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)

Six cases of testicular tumors in children are presented: 3 patients had teratoma, 1 embryonal carcinoma, 1 orchioblastoma, and 1 paratesticular rhabdomyosarcoma. Three of the 6 patients presented with hydroceles. The treatment consisted of orchiectomy alone. All patients were alive and free of disease one and one-half to eight and one-half years after orchiectomy. It is suggested that orchiectomy alone is curative in most children with testicular tumors under the age of two years.
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PMID:Testicular neoplasms in children. 88 61

The production and detailed immunostaining properties of a new rat monoclonal antibody (ICR.2) to epithelial membrane antigen are reported. The antibody was selected for its ability to compete with the polyclonal antiserum (M7), used in the original immunohistological studies, in order that it might serve as a direct replacement in diagnosing epithelial tumours. Most of the staining reactions on normal tissues were identical to those previously reported with M7 but there were some important differences. They included: positivity of renal and adrenal capsular fibroblasts, perineurium, some myoepithelial and smooth muscle cells, occasional osteoblasts and squamous and thyroid follicular epithelium in the normal state. The intercellular canaliculi of sweat glands and secretory canaliculi of gastric oxyntic cells were clearly demonstrated. These staining reactions could be obtained with M7 when a sensitive detection system was used although the results were usually weak and inconsistent. Nearly all adenosquamous and transitional carcinomas were positive. The remaining tumours fell into three major groups: (1) those which were consistently or nearly consistently negative--melanoma, seminoma, rhabdomyosarcoma, alveolar soft part sarcoma, adrenal cortical carcinoma, granulocytic sarcoma, paraganglioma, non-Hodgkin's lymphoma. Hodgkin's disease and embryonal carcinoma: (2) those which were either negative or positive with distinctive patterns of staining--basal cell carcinoma, embryonal tumours: and (3) non-epithelial tumours that were consistently positive--epithelioid sarcoma, synovial sarcoma, osteosarcoma, chordoma and myeloma--or positive in a significant minority of cases--leiomyosarcoma, malignant fibrous histiocytoma, clear cell sarcoma of tendon sheath, various neuroectodermal tumours.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detailed investigation of the diagnostic value in tumour histopathology of ICR.2, a new monoclonal antibody to epithelial membrane antigen. 169 88

The metabolism of arachidonic acid was investigated by radioimmunoassay and chromatographic techniques in 5 sarcomas and one embryonal carcinoma of human origin maintained as transplantable tumors in nude mice. The results obtained indicate that: the absolute quantities of arachidonic acid metabolites produced by a given tumor varied between experiments but the overall distribution pattern of these products, in general, remained constant from passage to passage; each tumor showed a different arachidonic acid metabolite profile in quality and quantity; 2 sarcomas of the same histological type could be clearly distinguished by their arachidonic acid metabolites; the predominant product in all tumors was 12-HETE or 15-HETE, whereas thromboxane A2 was synthesized in low quantities by all tumors; PGF2 alpha was synthesized at the highest rate by an alveolar rhabdomyosarcoma; PGE2 synthesis was highest in a malignant fibrous histiocytoma; and total prostaglandin synthesis was low in the chondrosarcoma and synovial-cell sarcomas. All results reported in this study are for the complete tumor which includes both neoplastic and stromal cells. The role that these products play in the biological behavior of mesenchymal tumor cells and normal tissues of the host remains to be determined.
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PMID:Prostaglandin and hydroxyeicosatetraenoic acid synthesis by human mesenchymal tumors. 299 Nov 46

A case of testicular teratoma metastasized to the retroperitoneum and after cytoreductive chemotherapy was noted to contain areas of frank sarcoma. Sarcomatous areas included embryonal rhabdomyosarcoma with a pattern of sarcoma botyroides, alveolar rhabdomyosarcoma, and fibrosarcoma. These areas differed markedly from areas of immature teratoma, which composed the remainder of the retroperitoneal lesion and which also characterized the primary tumor. These sarcomatous areas were characterized by numerous mitoses, marked cellular pleomorphism and diagnostic histologic, ultrastructural, and immunocytochemical features. Residual germ cell tumors following cytoreductive chemotherapy are traditionally categorized as teratoma only or teratoma with embryonal carcinoma or choriocarcinoma for therapeutic and prognostic reasons. This case does not conform to this simple categorization and raises serious questions concerning subsequent therapeutic decisions.
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PMID:Sarcoma arising in a residual testicular teratoma after cytoreductive chemotherapy. 647 36

