Skull Base 2011; 21(2): 103-108
DOI: 10.1055/s-0031-1275256
ORIGINAL ARTICLE

© Thieme Medical Publishers

Secondary Skull Base Malignancies in Survivors of Retinoblastoma: The Memorial Sloan Kettering Cancer Center Experience

Jeffrey C. Liu1 , Babak Givi1 , Suzanne Wolden1 , Ruth A. Kleinerman2 , Ira J. Dunkel1 , Nancy Lee1 , Jatin P. Shah1 , David H. Abramson1 , Dennis H. Kraus1
  • 1Memorial Sloan Kettering Cancer Center, New York, New York
  • 2Radiation Epidemiology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, NIH, DHHS, Bethesda, Maryland
Further Information

Publication History

Publication Date:
16 March 2011 (online)

ABSTRACT

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.

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Jeffrey C LiuM.D. 

Box 240 Memorial Sloan Kettering Cancer Center

New York, NY 10065

Email: JCL2006@gmail.com