Sirolimus therapy for fibromatosis and multifocal renal cell carcinoma in a child with tuberous sclerosis complex
A male with tuberous sclerosis complex (TSC) developed a chest wall fibromatosis and bilateral multifocal renal cell carcinoma (RCC). The fibromatosis tumor was initially resected during infancy but recurred 5 years later. At that time, bilateral RCC was also detected, leading to the resection of th...
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| Veröffentlicht in: | Pediatric blood & cancer 2010-07, Vol.54 (7), p.1035-1037 |
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| container_issue | 7 |
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| container_title | Pediatric blood & cancer |
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| creator | Pressey, Joseph G. Wright, Jordan M. Geller, James I. Joseph, David B. Pressey, Christine S. Kelly, David R. |
| description | A male with tuberous sclerosis complex (TSC) developed a chest wall fibromatosis and bilateral multifocal renal cell carcinoma (RCC). The fibromatosis tumor was initially resected during infancy but recurred 5 years later. At that time, bilateral RCC was also detected, leading to the resection of the more extensively affected right kidney. In an attempt to avoid bilateral nephrectomies, the patient was treated with the mTOR inhibitor sirolimus. Within 6 months of therapy, the fibromatosis and remaining RCC tumors responded substantially with minimal adverse effects. Pediatr Blood Cancer 2010;54:1035–1037 © 2010 Wiley‐Liss, Inc. |
| doi_str_mv | 10.1002/pbc.22401 |
| format | Article |
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The fibromatosis tumor was initially resected during infancy but recurred 5 years later. At that time, bilateral RCC was also detected, leading to the resection of the more extensively affected right kidney. In an attempt to avoid bilateral nephrectomies, the patient was treated with the mTOR inhibitor sirolimus. Within 6 months of therapy, the fibromatosis and remaining RCC tumors responded substantially with minimal adverse effects. 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Pediatr Blood Cancer 2010;54:1035–1037 © 2010 Wiley‐Liss, Inc.</description><subject>Antibiotics, Antineoplastic - therapeutic use</subject><subject>cancer genetics</subject><subject>Carcinoma, Renal Cell - drug therapy</subject><subject>Carcinoma, Renal Cell - etiology</subject><subject>Carcinoma, Renal Cell - pathology</subject><subject>Child</subject><subject>developmental therapeutics</subject><subject>Fibroma - congenital</subject><subject>Fibroma - drug therapy</subject><subject>Fibroma - pathology</subject><subject>Humans</subject><subject>Kidney Neoplasms - drug therapy</subject><subject>Kidney Neoplasms - etiology</subject><subject>Kidney Neoplasms - pathology</subject><subject>Loss of Heterozygosity</subject><subject>Male</subject><subject>pathology</subject><subject>renal cell carcinoma</subject><subject>Sirolimus - therapeutic use</subject><subject>soft tissue sarcoma</subject><subject>Thoracic Wall - pathology</subject><subject>Tuberous Sclerosis - complications</subject><subject>Tuberous Sclerosis - genetics</subject><subject>Tumor Suppressor Proteins - genetics</subject><issn>1545-5009</issn><issn>1545-5017</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2010</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kM1O3DAUha0KVChl0ReovENdZPBvnCzbEYVKI0CCiqXlONcat84PdqJh3r6eDsyOzfVdfPfT8UHoCyULSgi7HBu7YEwQ-gGdUilkIQlVR4ed1CfoU0p_MloSWX1EJ4xQUnHBT9Hzg49D8N2c8LSGaMYtdkPEzjdx6Mw0JJ-w6VvczWHybrAm4Ah9nhZCHiZa32cQ-x4bbNc-tHjjpzWe5gbikK3JhrzsNHboxgAvn9GxMyHB-et7hn7_vHpc3hSru-tfy--rwvKS0YLaBqSpqWLWtdJxZqhSJRDpnDLCguVNZVpKG1dBnf_LbG0EcK4aUValEfwMXey9YxyeZ0iT7nzapTY95GBacS5FxajM5Lc9aXPQFMHpMfrOxK2mRO8K1rlg_b_gzH59tc5NB-2BfGs0A5d7YOMDbN836fsfyzdlsb_waYKXw4WJf3WpuJL66fZa8wcpaK1uNOH_AGQSlZA</recordid><startdate>20100701</startdate><enddate>20100701</enddate><creator>Pressey, Joseph G.</creator><creator>Wright, Jordan M.</creator><creator>Geller, James I.</creator><creator>Joseph, David B.</creator><creator>Pressey, Christine S.</creator><creator>Kelly, David R.</creator><general>Wiley Subscription Services, Inc., A Wiley Company</general><scope>BSCLL</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20100701</creationdate><title>Sirolimus therapy for fibromatosis and multifocal renal cell carcinoma in a child with tuberous sclerosis complex</title><author>Pressey, Joseph G. ; 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| subjects | Antibiotics, Antineoplastic - therapeutic use cancer genetics Carcinoma, Renal Cell - drug therapy Carcinoma, Renal Cell - etiology Carcinoma, Renal Cell - pathology Child developmental therapeutics Fibroma - congenital Fibroma - drug therapy Fibroma - pathology Humans Kidney Neoplasms - drug therapy Kidney Neoplasms - etiology Kidney Neoplasms - pathology Loss of Heterozygosity Male pathology renal cell carcinoma Sirolimus - therapeutic use soft tissue sarcoma Thoracic Wall - pathology Tuberous Sclerosis - complications Tuberous Sclerosis - genetics Tumor Suppressor Proteins - genetics |
| title | Sirolimus therapy for fibromatosis and multifocal renal cell carcinoma in a child with tuberous sclerosis complex |
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