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)

Radio-labelled antimyosin-monoclonal antibodies (AMA) have been introduced to demonstrate myocardial necrosis after cardiac infarction or in cardiac allograft transplants. As rhabdomyosarcoma (RMS) and leiomyosarcoma (LMS) are tumors of myogenic origin, thus often containing myosin, we decided to use the 111In-labelled Fab fragment of AMA (Centocor) in scintigraphic tumor detection. We examined 13 children with histologically-confirmed RMS and LMS, and five other children with other types of soft tissue sarcomas. Conventional techniques were used to determine the extent of the tumor. An uptake of the tracer was observed in all known tumor sites in seven RMS patients. As the scans were negative in three RMS patients who were in complete remission (CR) and in two other patients (fibrosarcoma and haemangiopericytoma) with a measurable tumor mass, we considered them to be "true negative". In the three remaining CR patients (1 RMS, 2 LMS) the scans were positive but weak in the primary tumor site in two cases and in a distant site (bone) in the third respectively. We considered them to be "false positive" as no tumor cells were evident in the biopsy specimen. In one case, the antimyosin uptake was presumably the result of damage to the muscles after radiation. Interestingly, in three patients with other malignancies such as rhabdoid and peripheral neuroectodermal tumors there was a noticeably strong uptake of the tracer in the primary tumors and the scans turned negative after complete remission was achieved. The diagnostic AMA scanning showed no side-effects. The reason why antimyosin antibodies permeate the membrane of the tumor cells is yet undetermined.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Radio-immunodetection of myosarcoma using 111indium antimyosin. 216

One hundred and forty adult patients with advanced sarcomas (125 soft tissue and 15 bone) were treated with a combination chemotherapy regimen consisting of cyclophosphamide, vincristine, Adriamycin, and DTIC (CYVADIC). There were 21 (15%) complete and 45 (32%) partial responders, with an overall response rate of 47%. The response rate was 50% (17% complete) for patients with soft tissue sarcomas compared with 20% (none complete) for patients with bone sarcomas. The median duration of response was 9.5 months (range, 1-40+ months) for complete responders and 7 months (range, 1-31 months) for partial responders. The median time to achieve response was 9 weeks and the median number of courses of therapy to response was three. The median survival time was 16 months for responders compared with 7 months for nonresponders (P = 0.001). The most responsive tumor types were neurofibrosarcoma, rhabdomyosarcoma, leiomyosarcoma, fibrosarcoma, and angiosarcoma. Pulmonary and soft tissue metastases were more responsive than bone and liver metastases. CYVADIC toxicity was predominantly limited to myelosuppression, vomiting, fever of unknown origin, and neuropathy. CYVADIC is an effective combination chemotherapy regimen in the treatment of advanced sarcomas.
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PMID:Cyclophosphamide, vincristine, adriamycin, and DTIC (CYVADIC) combination chemotherapy for the treatment of advanced sarcomas. 737 60

Nestin is an intermediate filament that was first identified in neuroepithelial stem cells. During embryogenesis, nestin is expressed in a number of cell types, including neural crest cells and developing myocytes. We have recently shown that nestin is expressed in human podocytes and nephrogenic blastema. We sought to determine the utility of nestin expression in distinguishing pediatric tumors in the region of the kidney. Cases studied included Wilms tumor (n=24), nephroblastomatosis (n=6), renal cell carcinoma (n=19), renal clear cell sarcoma (n=9), mesoblastic nephroma (n=9), neuroblastoma (n=11), malignant rhabdoid tumor (n=8 including 2 renal), Ewing sarcoma (n=16 including 1 renal, 7 soft tissue, and 8 bone), intra-abdominal desmoplastic small round cell tumor (n=5), and rhabdomyosarcoma (n=8, all extrarenal). Nestin expression was assessed semiquantitatively by immunohistochemistry and then scored as positive or negative. All cases of Wilms tumor, mesoblastic nephroma, rhabdomyosarcoma, neuroblastoma, malignant rhabdoid tumor, and desmoplastic small round cell tumor were nestin-positive. In Wilms tumor and nephroblastomatosis, nestin was expressed in blastema and glomeruloid structures, but not tubules. In neuroblastoma, positive staining was detected regardless of degree of differentiation. The majority of Ewing sarcoma and renal cell carcinoma were negative. Expression in clear cell sarcoma was variable with 5 cases negative and 4 cases positive. Thus, nestin is a highly sensitive, but nonspecific, marker of Wilms tumor in the context of tumors that may occur in or around the kidney. Nestin reactivity may be useful in differentiating Wilms tumor from Ewing sarcoma, renal cell carcinoma, or nestin-negative clear cell sarcoma.
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PMID:Diagnostic utility of nestin expression in pediatric tumors in the region of the kidney. 1941 21