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  • 1
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Extraskeletal myxoid chondrosarcomas (EMC) are characterized by recurrent t(9; 22) or t(9; 17) translocations resulting in fusions of the NH2-terminal transactivation domains of EWS or TAF2N to the entire TEC protein. We report an EMC with a new translocation, t(9; 15)(q22; q21), and a third type ...
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 416 (1990), S. 237-248 
    ISSN: 1432-2307
    Keywords: Neurilemoma ; Pseudosarcoma ; Electron microscopy ; Immunohistochemistry ; Cytogenetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A series of 29 cellular schwannomas is described in terms of their clinical presentation and course, light and electron-microscopic appearance, immunohistochemical properties and cytogenetics. The study indicates that cellular schwannoma can be defined as a subtype of classical schwannoma, characterized by spindle cells forming a compact fascicular, sometimes fibrosarcoma-like growth pattern, a low mitotic activity, a generally moderate nuclear and cellular polymorphism and a high degree of Schwann cell differentiation as seen by electron microscopy and immunohistochemistry. The tumour is characteristically located close to the vertebral column, in the mediastinum or retroperitoneum and has a benign course. Occasionally bone destruction and neurological symptoms develop. The clinical appearance together with the high cellularity, fascicular pattern and mitotic activity had led to the erroneous diagnosis of a soft tissue sarcoma in a few cases, and cellular schwannoma may thus be considered to be a pseudosarcoma. Immunohistochemically, cellular schwannomas appear to deviate from classical schwannomas and malignant peripheral nerve sheath tumours by their expression of glial fibrillary acidic protein. The chromosome analysis revealed a normal diploid stemline karyotype, with a variety of abnormal clones, including one with monosomy 22.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 417 (1990), S. 377-388 
    ISSN: 1432-2307
    Keywords: Malignant melanoma ; Soft tissue sarcoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Metastases of cutaneous malignant melanoma (MM) of ordinary type can resemble various types of soft tissue sarcoma light microscopically to a degree which has not been previously recognized. Twenty-one cases are described, in which the tumours were originally diagnosed as a soft tissue sarcoma. Seven tumours were predominantly of blue and spindle-cell, fascicular type, resembling malignant peripheral nerve sheath tumour and at times monophasic synovial sarcoma. Ten tumours which were of fascicular and predominantly storiform type, and included uni- and multi-nuleated pleomorphic cells resembled malignant fibrous histiocytoma. Due to the presence of multivacuolated lipoblast-like tumour cells, 2 of these 10 tumours resembled pleomorphic liposarcoma. One had a predominantly myxoid and hypocellular appearance and 5 additional tumours included such areas. The diagnoses were revised after ultrastructural examination with the demonstration of melanosomes in 13 of 16 studied cases and the immunohistochemical demonstration of positivity using anti-S-100 protein antibodies and the anti-melanoma antibody NKI/C3 in all cases. The anti-melanoma antibody HMB 45 gave a positivity in 9 of 21 cases. Light microscopically, sparse amounts of melanin were noted in 7 tumours using the Whartin-Starry technique. Eleven tumours occurred at sites close to major lymph node groups and in 9 of these cases, lymphoid tissue was associated with the tumours, suggesting that they represented lymph node metastases. Following a review of the patients' clinical histories and renewed clinical examination, primary cutaneous MM was demonstrated in 10 of 21 patients and in 1 case an MM in regression was detected. The origin of the 10 tumours without a detected primary is discussed, including the possibility of an overlooked primary, spontaneous regression of a primary and a de novo origin from lymph nodes and soft tissues.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 409 (1986), S. 433-451 
    ISSN: 1432-2307
    Keywords: Malignant schwannoma ; Electromicroscopy ; Immunohistochemistry ; S-100 protein ; Neuronspecific enolase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report a light and electron microscopic, immunohistochemical, clinical and prognostic study of 14 patients with epithelioid malignant schwannoma. In 8 patients the tumour involved a major nerve. The tumours were rather small in most instances, the largest diameter being less than 5 cm in 7 cases. Light microscopically, they showed highly cellular areas of epithelioid, polygonal or rounded cells characteristically forming cords and rows and arranged in nodules of varying size. Spindle cell sarcoma areas as in classical malignant schwannoma were seen in 9 cases, and neurofibromatous areas in one case. Four cases were entirely epithelioid in appearance. Electron microscopically the epithelioid tumour cells showed nuclei with mostly even contours containing one or two trabecular or reticular nucleoli, cytoplasmic projections, intra-cytoplasmic myelin-like figures, intercellular junctions and discontinuous, sometimes multilayered external lamina material. The ultrastructural findings indicate that epithelioid malignant schwannoma is a tumour of neural crest derivation having features of Schwann cell differentiation. Immunohistochemically, S-100 protein was demonstrated in 7 tumours and neuron specific enolase in 3. There was a female predominance, 9/14, and a median age of 38.5 years (range 17-74). The extremities, including the hip and shoulder regions, were the most common sites, 12/14. The tumour proved highly malignant; 9 of 14 patients were dead at the time of follow-up and a high incidence of metastasis (7 of 14) was observed.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 401 (1983), S. 109-128 