A total of 114 children with solid tumors refractory to conventional therapy were evaluated for response and/or toxic effects after receiving cisplatin at doses of 3.0-4.5 mg/kg with aggressive hydration and mannitol diuresis every 3 weeks; a minimum of two courses was required for evaluation of response (110 patients). Objective responses were noted in 18 patients: rhabdomyosarcoma (three), Wilm's tumor (three), osteogenic sarcoma (three). Ewing's sarcoma (two), neuroblastoma (one), undifferentiated sarcoma (one), hepatoblastoma (one), ovarian teratoma (one), hepatocellular carcinoma (one), embryonal carcinoma of the mediastinum (one), and thymoma (one). Twenty-six patients had some evidence of renal toxicity. Asymptomatic hearing loss was commonly found when audiometry was performed (eight of 18 patients tested). Eight additional patients had symptomatic hearing problems--tinnitus or hearing loss. Myelosuppression was mild. Hypomagnesemia and/or hypocalcemia were common but only one patient had symptoms. Cisplatin, administered at a dose of 3.0 mg/kg with aggressive hydration and mannitol diuresis, is reasonably well-tolerated. Its role in the therapy for those tumors against which it shows activity remains to be determined.
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PMID:Phase II trail cisplatin in refractory childhood cancer: Children's Cancer Study Group Report. 694 56

This is a report of prospective study of eight patients with testicular tumours seen at the Urology Unit of the Lagos University Teaching Hospital over a five-year period (1979-1983). The mean age was 32.7 years. Four patients (50%) had germ cell tumours including embryonal carcinoma 25%, seminoma 12.5% and malignant teratoma undifferentiated (MTU) 12.5%. The seminoma in this group originated from a testis which was previously undescended but brought into the scrotum at six years of age. The other four patients (50%), had non germ cell tumours. Two of these patients (25%) had paratesticular tumours including rhabdomyosarcoma of paratesticular adnexae and liposarcoma. One (12.5%) had adenomatoid tumour of the epididymis while the last patient (12.5%) had malignant fibrous mesothelioma of the tunica vaginalis. This study reaffirms the fact that testicular tumours are rare in blacks and that Nigeria has the lowest incidence reported at 0.1 per 100,000 per annum.
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PMID:Testicular cancer in Nigerians. 749 39

The 14 tumors reported in Rubinstein-Taybi syndrome since 1989, when added to the 22 previously reported, are beginning to show a pattern of neural and developmental tumors, especially of the head, which is malformed in the syndrome. Among the neoplasms were 12 of the nervous system: 2 each of oligodendroglioma, medulloblastoma, neuroblastoma, and benign meningioma, a pheochromocytoma, and 3 other benign tumors; 2 of nasopharyngeal rhabdomyosarcoma; and 1 each of leiomyosarcoma, seminoma, and embryonal carcinoma. Among the other benign tumors were an odontoma, a choristoma, a dermoid cyst, and 2 pilomatrixomas.
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PMID:Tumors in Rubinstein-Taybi syndrome. 955 2

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

We investigated 115 testicular and 3 epididymal tumors and 6 cases of the complete androgen insensitivity syndrome (AIS) for the expression of inhibin-alpha, CD99, HEA125, PLAP, and chromogranin, using monoclonal antibodies and standard immunhistochemical techniques. Ihibin-alpha was detected in the neoplastic cells in 27 of 27 primary Leydig cell tumors (LCTs), 1 of 1 metastatic LCT, 6 of 20 Sertoli cell tumors (SCTs), 4 of 5 juvenile granulosa cell tumors (GCTs), and 2 of 5 unclassified sex cord-stromal tumors (USCSTs). Except for 2 choriocarcinomas, the choriocarcinomatous component of 1 mixed germ cell tumor, and a small focus of inhibin-positive syncytiotrophoblast in 1 embryonal carcinoma, inhibin-a immunoreactivity was not present in the neoplastic cells of the 38 remaining testicular germ cell tumors; 11 B-cell and 1 T-cell lymphomas; 1 granulocytic sarcoma; and 1 rhabdomyosarcoma of the testis; 1 adenoma of the rete testis, and 3 adenomatoid tumors of the epididymis. Inhibin-alpha immunoreactivity was present in the Sertoli cells and Leydig cells in 5 testicular hamartomas and in 1 Sertoli cell adenoma in 6 cases of AIS; both Sertoli and Leydig cells were also positive in the extranodular testicular parenchyma present in 2 of these cases. CD99 was detected in 10 of 15 primary LCTs, 1 of 7 SCTs, 3 of 5 JGCTs, and in 1 of 5 USCSTs but was not found in any tumor outside the sex cord-stromal category. HEA125 immunostaining was not detected in sex cord-stromal tumors; however, 3 of 12 seminomas, 3 of 12 embryonal carcinomas, 6 of 8 yolk sac tumors, and 1 of 2 teratomas were HEA125 positive. PLAP was not detected in sex cord-stromal tumors except for 4 of 15 primary LCTs but was present in most germ cell tumors. Chromogranin immunostaining was present in the sex cord-like element in 1 of 5 USCSTs, 1 of 8 YSTs, 1 of 2 teratomas, and in 1 of 1 rete adenoma, and in normal adjacent rete testis. In conclusion, although inhibin-alpha and PLAP, and, to a somewhat lesser extent, CD99 and HEA125 immunostaining are helpful in the differential diagnosis of certain testicular neoplasms that are difficult to distinguish on morphologic grounds, chromogranin is far less helpful in this context.
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PMID:Inhibin-alpha CD99, HEA125, PLAP, and chromogranin immunoreactivity in testicular neoplasms and the androgen insensitivity syndrome. 1101 71

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


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