Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 27-year-old man was found to have a mediastinal tumour and the histological diagnosis was immature teratoma. Remission was achieved by chemotherapy and total resection. However, he developed anaemia and leukoerythroblastosis after 2 years of remission, and was referred to our hospital. Rhabdomyosarcoma cells were detected in the bone marrow and pleural effusion. Moreover, karyotype analysis of peripheral blood and bone marrow cells revealed mosaic-type Klinefelter syndrome. We diagnosed the case as transformation of teratoma into rhabdomyosarcoma in Klinefelter syndrome. Although intensive chemotherapy was performed, the patient died with meningeal infiltration.
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PMID:Rhabdomyosarcoma in a patient with mosaic Klinefelter syndrome and transformation of immature teratoma. 1059 33

This retrospective study was undertaken to determine the prevalence, clinical characteristics, management methods and prognosis of testicular cancer at Kenyatta National Hospital, Nairobi. All histologically confirmed testicular cancer patients recorded at the Histopathology Department between 1993 and 1997 were analyzed. The mean age was 34.8 years with a peak incidence in the 30-44 year age group. About 10.26% of patients had history of cryptochirdism. The clinical symptoms presented were painless testicular swelling (n = 31, 79.49%), testicular pain (n = 11, 28.08%), scrotal heaviness (n = 9, 23.08%), abdominal swelling (n = 6, 15.38%), gynecomastia (n = 1, 2.56%), and eye swelling (n = 1, 2.56%). On examination, 32 patients (82.05%) had testicular masses, 10 (25.64%) had abdominal masses, 7 (17.91%) had supraclavicular and cervical lymphadenopathy, 1 had gynecomastia, and 1 had an orbital mass. More than 89% of patients had germ cell cancers with seminoma accounting for 67.35%, teratoma for 12.24%, embryonal carcinoma for 8.16%, rhabdomyosarcoma for 6.12%, and malignant germ cell tumor, orchioblastoma, and dysgerminoma each accounting for 2.04%. The various methods of treatment include orchidectomy and radiotherapy and chemotherapy in 3 patients (7.7%), orchidectomy and radiotherapy in 16 patients (41.03%), orchidectomy and chemotherapy in 6 patients (15.38%), and radiotherapy and chemotherapy in 10 patients (25.64%). No cisplatin-based chemotherapy was used. 18 patients were followed up, of whom 7 were alive after 5 years. Prognosis with current regimens was poor, with a 38.89% survival ratio in 5 years. Hence, cisplatin-based chemotherapy with up to 90% cure rates should be included in the testicular cancer management in this hospital.
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PMID:Testicular cancer at Kenyatta National Hospital, Nairobi. 1077 80

A case of ovarian immature teratoma with rhabdomyosarcomatous recurrence in a 6-year-old girl is described. The primary tumor consisted of a dermoid cyst and a solid nodule composed of mature and immature mesenchymal tissue. The most immature mesenchymal cells showed no distinctive differentiation except for focal rhabdomyoblastic differentiation. The primary tumor was diagnosed as immature teratoma, grade 2, stage IIa. Despite left oophorectomy and excision of uterine serosal implants, chemotherapy, and radiation, four intrapelvic recurrences developed within 3 years. Although the primary tumor contained only a few rhabdomyoblasts, the recurrent tumors became increasingly rhabdomyosarcomatous. The patient died of systemic disease 3 years after presentation. This case and previous reports indicate that the prognosis of patients with ovarian immature teratoma with rhabdomyosarcomatous recurrence is poor and similar to that of primary ovarian rhabdomyosarcoma.
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PMID:Immature teratoma of the ovary with a minor rhabdomyosarcomatous component and fatal rhabdomyosarcomatous metastases: the first case in a child. 1178 30

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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PMID:Testicular germ cell tumor with rhabdomyosarcoma successfully treated by disease-adapted chemotherapy including high-dose chemotherapy: case report and review of the literature. 1178 32

We report a case of rhabdomyosarcoma which occurred in a mediastinal teratoma in a 44-year-old man. Presentation symptoms were chest pain, hoarseness and a cough. Diagnosis was fortuitous, performed by the histological and immunohistochemical study of a mediastinal tumour biopsy specimen that showed embryonal carcinoma and yolk sac tumour components associated with the rhabdomyosarcoma. After cisplatin-based chemotherapy (bleomycin-etoposide-cisplatin), surgical resection of the residual mediastinal tumour was performed. Histological and immunohistochemical study of this tumour confirmed the presence of mature teratoma and embryonal rhabdomyosarcoma. Evolution was marked by a local extension of the mediastinal tumour, occurrence of multiple metastases and bone marrow involvement. The patient died 8 months after diagnosis despite chemotherapy and radiotherapy. A review of the literature reveals that the development of rhabdomyosarcoma in primary mediastinal teratomas is unusual in adults. The diagnostic, therapeutic and prognostic implications of such an association are reviewed.
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PMID:Rhabdomyosarcoma arising in mediastinal teratoma in an adult man: a case report. 1188 12

