Cases reported "Mesenchymoma"

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1/33. rhabdomyosarcoma metastasizing as a malignant ectomesenchymoma.

    rhabdomyosarcoma is a common childhood malignancy that may occasionally occur as a component of a mixed mesenchymal tumor, e.g., a triton tumor or malignant ectomesenchymoma. A case is reported of a 13-year-old boy who had resection of a paratesticular embryonal rhabdomyosarcoma with subsequent radiation and chemotherapy. Two years later, a retroperitoneal metastasis was resected. histology showed a mixture of rhabdomyoblasts, ganglion cells, and a third population of cells with combined features of these two distinct cell types. Electron microscopy confirmed the presence of rhabdomyoblasts with characteristic bundles of myofilaments and Z-band material, and ganglion cells with prominent nuclei and nucleoli, rough endoplasmic reticulum, dense core granules, filaments, and tubules. Notably, the third cell population showed features of both rhabdomyoblasts and ganglion cells. immunohistochemistry confirmed the mixed population of rhabdomyoblasts (positive for vimentin, desmin, negative for S-100, NSE), ganglion cells (positive for S-100 and NSE, negative for vimentin and desmin), and the third population expressing all test antigens. The features of this metastatic lesion are those of a malignant ectomesenchymoma with combined rhabdomyosarcoma and ganglioneuroma components. It is postulated that this lesion results from biphenotypic expression of tumor cells that previously expressed only rhabdomyoblastic differentiation. The role of prior chemotherapy and radiotherapy in this particular case is unclear.
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keywords = rhabdomyosarcoma
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2/33. A highly aggressive primitive mesenchymal tumor with a translocation (1;19)(q12;q13.2).

    Soft tissue sarcomas constitute a heterogeneous group of malignant tumors of mesenchymal origin, the classification of which may present a diagnostic challenge. We present here the cytological, histopathological, immunohistochemical, and cytogenetic findings of an unusual case of a highly aggressive sarcoma. Based on the morphology and the immunohistochemical profile, this primitive tumor and its metastases could not be conclusively classified as any of the defined subtypes of sarcomas, although the findings were suggestive of a variant of rhabdomyosarcoma. Cytogenetic characterization using G-banding, SKY, FISH, and CGH revealed almost identical chromosomal compositions of the primary tumor and the metastasis. The hypertetraploid karyotype was characterized by numerical imbalances as well as by an unbalanced translocation t(1;19)(q12;q13.2), which has not been previously reported.
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keywords = rhabdomyosarcoma
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3/33. Malignant mesenchymoma of the heart.

    An autopsy case of malignant cardiac mesenchymoma, which arose in the atrial septum was presented. This tumor contained fibrosarcomatous, rhabdomyosarcomatous, chondrosarcomatous and osseous elements. A small metastatic lesion was found in the lung. Histogenesis of this tumor was also considered with a survey of literature.
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keywords = rhabdomyosarcoma
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4/33. ganglioneuroblastoma associated with malignant mesenchymoma.

    A case of ganglioneuroblastoma associated with malignant mesenchymoma is reported. The tumor originated from the retroperitoneum and was comprised not only of neuroblastoma and ganglioneuroma, but also of typical rhabdomyosarcoma, liposarcoma, undifferentiated mesenchymoma, as well as cartilaginous tissue. No distinct border existed between thses different components. The histogenesis of the tumor is discussed. We suggest that the tumor derived from a remnant of neural crest (ectomesenchyme).
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keywords = rhabdomyosarcoma
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5/33. Malignant mesenchymoma of the orbit: case report and review of the literature.

    OBJECTIVE: Malignant mesenchymoma are rare tumors of the orbit. From 1961 using English-language literature, we present the sixth such case and the first case with three malignant components. DESIGN: Interventional case report. methods: The clinical presentation, workup, surgical treatment, and pathology of a case of malignant mesenchymoma of the orbit are presented. RESULTS: Although very rare, these tumors should be in the differential diagnosis of any tumor of the orbit. The prognosis is unknown because of the lack of follow-up of reported patients. CONCLUSIONS: This malignant orbital mesenchymoma, an entity not accepted by all pathologists, was more complex than cases previously reported in the English literature in demonstrating rhabdomyosarcomatous, chondrosarcomatous, and osteogenic differentiation.
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keywords = rhabdomyosarcoma
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6/33. Primary osteochondrorhabdomyosarcoma (malignant mesenchymoma) of the fibula: a rare tumour in an unusual site -- case report and review of the literature.

