Cases reported "Fibrosarcoma"

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1/8. Oncogenous osteomalacia: a case study.

    A case of oncogenous osteomalacia due to a fibrosarcoma of the maxilla is reported, with a 19 year course before treatment. Metabolic studies of calcium and phosphorus were performed 3 and 19 years after the first symptomology. There was a negative balance for both phosphorus and calcium with low serum levels of 1,25-dihydroxyvitamin D which were corrected by resection of the tumor. Portions of the tumor were cultured and the supernatant did not affect phosphorus transport by a proximal tubule kidney cell line. Other portions were injected into athymic nude mice where they resulted in hypophosphatemia and phosphaturia, thus confirming the endocrine nature of the oncogenous osteomalacia factor.
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2/8. The fibroblastic nature of dermatofibrosarcoma protuberans: morphological investigations in vivo and in vitro.

    Six cases of dermatofibrosarcoma protuberans were studied ultrastructurally. biopsy material from all six cases as well as cell cultures derived from four cases were examined. In all cases, the tumor cells in vivo and in vitro were related to fibroblasts. The two cases with histiocytoid features and a small spiral pattern exhibited numerous lipid vacuoles but no histiocytic cell markers. In these two cases, cultured cells contained a higher number of intracytoplasmic vacuoles than the control fibroblasts. Two other cases exhibited some basement-membrane-like material surrounding tumor cells. All of the investigated cell strains obtained from the tumors showed synthesis of collagenous proteins similar to that found in fibroblasts. However, the level of total collagen was reduced, and type-III collagen was absent. The morphological variations in these cases of dermatofibrosarcoma protuberans appeared to be related to the histological pattern and may reflect the heterogeneity of normal fibroblasts.
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3/8. Chromosome changes in soft tissue sarcomas.

    An analysis of chromosome aberrations in human tumors was performed in 29 cases of soft tissue sarcoma. The tumor tissues were disaggregated with collagenase and the cells cultured for 2-3 days. Analyzable metaphases were obtained in 15 cases, 4 of which showed only normal karyotypes. The remaining 11 tumors showed various numerical and structural abnormalities in their karyotypes: 8 tumors were near-diploid and the remaining 3 were near-triploid. G- and Q-banding analyses revealed clonal abnormalities in the 11 cases with the presence of marker chromosomes; 15 different chromosomes were involved in chromosome rearrangements, chromosomes 1 and 2 being the most frequently affected. Because of the heterogeneity of the tumor group investigated (neurogenic sarcoma, 2 liposarcomas, neurofibrosarcoma, synovial cell sarcoma, fibrosarcoma, mesothelioma, leiomyosarcoma, rhabdomyosarcoma, Ewing's sarcoma, and hemangiopericytoma), it was impossible to reach any conclusion on the specificity of the cytogenetic abnormalities for a particular tumor type.
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4/8. dermatofibrosarcoma protuberans and its tissue culture study -- ultrastructural, enzyme histochemical and immunological study.

    dermatofibrosarcoma protuberans arizing in the thigh of a 23-year-old female is reported with an investigation of tumor cell character by electron microscopy, enzyme histochemistry, immunological and in vitro tissue culture techniques. The tumor tissue showed histologically a peculiar storiform arrangement of plump spindle cells with dense proliferation. Electron microscope revealed histiocytic cell structures of some tumor cells: lysosomes, phagosomes, and sparse rough endoplasmic reticula. They revealed diffuse positive reactions to acid phosphatase (Ac Pase) and non-specific esterase. Short-time culture of the tumor tissue indicated rather round and polygonal cells admixed with spindle cells, positive to Ac Pase and non-specific esterase with diffuse reaction. Most of the round cells possessed Fc and C3 receptors on their surface and phagocytic activity. dermatofibrosarcoma protuberans is probably a histiocytic tumor and structural characteristics of schwann cells were not recognized.
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5/8. dermatofibrosarcoma protuberans: a case report and clinicopathologic review.

    dermatofibrosarcoma protuberans is a soft tissue, low-grade malignant tumor. Histologically, the tumor is a low-grade sarcoma with a constant storiform growth pattern. The tumor may possibly arise from histiocytes, a thesis supported by recent tissue culture and electron microscopic studies. Treatment consists of the wide local excision. Conservative excision often results in recurrences. lymph node and other distant metastases, although rare, have been documented. Most often, the clinical course shows this tumor to be slow growing and often painless. Presented herein is a patient in whom this uncommon tumor developed.
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6/8. The histiocytic nature of dermatofibrosarcoma protuberans. Tissue culture and electron microscopic study.

    Specimens from four cases of dermatofibrosarcoma protuberans when explanted in vitro exhibited a typical ameboid outgrowth, confirming the histiocytic nature of these tumors. The cultures displayed the same evolution from macrophages to fibroblast-like cells observed previously in cultures of other types of histiocytomas. Electron microscopic study of the cultures confirmed the light microscopic findings and suggested that the fibroblast-like cells were histiocytes with some secondarily acquired fibroblastic features. The possibility that neoplastic histiocytes may transform into true fibroblasts was deemed unlikely, but could not be excluded.
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7/8. Translocation t(12;22)(q13;q12.2-12.3) in a clear cell sarcoma of tendons and aponeuroses.

    cytogenetic analysis of a short-term culture from a clear cell sarcoma revealed a complex karyotype with the mainline of 49,XY,t(7;18)(p11.2;q21.3), der(7)t(7;18)(p11.2;q21.3), 8, der (8;17)(q10;q10),t(12;22)(q13;q12.2-12.3),add(13)(p13). An apparently identical translocation t(12;22) has been described recently in four clear cell sarcomas, indicating that this constitutes a primary cytogenetic change specific for this type of tumor. In our case, the breakpoint on chromosome 22 could be assigned to band 22q12.2 or 22q12.3. Together with the present case, trisomy or tetrasomy 8 has been found in six of nine clear cell sarcomas, suggesting that, as in Ewing's sarcoma and myxoid liposarcoma, trisomy/tetrasomy 8 represents a nonrandom secondary aberration. We conclude that the finding of the specific translocation t(12;22) may prove to be an important marker in the differential diagnosis of clear cell sarcoma from some other soft tissue sarcomas and malignant melanoma.
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8/8. cytomegalovirus infection in two infants with congenital or neonatal solid tumors.

    Two infants with fibrosarcoma and rhabdomyosarcoma diagnosed prenatally and at 2 months of age, respectively, and cytomegalovirus (CMV) infection are reported. Concomitant CMV infection was revealed by positive urine culture and/or CMV dna, and CMV-specific IgM, IgA, and complement-fixing antibodies. The patients, showing very low levels of immune T cells, died at 3 and 8 months of age, respectively. A pathogenic role for CMV in the progression of the tumors is suggested.
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