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Size dependent enzyme activities of multinucleated (osteoclastic) giant cells in bone tumors.

In osteoclastic giant cells of six different tumors of bones and joints (fibrous dysplasia, proliferating giant cell tumor, malignant giant cell tumor, osteosarcoma after chemotherapy, malignant synovioma and Ewing's sarcoma) activities of tartrate-resistant acid phosphatase, NADH-tetrazolium-oxidoreductase and, in three of them, of non-specific esterase are determined by enzyme histochemical methods. Quantitative microphotometry makes it possible to determine relative enzyme activities in the cut sections of giant cells of different sizes. Giant cells of the various tumors reveal similar trends: With an increase in cell size, mean extinctions of NADH-tetrazolium-oxidoreductase and non-specific esterase decrease. Mean extinctions of tartrate-resistant acid phosphatase increase in cells of medium size, whereas the large cells reveal in part low activities. An additional ultrastructural examination of the giant cells in the proliferating giant cell tumor as well as in the osteosarcoma shows morphological signs of degeneration in the large cells. Electron probe microanalysis of the proliferating giant cell tumor exhibits evidence of phagocytosis of Ca and/or Fe containing particles. The similar size dependent reaction pattern of enzymes in osteoclastic giant cells of different tumors favors the concept of a common histogenesis, i.e. a host reaction.

Acid Phosphatase↗

McCune-Albright syndrome associated with non-autoimmune type of hyperthyroidism with development of thyrotoxic crisis.

We report on a patient having McCune-Albright syndrome (MAS) associated with non-autoimmune hyperthyroidism associated with thyrotoxic crisis. Polyostotic fibrous dysplasia developed at age 8, and café-au-lait pigmentation was noted on the skin. At age 18, he developed hyperthyroidism with multiple adenomatous changes. The hyperthyroidism had been controlled with an antithyroid drug, but the antithyroid medication was discontinued by the patient at age 23. One year later, thyrotoxic crisis developed with fever, convulsions and loss of consciousness. Thyroid function tests showed serum concentrations of free T(4) of 5.1 ng/dl, and serum TSH of <0.1 microU/ml. Serum thyroglobulin concentrations were markedly increased (1,280 ng/ml). Three major thyroid-related autoantibodies (TSH receptor antibody, antithyroglobulin, and antimicrosomal antibodies) were not detected in serum. Serum GH concentrations were increased, and not suppressed by the glucose tolerance test, but increased paradoxically by TRH. The thyrotoxic crisis was ameliorated by treatment with a beta-adrenergic receptor-blocking agent, glucocoroticoid, iodine, antithyroid drug, and antibiotics. The cause of thyroidal defect in our patient is not considered to be autoimmune hyperthyroidism, but hyperthyroidism due to constitutive activation of G(s)alpha by inhibition of its GTPase. This paper describes, as far as we know, the first case of MAS associated with thyrotoxic crisis. Because hyperthyroidism in this patient recurred quickly after discontinuation of the antithyroid drug, the mode of treatment for MAS-associated hyperthyroidism appears to be total surgical ablation or repetitive radioiodine therapy.

Adult↗

Oncogenic osteomalacia: a clinicopathologic study of 17 bone lesions.

Oncogenic osteomalacia is an unusual and rare clinicopathologic syndrome characterized by mesenchymal tumors that apparently produce osteomalacia and biochemical abnormalities consisting of hypophosphatemia, normocalcemia, and increased levels of alkaline phosphatase. We collected from the Mayo Clinic files and from our consultation files the records for 17 cases of osteomalacia associated with bone lesions. There were five cases of fibrous dysplasia, three of hemangiopericytoma, and two of phosphaturic mesenchymal tumor. There was one case each of osteosarcoma, chondroblastoma, chondromyxoid fibroma, malignant fibrous histiocytoma, giant cell tumor, metaphyseal fibrous defect, and hemangioma. In this study we can figure out that the most common characteristic histologic features of our cases were hemangiopericytomatous vascular proliferation, fine lace-like stromal calcification, and stromal giant cells. In most of the cases, the clinical and biochemical symptoms and signs resolved soon after complete resection of the lesion. When the lesion recurred or metastasized, the symptoms and signs also recurred.

Adolescent↗

[Fibrous dysplasia].