    Malignant mesenchymoma, defined by Stout as sarcomas comprising two or more unrelated differentiated tissue elements other than a fibrosarcoma component, is rare. We report a case of primary malignant mesenchymoma of the proximal fibula in a 10-year-old female student who presented with pain and swelling of the right knee for 2 months. Initial biopsy showed features of rhabdomyosarcoma only, but the resected specimen revealed additional osteosarcomatous and chondrosarcomatous elements. The patient remained well more than 5 years after initial presentation. Including our present patient, 16 cases of primary malignant mesenchymoma of bone are found in the English literature, affecting mainly adolescents and young adults, with a slight male predominance and predilection for the metaphysis of long bones, especially around the knee. More than 60% of the patients develop metastasis, almost invariably to the lung, but occasionally to the brain. About 60% of the patients, all with metastasis, died mostly within one year of diagnosis. The clinical features of primary malignant mesenchymoma of bone thus resemble those of conventional osteosarcoma. Moreover, our case illustrates that, with combination chemotherapy targeted for individual elements, the prognosis of this rare tumour might be much improved, as in osteosarcoma.
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ranking = 2.5
keywords = rhabdomyosarcoma
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7/33. Malignant ectomesenchymoma in the wrist of a child: case report and review of the literature.

    Malignant ectomesenchymomas, rare and potentially aggressive tumors, occur in children and exhibit mesenchymal and neuroectodermal components. This report describes the first patient diagnosed with a malignant ectomesenchymoma of the hand. The patient was a 17-month-old male who developed a hypothenar mass on his left hand that was surgically excised. Microscopic evaluation revealed ganglioneuroblastic, rhabdomyosarcomatous, and chondrosarcomatous elements. Following excisional biopsy he was treated with cyclophosphamide, doxorubicin, vincristine, ifosfamide, and etoposide. After 3 courses of chemotherapy the patient had a wide reexcision with no residual tumor. The patient is 4 years from diagnosis, without evidence of disease.
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ranking = 0.5
keywords = rhabdomyosarcoma
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8/33. osteosarcoma with rhabdomyosarcomatous component or so-called malignant mesenchymoma of bone.

    BACKGROUND: Primary malignant mesenchymoma of the bone is a rare neoplasm consisting of two or more unrelated malignant mesenchymal components. The literature reports fewer than 20 cases, most of which were composed of osteosarcoma and liposarcoma. observation: We report an exceedingly rare case of primary malignant mesenchymoma of bone composed of rhabdomyosarcoma, osteosarcoma, and a minor chondrosarcoma component, arising in the right proximal humerus of a 15-year-old girl. The rhabdomyosarcomatous component was present in the initial biopsy and persisted in surgical specimen following chemotherapy. CONCLUSION: Effect of chemotherapy is enigmatic since rhabdomyosarcomatous component could appear, persist or disappear after chemotherapy according to literature.
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ranking = 3.5
keywords = rhabdomyosarcoma
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9/33. desmin positivity in primitive neuroectodermal tumors of childhood.

    In this report, we describe two rosette-forming primitive neuroectodermal tumors that were found to contain desmin by both immunohistochemistry and Western blotting. Electron microscopy on both cases was consistent with primitive neuroectodermal tumors and revealed that the tumor cells contained cytoplasmic bundles of intermediate filaments. In both cases, studies for MyoD1 protein using immunohistochemistry and Western blotting were negative. Thus, the detection of desmin in a pediatric neoplasm does not absolutely exclude the diagnosis of primitive neuroectodermal tumor and should not be considered as prima facie evidence that a small-cell tumor is a rhabdomyosarcoma.
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ranking = 0.5
keywords = rhabdomyosarcoma
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10/33. Malignant mesenchymoma of the brain. A case report.

    A case of malignant mesenchymoma of the brain, containing rhabdomyosarcomatous, osteoid and cartilaginous components is presented. The patient received surgery and irradiation for his tumor, but died 10 months after the operation. Previous reports of malignant mesenchymoma of the brain are exceptional. The neurobiological behavior of this tumor is yet to be determined.
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ranking = 0.5
keywords = rhabdomyosarcoma
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