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PubMed1977

Radionuclide angiography--brain and bone imaging in craniofacial fibrous dysplasia (CFD): case report.

Craniofacial fibrous dysplasia (CFD) may show arterial displacement or increased uptake during arterial, capillary, venous, and blood-pool phases of radionuclide angiography. Extensive single or multiple areas of uptake are present on brain and bone images. These findings are probably due to the highly vascular bone often found in CFD. The distribution of the areas of abnormal uptake, along with pertinent clinical history and skull radiographs, usually alllows conditions to be excluded.

Adult↗
PubMed1982

[MacCune-Albright syndrome. Apropos of a case].

The MacCune-Albright's syndrome as described by these authors in 1936 and 1937 is extremely rare if one considers the complete form consisting in the triad: café-au-lait spots, fibro-osseous dysplasia, endocrine disturbances. We report an additional case in a 5 year old girl with an precocious puberty. Within a 10 year follow up occurred a polyostotic fibrous dysplasia with pathologic bone fractures in cystic areas, and a right exophthalmos induced by a cyst of the large wing of the sphenoid bone. All these lesions led to orthopaedic and neural surgical procedures. The early puberty of central origin (as proved by the LH-RH test) was treated during 7 years by medroxyprogesterone acetate with an excellent result. No peripheral endocrine syndrome was evidenced. This case stresses the multiple problems arising in the disease and the necessity of a long lasting careful follow up.

Child, Preschool↗
PubMed2004

Pseudoanaplastic tumors of bone.

OBJECTIVE: To discuss the concept of pseudoanaplastic tumors of bone, which pathologically show hyperchromatism and marked pleomorphism with quite enlarged, pleomorphic nuclei, but with no to extremely rare, typical mitoses, and to propose guidelines for their diagnosis. DESIGN AND PATIENTS: From a database of 4,262 bone tumors covering from 1971 to 2001, 15 cases of pseudoanaplastic bone tumors (0.35% of total) were retrieved for clinical, radiographic and pathologic review. Postoperative follow-up after surgical treatment was at least 3 years and a maximum of 7 years. RESULTS: There were eight male and seven female patients. Their ages ranged from 10 to 64 years with average of 29.7 years. Pathologic diagnoses of pseudoanaplastic variants of benign bone tumors included: osteoblastoma (4 cases), giant cell tumor (4 cases), chondromyxoid fibroma (3 cases), fibrous dysplasia (2 cases), fibrous cortical defect (1 case) and aneurysmal bone cyst (1 case). Radiography of all cases showed features of a benign bone lesion. Six cases, one case each of osteoblastoma, fibrous dysplasia, aneurysmal bone cyst, chondromyxoid fibroma, giant cell tumor and osteoblastoma, were initially misdiagnosed as osteosarcoma. The remaining cases were referred for a second opinion to rule out sarcoma. CONCLUSIONS: Despite the presence of significant cytologic aberrations, none of our cases showed malignant behavior following simple curettage or removal of bony lesions. Our observation justifies the concept of pseudoanaplasia in some benign bone tumors as in benign soft tissue tumors, especially in their late evolutionary stage when bizarre cytologic alterations strongly mimic a sarcoma.

Adolescent↗
PubMed1986

[Course and differential diagnosis of McCune-Albright syndrome].

The McCune-Albright syndrome consists of a combination of fibrous dysplasia of bone and endocrine lesions with abnormalities of pigmentation. The condition is rare and may be missed, as happened in our case. The case showed extreme skeletal deformities, menarche at one year and peculiar histological appearances.

Adult↗
PubMed1986

Monostotic fibrous dysplasia of the ethmoid. Surgical treatment using a combined endocranial and transfacial approach.

Two cases of monostotic fibrous dysplasia of the ethmoid are presented. This condition is of interest to the rhinologist because of the difficulty of differential diagnosis, its location, and the frequent tendency to local recurrence. The two patients presented in this paper were aged fifteen and seven respectively, with almost identical lesions and with very similar x-rays. A clinical picture of each patient is given, following a brief description of fibrous dysplasia. Special emphasis has been placed on the treatment which is necessarily surgical. In both cases extensive radical surgery of the lesion was performed using a combined craniofacial approach. Finally, a brief evaluation is made of these two cases.

Adolescent↗
PubMedDate not supplied

[Pseudo precocious puberty in the McCune-Albright syndrome].

McCune Albright Syndrome is characterized by the triad of polyostostic fibrous dysplasia of bones, skin pigmentation (café au lait-spots) and sexual precocity. The authors report a case of a 7 years girl with this syndrome in whom the sexual precocity is due to an autonomous hyperfunctioning of luteinized follicular cysts of ovary. The pathogenic mechanisms of this autonomy are debate.

Child↗