    ISSN: 1432-2307
    Keywords: Clear-cell sarcoma ; Electron microscopy ; Immunohistochemistry ; Neural crest ; S-100 protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A clinico-pathological, light microscopic and immunohistochemical study of 15 clear-cell sarcomas, with an ultrastructural analysis of 6 of the tumors, is presented. The tumors showed a strong predilection for tendons and aponeuroses of the extremities in predominantly young and middle-aged people. The clinical setting, course and light microscopic appearance agree well with the original description by Enzinger (1965). Nine of the 15 patients developed metastases, most of them including lymph nodes, and 8 of the patients had died at the time of follow-up (median follow-up time 4.8 years). Reducing pigment was demonstrated within the cells of 2 tumors. Ultrastructurally the 6 tumors studied had a uniform appearance with characteristically rounded or oval tumor cells with a single nucleus containing one or two very prominent nucleoli, a light-staining cytoplasm with a moderate amount of organelles and a variable content of glycogen. Polymorphic melanosomes were seen in the cells of one of the tumors. External laminas enclosed groups of tumor cells and invested parts of individual tumor cells. With immunoperoxidase analysis for S-100 protein positive staining was observed in the vast majority of the tumor cells of all 15 clear-cell sarcomas. Metastases appearing in 9 of the 15 cases showed positive staining for S-100 protein. There was a strong staining of the cytoplasm and generally a weak and varying staining of nuclei. The immunohistochemical and electron microscopic findings indicate that clear-cell sarcoma is a homogenous entity among soft tissue sarcomas, of probable neural crest derivation.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 419 (1991), S. 439-445 
    ISSN: 1432-2307
    Keywords: Angiosarcoma ; Cell culture ; Cytogenetics ; Lectins ; Stewart-Treves syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A morphological and cytogenetic analysis of a multifocal angiosarcoma in a typical case of Stewart-Treves syndrome is reported. The morphological analysis indicated differentiation along both blood and lymph vessel endothelium lines. By light and electron microscopy there were areas with well-developed erythrocyte-containing, capillary-like vessels and poorly differentiated areas with abortive vascular formations. In these the endothelium revealed immunoreactivity to factor VIII RAg, binding ofUlex europaeus I andPsophocarpus tetragonolobus agglutinin lectins, Weibel-Palade bodies ultrastructurally and presented a continuous enclosing external lamina and immunoreactivity for laminin and collagen IV, all features of blood-vessel differentiation. There were also lymphangioma-like areas as well as poorly differentiated areas where the immunohistochemical, lectin-binding and ultrastructural features were compatible with a lymph vessel differentiation. Cytogenetic analysis of cultured tumour cells revealed chromosome counts in the diploid region. About 40% of the cells analysed had a normal diploid karyotype. The remaining cells showed a multitude of mainly nonclonal structural alterations; 17 unique marker types resulting from different translocations and deletions were observed. There were also a few cells with clonal numerical deviations showing monosomy 22, monosomy X and trisomy 2 respectively. It is of interest that the losses of chromosome 22 and the X chromosome also have been observed in Kaposi's sarcoma and that the PD-ECGF gene, a novel angiogenetic factor, has been mapped to chromosome 22.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 381 (1979), S. 121-139 
    ISSN: 1432-2307
    Keywords: Myxofibrosarcoma ; Fibroblast ; Histiocyte ; Myofibroblast ; Sarcoma ; Soft tissue tumour ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An ultrastructural study of 11 myxofibrosarcomas is presented. The tumours were graded light-microscopically on a four-point scale according to cellularity, cell atypia and mitotic activity, as described in a previous paper: 3 were grade I, 2 grade II, 4 grade III and 2 grade IV. Fibroblast-like and myofibroblast-like cells predominated in the grade I and II tumours, while histiocyte-like cells were relatively few; in grade III and IV tumours histiocyte-like cells predominated. The fibroblast-like cells were spindle-shaped with fairly smoothly outlined nuclei, and contained abundant parallel membranes and wide cisternae of endoplasmic reticulum. The histocyte-like cells were irregular in shape and showed indented nuclei with large nucleoli and coarse, peripherally arranged clumps of heterochromatin. The cytoplasm of these cells showed prominent pseudopodiae, microvillous projections, conspicuous systems of vesicles and vacuoles, inclusions of lipid and pigment, numerous lysosomal structures and large digestive vacuoles indicating auto- and exophagocytosis. The myofibroblast-like cells showed abundance of parallelly arranged cytoplasmic microfilaments with dense body-like structures and condensation at the plasmic membrane. Occasional cells appeared to be intermediate forms and primitive looking, undifferentiated cells were also encountered. A few multinucleated tumours cells were seen in the grade IV tumours. Thus, the present study shows the composite fibroblastic, myofibroblastic and histiocytic character of the tumour cells of myxofibrosarcoma; the grade III and IV tumours, with a tendency to contain solid areas, seem to be closely related to the pleomorphic type of malignant fibrous histiocytoma.
