[Histologic and histochemical study of the articular lesions of chondrocalcinosis occurring during the course of a hemochromatosis].
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Biomedical subjects
Publications and source records attributed to A Mazabraud.
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The local intra-osseous injection of double zinc beryllium silicate into the tibial or femoral epiphysis of a rabbit causes an osteogenic sarcoma in 70 p. 100 of cases. These experimental conditions make it possible to reveal early non specific radiological alterations, later on secondary alterations corresponding to the development of the sarcoma and finally to follow the spontaneous evolution of the tumor. Moreover, this experimental process of induction of an osteogenic sarcoma by means of a local intra-osseous injection is vastly better than an intra-venous injection which causes straight-away multiple visceral lesions.
A cyto-enzymologic study of giant cells tumor revealed a high acid phosphatase activity in multi-nuclear cells (as in osteoclasts) and a very low alcaline phosphatase and peroxydase activity. Acid phosphatase was located in lysosom which were concentrated in definite cytoplasmic areas. The blood rate of this enzyme should be raised if it were excreted by the cells. Therefore a study of acid phosphatase blood rate could reinforce a diagnosis based on X-rays and a raising rate after surgery could help for early detection of recurrences.
Two cases of extra-articular synovial sarcoma are reported together with a review of the literature. The analysis of 44 cases shows that this tumor appears among young people particularly between 10 and 30 years of age and is twice more frequent among males. There can be metastasis mostly in lungs (11 times), which can sometimes appear much later (more than 10 years after the first diagnosis). The 5-year survival rate is here, 13/18; this is higher than for similar tumors located in the juxta-articular area. The great size of the tumoral nodule (more than 5 centimeters) is of a bad omen for the prognosis. As regards therapy, isolated surgical exeresis seems to have given results at least as good as these of surgery associated to radiotherapy. In all cases, the diagnosis is based on a light microscopic study which shows two tightly linked histological patterns: a proliferation of fusiform cells and glandular like formations. This feature is not definitely pathognostic and is very similar to tumors of glandular origin, with myoepithelial proliferation. Some examples could indeed be classified as extra-articular cervical synovialo-sarcoma. They could originate in pharyngeal and laryngeal mucous glands. This histogenetic hypothesis could account for the highly favorable evolution of some cases which were reported as synovialo-sarcoma.
The authors report the pathological and clinical findings in five cases of chondrosarcoma resembling chondroblastic chondrosarcomas as described by Lichtenstein and Bernstein in 1959 and, above all, clear cell chondrosarcomas as described by Unni et al. (1976). These chondrosarcomas have a double peculiarity:--topographic, for their site is often epiphyseal :--anatomica, for they associate, in the usual chondrosarcomatous sectors, areas rich in clear cells, with a rich blood supply, and in some cases a diffuse reactional osteogenesis. Although the long term prognosis is similar to that of all chondrosarcomas, their course is sometimes very slow. One should thus distinguish carefully these tumours from chondroblastomas in spite of certain topographic and radiological similarities.
Malignant hemangioendothelioma, localised to the skeleton of the lower limbs, to the exclusion of any other bony or visceral involvement, complicated by hemolytic anemia and spontaneous fractures, edl to death in spite of bilateral amputation, less than one year after its chance discovery. The multifocal character of the bony lesions, which is common, may suggest either rapidly developing metastases, or multicentric development of the tumour. The clinical, radiological and laboratory signs--pure osteolysis of large size without condensation nor periostosis, are not specific. Pathological examination permits recognition of the vascular origin of the tumour but does not always permit one to recognize its malignant nature. Treatment, which is mainly surgical, is often ineffective.
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