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

Immunotherapy using a Wilms tumor (WT1) peptide has been undergoing clinical trials for adulthood leukemia and solid cancer with promising results. In this study, the authors used WT1 peptide vaccination to treat a 6-year-old girl with metastatic alveolar rhabdomyosarcoma. She received weekly intradermal injection with HLA-A*2404-restricted, 9-mer WT1 peptide against residual bone disease. After 3 months her bone disease disappeared, concurrent with an increase in the frequency of WT1-specific cytotoxic T lymphocytes (CTLs). A high proportion of WT1-specific CTLs with effector or effector memory phenotype were detected in peripheral blood of this patient. She is currently still on continued WT1 peptide immunotherapy in a disease-free condition for 22 months. WT1 peptide-based immunotherapy should be a promising option for high-risk rhabdomyosarcoma in childhood.
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PMID:WT1 (Wilms tumor 1) peptide immunotherapy for childhood rhabdomyosarcoma: a case report. 1920 12

Wilms' tumor protein (WT1) has been immunohistochemically detected in the cytoplasm of some developing, adult normal and neoplastic human tissues, suggesting its complex regulator activity in transcriptional/translational processes. Among neoplastic tissues, WT1 has been documented in the cytoplasm of benign and malignant vascular tumors and in rhabdomyosarcoma, while there are no available studies about its expression in myofibroblastic tumors. Accordingly, we studied immunohistochemically the potential expression of WT1 in mammary myofibroblastoma (MFB), a prototypical myofibroblastic tumor. A series of 18 cases of mammary MFB, including several morphological variants (classic, fibrotic, myxoid, lipomatous, Schwannian-like, and epithelioid variants), were tested with antibodies against the N-terminal of WT1. The most striking finding was a diffuse and strong WT1 cytoplasmic immunostaining restricted to the "epithelioid cell MFB", a rare and diagnostically challenging variant. Conversely the other variants of MFB, including the classic-type, were negative or only focally positive. The present study shows that mammary epithelioid cell MFB should be added to the list of mesenchymal tumors which express WT1 in the cytoplasm of neoplastic cells. Accordingly, we suggest that the detection of WT1 cytoplasmic immunoreactivity is of complementary diagnostic value to conventional myofibroblastic markers in identifying epithelioid cell myofibroblastoma.
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PMID:Wilms' tumor protein (WT1) in mammary myofibroblastoma: an immunohistochemical study. 2470 16