Cases reported "Testicular Neoplasms"

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1/54. 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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ranking = 1
keywords = rhabdomyosarcoma
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2/54. Paratesticular rhabdomyosarcoma in a neonate.

    A 13-day-old boy presented with left scrotal tumor and coronary hypospadias. Left radical orchiectomy was performed. Histological diagnosis was embryonal-type paratesticular rhabdomyosarcoma. To the authors' knowledge, this is the first reported case of paratesticular rhabdomyosarcoma in a neonate in English-language literature. Also, the association of a testicular tumor with hypospadias has not been noted.
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ranking = 3
keywords = rhabdomyosarcoma
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3/54. Case report of embryonal rhabdomyosarcoma of the testis with an 8-year survival.

    The low incidence of embryonal rhabdomyosarcoma prevents any one person or institution from presenting any meaningful data in the form of effective treatment based on clinical experience. We realize that this is an antidotal case but the stakes are high when treating any malignancy of the young. Therefore, we advocate early and aggressive therapy using all modalities of treatments, and cite this case which initially seemed hopeless but to date has all the hallmarks of a cure.
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ranking = 2.5
keywords = rhabdomyosarcoma
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4/54. Testicular germ cell tumor with rhabdomyosarcoma successfully treated by disease-adapted chemotherapy including high-dose chemotherapy: case report and review of the literature.

    Treatment and prognosis have not been well characterized in germ cell tumors (GCT) with a malignant nongerm cell component. patients with a mediastinal tumor, neural or rhabdomyosarcomatous differentiation and distant metastases have the poorest prognosis. We report a rare case of mixed GCT composed of seminoma, teratoma and rhabdomyosarcoma with the rhabdomyosarcomatous component metastasized into the liver and bone marrow (BM) causing hypercalcemia. The patient was treated with differentiation-tailored chemotherapy (CHT) including a disease-adapted high-dose (HD) CHT regimen with purified autologous PBSCT (APBSCT) and pamidronate. To date, remission has lasted for 4 years. Tumor-adapted CHT including HD-CHT with APBSCT can induce long term remissions in high-risk patients with transformed GCT. A review of the literature is given.
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ranking = 3.5
keywords = rhabdomyosarcoma
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5/54. Mixed tumor of paratesticular rhabdomyosarcoma and an adenomatoid tumor in an elderly patient.

    We report a case of a mixed tumor of paratesticular rhabdomyosarcoma and an adenomatoid tumor in an elderly patient. rhabdomyosarcoma is one of the most common childhood tumors; however, it rarely occurs in the elderly. In addition, to our knowledge, paratesticular rhabdomyosarcoma and adenomatoid tumor rarely form mixed tumors. Finally, we consider the pathogenesis of diploblastic tumors.
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ranking = 3
keywords = rhabdomyosarcoma
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6/54. Metastatic testicular rhabdomyosarcoma--a report of two cases.

    rhabdomyosarcoma is the most common type of soft tissue sarcoma in children. The tumor spreads by local extension, to regional lymph nodes, or by distant metastases. Metastatic spread to the testicle has been rarely described. The authors describe 2 boys who were found to have intratesticular metastases after presenting with primary tumors in their extremities. The first patient, an 11-year-old boy presented with primary disease in his left foot and an enlarged testicle. rhabdomyosarcoma was found histologically in both the foot and the testicle. A second boy 17 years of age had a primary tumor involving the left upper extremity treated with amputation, chemotherapy, and radiotherapy. A relapse was noted 2 years later in the left testicle and was treated with orchiectomy. The authors discuss the implications and the management of this rare presentation of metastatic rhabdomyosarcoma.
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ranking = 2.5
keywords = rhabdomyosarcoma
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7/54. Melanotic neuroectodermal tumor of infancy: an important mimicker of paratesticular rhabdomyosarcoma.

    We report a case of a paratesticular tumor in a 6-month-old infant. This tumor was originally believed to represent a poorly differentiated sarcoma of the cord but upon further pathological consultation it was recognized as a rare melanotic neuroectodermal tumor of infancy involving the epididymis. Since the former is a highly aggressive lesion and the latter an indolent tumor, treatment of these 2 entities differs markedly. Therefore, although the histological distinction between these lesions is often difficult, it is of critical importance.
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ranking = 2
keywords = rhabdomyosarcoma
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8/54. Paratesticular rhabdomyosarcoma in a 2-year-old boy.

    Paratesticular rhabdomyosarcoma is a rare but highly malign tumor. We report a case of paratesticular rhabdomyosarcoma and discuss the management of this neoplasm in the light of the literature.
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ranking = 3
keywords = rhabdomyosarcoma
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9/54. Nephroblastoma arising in a germ cell tumor of testicular origin.

    We report a nephroblastoma arising in a germ cell tumor of testicular origin occurring in a 22-year-old man. orchiectomy demonstrated a malignant mixed germ cell tumor composed of mature and immature teratoma with nephroblastoma and rhabdomyosarcoma. Following chemotherapy, the patient developed supraclavicular and retroperitoneal lymphadenopathy. Excision demonstrated metastatic teratoma at both sites. No recurrence was noted with 21 months of additional follow-up. Using tissue microdissection and loss of heterozygosity analysis, we investigated the clonality of the mature teratoma, immature teratoma, nephroblastoma, and rhabdomyosarcoma components of the primary tumor and of the metastatic mature teratoma at the two separate distant sites. Nine microsatellite polymorphic makers were used to examine the pattern of allelic loss in both primary and metastatic tumors. loss of heterozygosity was found in 4 dna loci, and the same pattern of allelic loss was demonstrated at all 4 loci in all of the different components of the primary tumor and the metastatic mature teratomas, supporting the germ cell tumor origin of the nephroblastoma component. loss of heterozygosity on chromosome 17p13 (TP53) was detected in metastatic mature teratoma, but not in the primary tumor. loss of heterozygosity was observed at 11p13, the locus of WT1 inactivation in patients genetically predisposed to nephroblastoma, and this loss may be an important genetic mechanism in nephroblastomatous differentiation of germ cell tumors. These data support a common clonal origin for nephroblastoma and the other germ cell tumor components.
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ranking = 1
keywords = rhabdomyosarcoma
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10/54. Concurrent cystic mediastinal lymphangioma and paratesticular rhabdomyosarcoma.

    The current report describes a 23-month-old boy with paratesticular rhabdomyosarcoma who was incidentally diagnosed as also having an isolated mediastinal cystic lymphangioma. The association of childhood rhabdomyosarcoma with various congenital anomalies and genetic alterations such as p53 mutations have been well known. However, mediastinal cystic lymphangioma has not been reported among the congenital anomalies diagnosed in rhabdomyosarcoma. Both rhabdomyosarcoma and lymphangioma originate from mesenchymal cells. This association may be coincidental or may point to a common genetic and/or developmental disorder of the mesencymal tissue.
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ranking = 4
keywords = rhabdomyosarcoma
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