Abstract
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.
Copyright 2010 Wiley-Liss, Inc.
MeSH terms
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Antibiotics, Antineoplastic / therapeutic use*
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Carcinoma, Renal Cell / drug therapy*
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Carcinoma, Renal Cell / etiology
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Carcinoma, Renal Cell / pathology
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Child
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Fibroma / congenital
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Fibroma / drug therapy*
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Fibroma / pathology
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Humans
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Kidney Neoplasms / drug therapy*
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Kidney Neoplasms / etiology
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Kidney Neoplasms / pathology
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Loss of Heterozygosity
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Male
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Sirolimus / therapeutic use*
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Thoracic Wall / pathology
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Tuberous Sclerosis / complications*
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Tuberous Sclerosis / genetics
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Tuberous Sclerosis Complex 2 Protein
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Tumor Suppressor Proteins / genetics
Substances
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Antibiotics, Antineoplastic
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Tuberous Sclerosis Complex 2 Protein
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Tumor Suppressor Proteins
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Sirolimus