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1/95. Endometroid adenocarcinoma of the cervix in a 9-year-old girl.

    We present the first reported case of endometroid adenocarcinoma of the uterine cervix in a young girl. The differential diagnosis of a vaginal mass in this age group is usually rhabdomyosarcoma, although other, rarer tumours also occur.
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ranking = 1
keywords = rhabdomyosarcoma
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2/95. Sarcoma botryoides of the cervix. Report of a case with cytopathologic findings.

    BACKGROUND: Cytologic findings of sarcoma botryoides were still equivocal because sarcoma botryoides of the uterine cervix is an extremely rare neoplasm, and few cases have been reported to date. CASE: A 17-year-old female was diagnosed with sarcoma botryoides of the uterine cervix. The entire vaginal canal was occupied with polypoid masses, which arose from the anterior lip of the uterine cervix, and the tumor was classified as group I (Intergroup rhabdomyosarcoma Study). After wedge resection and six courses of combination chemotherapy, the tumor recurred in the same location of the cervix as the primary lesion. touch smear of the polypoid mass formed loose clusters and also showed short spindle cells in a necrotic background. The nucleus of the tumor cells had a thin nuclear membrane, fine chromatin pattern and partly clear nucleolus, showing mild nuclear atypia. Immunohistochemically, some of the tumor cells showed positive staining for myoglobin and desmin. CONCLUSION: The cytologic findings of sarcoma botryoides of the female genital tract are typical features of nonepithelial malignant tumor. Immunohistochemical study is useful for the diagnosis of rhabdomyosarcoma.
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ranking = 1
keywords = rhabdomyosarcoma
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3/95. Clinical aspects of alveolar rhabdomyosarcoma with translocation t(1;13)(p36;q14) and hypotetraploidy.

    Although most cases of alveolar rhabdomyosarcoma (RMS) are characterized by the chromosomal translocation t(2;13)(q35;q14), several cases have been reported with a variant t(1;13)(p36;q14). We present the clinical, morphological and cytogenetic features of an alveolar RMS in a 4-year-old boy. Chromosomal analysis revealed a hypertriploid to hypotetraploid karyotype with a t(1;13)(p36;q14) in all tumor cells. It appears that alveolar RMS with t(1;13) occurs in younger children and displays a higher incidence to upper and lower extremity than tumors with t(2;13).
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ranking = 5.0495080197189
keywords = rhabdomyosarcoma, alveolar
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4/95. Implant brachytherapy: a novel treatment for recurrent orbital rhabdomyosarcoma.

    BACKGROUND: Orbital rhabdomyosarcoma is the most common primary malignancy of the orbit in childhood. Tumor resection and exenteration were the preferred treatment modalities for rhabdomyosarcoma. In the past 20 years, however, combined local radiation and systemic chemotherapy have shown excellent survival results. Tumor recurrence after any of the aforementioned therapies is almost always fatal. We have developed a novel treatment for recurrent disease that has resulted in long-term survival for three patients. methods: Three patients with recurrent orbital rhabdomyosarcoma were previously treated with primary radiation and chemotherapy. At the time of recurrence, exenteration and localized brachytherapy were performed. An individually molded poly(methylmethacrylate) (Lucite; E. I. du Pont de Nemours & Co., Wilmington, Del.) device loaded with radioactive iodine seeds delivered localized high-dose radiation, 6000 cGy over 6 days, to the orbit without irradiating the brain. RESULTS: All patients are alive and free of disease with follow-up ranging from 4 years and 4 months to 8 years and 4 months. CONCLUSION: A novel technique of delivering localized radiation to the orbit of three children with recurrent orbital rhabdomyosarcoma appears curative.
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ranking = 8
keywords = rhabdomyosarcoma
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5/95. So-called 'hybrid' lesion of desmoplastic and conventional ameloblastoma: report of a case and review of the literature.

