The incidence of secondary neoplasms is 3-12% within the first 20 years from diagnosis. Childhood cancer survivors have 10-20 times the lifetime risk of second malignant neoplasms compared to age-matched controls. Second malignant neoplasms are the most common cause of death in longtime survivors after recurrence of the primary cancer.
Patients with retinoblastoma, Hodgkin disease, and Ewing sarcoma are at the greatest risk for developing second malignant neoplasms. An estimated 10% of children with ALL have a defect in the drug metabolizing enzyme (thiopurine methyl transferase), which places them at increased risk for the development of a second cancer. Long-term survivors who have an underlying inherited susceptibility to cancer have a second malignant neoplasm rate that approaches 50%. Neurofibromatosis also increases the risk of additional neoplasms. Li-Fraumeni syndrome can be associated with breast cancer and sarcomas at an early age. Because hepatoblastoma has been associated with familial polyposis, children with hepatoblastoma should be examined for the polyposis gene and then screened for colon cancer if needed.
Table 27-13 lists the second malignant neoplasms, their predisposing factors, signs and symptoms, and recommendations for follow-up.
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