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Retinoblastoma and Secondary Cancer

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Janet L. Kwiatkowski, MD
Last Modified: November 1, 2001

Dear OncoLink "Ask the Experts,"
I had bilateral (hereditary) retinoblastoma when I was a child. What is my risk of developing other cancers later in life?

Janet L. Kwiatkowski, MD, OncoLink's Pediatric Oncology Section Editor, responds:

Dear OncoLink Reader:
Thank you for your question.

Two forms of retinoblastoma exist, a hereditary form and a non-hereditary form. Patients with retinoblastoma involving both eyes or patients with tumor in one eye and a family history of retinoblastoma are considered to have the hereditary form.

Patients with the hereditary form of retinoblastoma have an increased risk of developing other cancers later in life. This is attributable primarily to a germline (passed to all of the bodyís cells) mutation in the retinoblastoma tumor suppresser gene, RB1. Mutations in this gene have also been linked to other tumors in addition to retinoblastoma. The most common secondary tumors that develop in patients with hereditary retinoblastoma are bone and soft tissue sarcomas. Radiation therapy, one of the treatments frequently used in the treatment of retinoblastoma, further increases the risk of developing a second malignancy in patients with hereditary retinoblastoma. Therefore, it is important for all patients with a history of hereditary retinoblastoma to be followed closely by their physicians.

If you would like more information about retinoblastoma you can refer to the OncoLink Retinoblastoma section.