The observations in 222 cases of fibrous dysplasia of the Hamburg Bone Tumor Registry will be presented. This benign lesion is based on the appearance of postzygotic point mutations in a gene encoding the Gsa protein. It occurs as a mono- and a polyostotic variant and may affect every bone of the skeleton. Most often affected are the proximal femur, the skull and the ribs. Polyostotic lesions tend to occur on one side of the body. The monostotic form is 7.6 times more frequent, but both variants show no predilection for gender. Most cases are diagnosed during adulthood. On x-rays the lesion has a ground-glass appearance and is located intramedullary with a sharply defined edge. The cortical bone is arroded and the bone is expanded. Histologically typical signs are slender curved fibrous trabeculae with a C and Y shape embedded in a morphologically bland and moderately cellular fibrous stroma. Diagnostically important are collagen fibres emerging perpendicular from the surface of the trabeculae. Cartilage is present in 8% of the cases. Therapeutically, thorough clinical controls are indicated and operative procedures are rarely needed to prevent progressive deformities and fractures. The formerly applied radiotherapy is now obsolete because of the increased occurrence of malignant transformations.

Bone Resorption↗

Fibrous dysplasia of the jaws. Report of five cases.

A brief review of the literature concerning fibrous dysplasia and fibro-osseous lesions of the jaws is presented. Five cases of fibrous dysplasia are described. The manner of presentation, diagnosis, histologic appearance, and treatment of this disease entity are discussed in light of previously published data. Histologically, two cases showed the "classic" features of woven bone in the fibrous stroma. The remaining three cases showed variable amounts of lamellar bone. The presence of lamellar bone is not considered incompatible with a diagnosis of fibrous dysplasia of the jaw, especially in older patients and in those in whom the lesion is of longer duration.

Adult↗

Benign primary tumours of the ribs.

Benign primary tumours of the ribs are rare and may be identified during the investigation of a clinically suspected abnormality or as an incidental finding. This review describes the spectrum of benign tumours that can involve the rib and illustrates the radiographic, computed tomography (CT) and magnetic resonance (MR) imaging appearances that may facilitate diagnosis.

Bone Cysts, Aneurysmal↗

Atypical simple bone cysts of the jaws. II: A possible association with benign fibro-osseous (cemental) lesions of the jaws.

Four cases are described of simple bone cysts of the mandible arising concurrently with benign fibro-osseous (cemental) lesions. Previous reports of such findings, and a possible relationship to the so-called 'cementoma' of the long bones, are considered. The relevance of this to the aetiology is addressed, and it is suggested that at least some cases diagnosed as simple bone cysts are closely related to the fibro-osseous lesions.

Adult↗

Uremic mixed bone disease. A case report.

Renal osteodystrophy (ROD) is a complex disease of bone caused by renal failure. A case of uremic mixed bone disease with severe maxillofacial involvement is reported. The pathogenesis and various pathologic features of ROD are discussed.

Adult↗

Correlation of bone histology with parathyroid hormone, vitamin D3, and radiology in end-stage renal disease.

We analyzed transiliac bone biopsy specimens from 30 end-stage renal failure patients, taken at the time of admission for CAPD training. Results were compared with values of iPTH, bone alkaline phosphatase, 1,25-dihydroxyvitamin D3, skeletal survey, quantitative computed tomography (QCT) and single photon absorptiometry (SPA) bone density measurements. Osteitis fibrosa was the most common histological diagnosis, present in 15 of the 30 patients (50%), with eight classified as "severe" and seven as "mild." Eight patients (27%) had adynamic bone lesion, four mixed renal osteodystrophy (13%), and two (7%) osteomalacia. The mean age of the adynamic group was higher than the osteitis fibrosa group (41 +/- 12.1 vs. 56 +/- 10.2 years; P < 0.01), and than the mixed group (39 +/- 7.5 vs. 56 +/- 10.2 years; P < 0.02). Levels of iPTH enabled discrimination between groups, but not between individual patients, and values correlated with bone alkaline phosphatase (r = 0.62; P < 0.001). Erosion of the terminal phalanges was seen on the plain X-rays of 7 of 15 patients with mild or severe OF, and in three patients with another diagnosis. The majority of patients (> 90%) had bone density measurements within the normal range. No significant correlation existed between QCT or SPA scores and any of the histomorphometric parameters, or iPTH. We conclude that iPTH is the most helpful non-invasive investigation in this group of patients. Plain X-ray of the hands is the most useful radiological investigation, but single measurements of bone density are not diagnostic.

Adult↗