Secondary skull base malignancies in survivors of retinoblastoma: The memorial sloan kettering cancer center experience Academic Article uri icon


MeSH Major

  • Head and Neck Neoplasms
  • Perioperative Care


  • We report the pathology and outcome of secondary skull base tumors in patients previously treated with external beam radiation for retinoblastoma (Rb). Rb patients are at increased risk of second head and neck primary malignancies due to early radiation exposure during treatment and loss of RB1 protein in genetic carriers. An institutional database was reviewed for patients with retinoblastoma who had previously received radiation therapy and subsequently developed skull base tumors. Seventeen patients met the selection criteria. The median age of Rb diagnosis was 12 months. Thirteen cases underwent enucleation in addition to radiation therapy as part of initial Rb treatment. A median of 19 years elapsed between the diagnosis of Rb and diagnosis of skull base malignancy. The most common tumors were osteogenic sarcoma (39%) and leiomyosarcoma (22%). Eleven (71%) patients received postoperative chemotherapy, and 7 (41%) received postoperative radiotherapy. Three (24%) patients underwent salvage surgery for recurrent disease. Five-year survival was 68%, and 10-year survival was 51% by Kaplan-Meier analysis. Secondary malignancy in Rb patients is a well-defined event. The use of surgery with appropriate adjuvant therapy was associated with a 51% 10-year survival in this study population.

publication date

  • March 17, 2011



  • Academic Article



  • eng

PubMed Central ID

  • PMC3312595

Digital Object Identifier (DOI)

  • 10.1055/s-0031-1275256

PubMed ID

  • 22451810

Additional Document Info

start page

  • 103

end page

  • 8


  • 21


  • 2