From 1972 to 2000, 123 patients with solid tumors whose complaints had started in the first 28 days of life were retrospectively evaluated. Fifty-five patients were diagnosed in the first 28 days and 68 patients were diagnosed after 28 days. In the former group, 85.5% of patients had symptoms in the first day of life. In the latter group, 77.9% had the onset of symptoms in the first day. Tumor subgroups in the neonatal period included teratoma (34), neuroblastoma (11), rhabdomyosarcoma (3), Wilms tumor (1), and retinoblastoma (3), and the others (3). Three patients had other, less common tumors. In the second group the numbers were the following: for teratoma (32), neuroblastoma (15), germ cell tumors other than teratomas (8), rhabdomyosarcomas (4), the other soft tissue sarcomas (3), Wilms tumor (1), retinoblastoma (1), and other, rare tumors (4). There were 22 malignant tumors in the first group, and 44 in the second group. Fourteen patients in the first group died in the early postoperative period or with progressive disease. Nineteen of 44 patients died in the second group. Overall survival rates were 24.9% and 51.6% in first and second groups, respectively (p = 0.015). Event-free survival rates were 14.7% and 47.7% in these groups, respectively (p = 0.0063). This is the first report comparing clinical features and prognosis of tumors diagnosed in the first 28 days of the life with those diagnosed after 28 days. The prognosis was worse in infants diagnosed in the first 28 days of life.
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PMID:Solid tumors in the neonatal period. 1263 79

Eight separate neoplasms with five distinct morphologies are described in the present report. The spontaneous neoplasms were identified in farmed chinook salmon, Oncorhynchus tshawytscha (Walbaum), during processing. The masses were examined histologically and were classified as teratoma, rhabdosarcoma, biliary and renal cystadenomas, renal carcinoma and three leiomyomas. This represents one neoplasm detected for every 125000 fish processed during the sampling period.
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PMID:Sporadic neoplasms of farmed chinook salmon, Oncorhynchus tshawytscha (Walbaum), from New Zealand. 1294 8

A 23-year-old male was admitted to our hospital for the management of pulmonary metastases. He had undergone right high orchiectomy, chemotherapy with four courses of PEB regimen (cisplatin, etoposide, bleomycin) and retroperitoneal lymph node dissection the previous year. The pathological findings showed mixed germ cell tumor (seminoma, yolk sac tumor, embryonal carcinoma) in the testis and mature teratoma in the draining lymph node. Two courses of salvage chemotherapy using a VIP regimen (etoposide, ifosfamide, cisplatin) were performed after diagnosis of pulmonary metastases, but had no affect on tumor size. Video-assisted excision of pulmonary metastases was then performed, giving a pathological diagnosis of rhabdomyosarcoma in all three resected tumors. The operation was followed by three courses of CYVADIC (cyclophosphamide, vincristine, adriamycin, dacarbazin) chemotherapy and oral cyclophosphamide, as a small residual tumor was suspected. These chemotherapeutic interventions have appeared effective, with no apparent recurrence of lesions at present, one year after the excision of pulmonary metastases.
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PMID:[Pulmonary rhabdomyosarcoma generated during treatment of testicular tumor]. 1467 2

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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PMID:Nephroblastoma arising in a germ cell tumor of testicular origin. 1510 60

Most pediatric thoracic malignancy is pulmonary disease secondary to solid tumors of childhood. The management of isolated pulmonary metastases in adulthood is well documented. Little has been published to document the long-term outcome of pulmonary metastasectomy in childhood. A retrospective study was undertaken to assess the results of surgery for isolated pulmonary metastases. Twenty children underwent surgery over 12 years (mean follow-up 8 years). Five had Wilms' tumor (mean age 51 months), eight had osteogenic sarcoma (mean age 141 months), three had rhabdomyosarcoma (mean age 92 months), two had hepatoblastoma (mean age 30 months) and two had teratoma (mean age 72 months). Four had bilateral synchronous metastases and thoracotomies, and one had bilateral metachronous metastases and thoracotomies. Nineteen children were discharged well within 10 days of surgery. There was one early complication: a death due to pneumonia. Four children subsequently died postoperatively with cranial metastases (mean 29 months postoperatively). The remaining 16 children remain alive and well. As part of the combined therapy, these results would support an aggressive surgical approach to this disease. Preoperative assessment should include contrast enhanced computed tomogram of the head and chest as well as chest X-ray taken immediately preoperatively to exclude metastases. Bilateral synchronous and metachronous thoracotomy is well tolerated in childhood.
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PMID:Pulmonary metastasectomy for pediatric solid tumors. 1525 16


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