A mother with ovarian cancer asks you to perform a bilateral salpingo-oophorectomy (BSO) on her 5-year-old daughter to prevent her daughter from developing the same cancer that is likely to kill her. What would you do?
The mother has small cell carcinoma of the ovary-hypercalcemic type (SCCOHT). Treatment outcomes for SCCOHT are extremely poor even with aggressive surgery and chemotherapy—10-20% long-term survival. Genetic testing of the daughter reveals that she has the same pathogenic variant as her mother. This variant, however, has incomplete penetrance and not everyone with it will develop SCCOHT. While this cancer mainly affects adolescents and young adults, rare occurrences in prepubertal children have been reported. Surveillance strategies for other forms of ovarian cancer have proven ineffective, and the benefits of risk-reducing BSO are unproven.
How would you weigh the risks of cancer with the inability to have genetically related children and the need for hormone replacement, or the benefit of the daughter participating in the decision when she is older against the risk of her developing cancer before she is able? Would you be willing to perform the procedure even if you do not think that it is the best option?
In the month’s Pediatrics Ethics Rounds (10.1542/peds.2024-068269), a cancer geneticist; an oncologist and bioethicist; a general pediatrician; and an attorney, pediatrician, and bioethicist discuss these issues. Do you agree with them? What would you do if you were confronted with this decision?