    So-called 'hybrid' lesion of ameloblastoma, which is composed of desmoplastic ameloblastoma and conventional follicular/plexiform ameloblastoma, is an unusual variant of ameloblastoma and only eight cases of 'hybrid' lesion have been published in the English literature. To enhance knowledge of this interesting tumor, we add a case of 'hybrid' lesion that occurred in the right mandible of a 48-year-old Japanese male. Radiographic examination disclosed a honeycomb appearance at the anterior alveolar region, combined with a unicystic radiolucency in the molar region of the mandibular body. Histologically, the former showed microscopic features of desmoplastic ameloblastoma and the latter those of follicular ameloblastoma with focal granular cell transformation. The lesion was enucleated with curettage of surrounding bone and the lesional cavity was marsupialized. Although tumor tissues reappeared at 3, 5, 7 and 14 months after the surgery, the patient has remained disease free for 11 years after the last vaporization by CO2 laser of the recurred tumor. Many more cases of 'hybrid' lesion are needed to clarify the clinicopathological, histopathological and biological characteristics of this interesting variant of ameloblastoma.
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ranking = 0.0070725742455605
keywords = alveolar
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6/95. Re-irradiation for recurrent rhabdomyosarcoma of the nasopharynx in a child.

    There are few clinical situations where high dose re-irradiation with curative intent is indicated in children. Because of the potential for serious late sequelae, re-irradiation is considered only in the unusual situation where there is just local recurrence and surgery and/or chemotherapy are unlikely to provide long-term control. The authors present a case of nasopharynx rhabdomyosarcoma in a child which recurred after high dose irradiation and chemotherapy and was then re-irradiated to a high dose. The child is well without any serious sequelae more than 13 years following completion of the second course of radiotherapy.
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ranking = 5
keywords = rhabdomyosarcoma
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7/95. Alveolar rhabdomyosarcoma metastatic to the breast: long-term survivor.

    The case history is described of an adolescent girl with alveolar rhabdomyosarcoma of the maxillary sinus, who was treated with radical radiotherapy and adjuvant chemotherapy. She relapsed in the breast and, after incomplete excision, received radical radiotherapy resulting in long-term survival with breast conservation. The characteristics of patients with metastatic rhabdomyosarcoma with breast involvement are discussed. In adolescent girls, the breast is postulated to be a preferential metastatic site.
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ranking = 6.0070725742456
keywords = rhabdomyosarcoma, alveolar
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8/95. Alveolar soft part sarcoma of the tongue.

    We report a case of alveolar soft part sarcoma--a rare malignancy that presented as a swelling at the base of the tongue in a 5-year-old child. Only about one-quarter of the few reported cases arise within the head and neck, the tongue and orbit being the favoured sites.
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ranking = 0.0070725742455605
keywords = alveolar
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9/95. A malignant triton tumor with an unbalanced translocation (1;13)(q10;q10) and an isochromosome (8)(q10) as the sole karyotypic abnormalities.

    The karyotype of a malignant nerve sheath tumor with rhabdomyosarcomatous differentiation (malignant triton tumor) of a 58-year-old woman is reported. The tumor revealed an isochromosome for the long arm of chromosome 8 and an unbalanced translocation (1;13)(q10;q10) leading to a gain of the long arm of chromosome 1 as the sole karyotypic abnormalities.
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ranking = 1
keywords = rhabdomyosarcoma
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10/95. TCR-Vbeta repertoire analysis with RT-PCR was useful for the early detection of pulmonary relapsed T-cell lymphoma after autologous peripheral blood stem cell transplantation.

    Pulmonary recurrence of malignant lymphoma is a rare event after stem cell transplantation. We report here a 45-year-old male who was successfully diagnosed with relapsed pulmonary T-cell lymphoma using an RT-PCR method. Clonal expansion of T cells expressing identical TCR V-D-J junction size (Vbeta5-Jbeta1.5) was demonstrated in lymphocyte groups obtained from both bronchoalveolar lavage fluid at relapse, and paraffin embedded lymph node samples resected when he was first diagnosed with angioimmunoblastic T-cell lymphoma. This method provided evidence to diagnose relapsed pulmonary angioimmunoblastic T-cell lymphoma in its early phase.
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ranking = 0.0070725742455605
keywords = alveolar
(Clic here for more details about this article)
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