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 389 (1980), S. 27-41 
    ISSN: 1432-2307
    Keywords: Chondrosarcoma ; Clear-cell chondrosarcoma ; Glucosaminoglycans ; Histochemistry ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two cases of clear-cell chondrosarcoma located in the upper end of the right femur of men aged 30 and 40 years are reported. The roentgenologic appearances suggested a chondroblastoma. Both patients are alive, one and four years after surgical removal of the tumor. Glucosaminoglycans were studied with cationic dyes at different pH, with and without pretreatment with testicular hyaluronidase, and with the Scott technique at the light-microscopic level. Ultrastructurally, the glucosaminoglycans were studied with the high iron diamine and dialyzed iron techniques and glycogen with the PATCH-method. Light-microscopically, the tumors were characterized by clear vacuolated cells with distinct cytoplasm boundaries and scattered multinucleated giant cells of osteoclast type. Histochemical studies at the light-microscopic level indicate the presence of chondroitin 4- and 6-sulphate but no keratosulphate. Ultrastructurally, the predominant clear-cells showed features characteristic for chondroblasts. The cytoplasm showed areas lacking organelles and containing a low-density, finely granular matrix. These areas are considered to correspond to the clear cytoplasmic vacuoles seen under the light microscope. Most of the organelles were seen in the perinuclear region. The irregular tumor cells formed delicate protruding cytoplasmic extensions, which delineated intercellular spaces appearing as vacuoles under the light microscope. The benign multinucleated giant cells had an ultrastructural appearance typical of osteoclasts. Histochemical analysis at the electron-microscopic level showed the presence of sulphated glucosaminoglycans in the intercellular matrix and in association with the cytoplasmic membrane. Glycogen and non-sulphated acid glucosaminoglycans were found within the cytoplasm of the clear-cells.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Skeletal radiology 16 (1987), S. 364-376 
    ISSN: 1432-2161
    Keywords: Bone neoplasms ; Leiomyosarcoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Sixteen cases of primary leiomyosarcoma of bone are described. The patients, 11 males and 5 females, ranged in age from 9 to 74 years. The annual incidence of this tumor in Sweden was calculated to be 0.09 cases per million. This figure was obtained by reviewing a Swedish series of spindle cell sarcomas of bone of which one quarter (11/44) were diagnosed by us as primary leiomyosarcoma. The diagnosis was based on light- and electron-microscopic examinations using the same criteria as for leiomyosarcoma of soft tissues. Thirteen tumors were located in a long bone of an extremity (nine close to the knee joint) and three in the central skeleton. Radiographically, all the tumors presented as a purely osteolytic lesion, and three patients had sustained a pathologic fracture. In four of six cases angiography suggested malignancy by revealing hypervascularity, irregular tortuous vessels, and diffuse contrast opacification. Contrast-enhanced computed tomography, performed in two cases, showed hypervascular areas within the tumors. Scintigraphy showed a marked increase in radionuclide uptake in all five cases studied. The clinical behavior indicates that primary leiomyosarcoma of bone is highly malignant. Eight patients had died of the tumor and, of the eight patients who were alive at follow-up, two had metastases, and one had been operated on three times for a cutaneous metastasis, which had recurred locally twice. The remaining five patients had been continuously free of disease for 6.5 to 12.3 years.
    Type of Medium: Electronic Resource
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