Sirolimus therapy for fibromatosis and multifocal renal cell carcinoma in a child with tuberous sclerosis complex

Academic Article


  • 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. © 2010 Wiley-Liss, Inc.
  • Published In

    Digital Object Identifier (doi)

    Author List

  • Pressey JG; Wright JM; Geller JI; Joseph DB; Pressey CS; Kelly DR
  • Start Page

  • 1035
  • End Page

  • 1037
  • Volume

  • 54
  • Issue

  • 7