In the following case, we will discuss the clinical, ethical, and legal intricacies associated with the management of a young child with a hereditary cancer predisposition syndrome. Patients with germline pathogenic variants in SMARCA4 are at an increased risk for development of small cell carcinoma of the ovary-hypercalcemic type, malignant rhabdoid tumors, and some lung cancers. This case highlights the complexity of a case wherein a mother is found to have this genetic syndrome, and further testing reveals her daughter to have the same pathogenic variant. Through this case, we explore the oncologic, genetic, legal, and ethical considerations at play when making an irreversible decision for a child that affects her current and future medical and reproductive capacities. To do so would mitigate the risk of future malignancy, adding a layer of legal and ethical complexity. Although each contributor individually concludes that surgery in this case should be delayed, this case demonstrates the need for an individualized approach that considers medical evidence, patient and family interests, and child welfare.
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