications for surgical intervention. We used a vascularised fibular graft after curettage of the lesion. Function and radiological progress were serially monitored. Early radiological union of the graft occurred at periods ranging from 8 to 14 weeks. The mean period for reconstitution of the diameter of the bone was 14 months (12 to 18) predominantly through inductive formation of bone around the vascularised graft, which was a prominent feature in all patients. There were no recurrences and none of the grafts sustained a fracture or failed to unite. After operation function was excellent in three patients and good in five. Vascularised bone grafts provide a safe and reliable means of ensuring good continuity of bone with little risk of recurrence and failure.

Adolescent↗
PubMed1977

[Hemihypertrophy, nevus sebaceous, multiple bone cysts and cerebroretinal angiomatosis: a complex phakomatosis].

Report on a girl, who died at the age of 6 1/2 years having exhibited different dysplasias which are characteristic features of several phakomatoses: congenital cutaneous pigmentations, fibrolipoma of the scalp, hypertrophy of the right leg, multiple cysts of the long bones (fibrous dysplasia), and an aneurysma racemosum of the retina and mid-brain (syndrome of Bonnet-Blanc-Dechaume).

Angiomatosis↗
PubMedDate not supplied

Fibrous dysplasia of the temporal bone. Update with case reports.

Fibrous dysplasia is a fairly common, well demarcated, misdifferentiation of the bone-forming mesenchyme affecting a single, several, or many bones, in which skeletal changes are the salient feature, but in which certain endocrinopathies, abnormal pigmentation of skin and mucous membranes, and occasionally other abnormalities form part of the entire disease process. The clinical and pathological aspects of the disease and its involvement with the temporal bone have been previously discussed. Two recent case histories are added here.

Adult↗
PubMed1978

Ossifying fibroma of long bone: its distinction from fibrous dysplasia and its association with adamantinoma of long bone.

Two cases of ossifying fibroma of long bones are presented. This tumor is confused with monostotic fibrous dysplasia, but can be distinguished by its intracortical location, as demonstrated radiographically, and by its histologic pattern. Distinction from fibrous dysplasia is important since ossifying fibroma of long bone is a more aggressive lesion with different therapeutic implications. It appears that ossifying fibroma and adamantinoma of long bones are somehow related, and that lesions resembling fibrous dysplasia in association with adamantinomas of long bones are actually ossifying fibromas.

Adolescent↗
PubMed1993

Systemic fibrous dysplasia involving multiple bones in human body. Report of 4 cases.

Four cases of systemic fibrous dysplasia were selected from 356 cases of fibrous dysplasia. All of them were verified pathologically. In these 4 cases, the long, short, flat and irregular bones were involved. Some of the changes were characteristic in comparison with those of the monostotics or regional types. This disorder was often seen clinically in females, started in early childhood and exacerbated during puberty. Its main symptoms were primary or secondary deformities. Radiologically, the main changes were diffuse and uniform expansion and developmental disturbances of the bones and joints.

Adolescent↗