What condition might require prophylactic thyroidectomy before age 5?

The condition that may necessitate a prophylactic thyroidectomy before age 5 is Multiple Endocrine Neoplasia type 2A (MEN2A). This genetic syndrome is associated with medullary thyroid carcinoma, which develops from the parafollicular C cells of the thyroid. Individuals with MEN2A have a high lifetime risk of developing this cancer, often at a young age. In fact, guidelines recommend that children with a germline mutation in the RET proto-oncogene, which causes MEN2A, undergo a prophylactic thyroidectomy typically around the age of 5 to effectively minimize the risk of thyroid cancer.

While conditions like MEN1, DICER1, and Xeroderma Pigmentosum are associated with different endocrine or genetic profiles, they do not have the same high early risk for thyroid cancer as MEN2A does. For MEN1, while it is a hereditary condition involving multiple endocrine tumors, prophylactic thyroidectomy is not standard practice and management focuses on other glands. DICER1 syndrome is linked to other types of tumors, such as pleuropulmonary blastoma and thyroid neoplasms, but does not have the same specific recommendations for early thyroidectomy. Xeroderma Pigmentosum