Diagnosis and management of pediatric endocrine neoplasms

To guide the clinician in the diagnostic evaluation of endocrine neoplasms, to distinguish between benign and malignant and determine when surgical referral is indicated.

Thyroid nodules are uncommon but malignant in as many as 27% of patients. Fine needle aspiration should be considered in adolescents, in which accuracy is as high as 90%; surgical resection should be undertaken in all preadolescents (<13 years) with a thyroid nodule. Prognosis for most primary thyroid malignancies is favorable. Primary hyperparathyroidism is rare and due to an adenoma in up to 70% of patients. Surgical resection carries a cure rate of 95% with the use of intraoperative parathyroid hormone assays. Adrenal neoplasms cover a wide spectrum of disorder. They are functional in 95% of patients and require a thorough diagnostic evaluation prior to surgical resection. Malignant lesions of the adrenal gland carry a poor prognosis when complete surgical resection cannot be achieved. Carcinoids are rare neuroendocrine neoplasms, primarily of the appendix, associated with carcinoid syndrome in 10% of patients. The indolent course warrants aggressive surgical control.

Endocrine neoplasms are unusual in the pediatric population. Their presence should raise concern about a multiple endocrine neoplasia syndrome and appropriate diagnostic and endocrine work-up. Most neoplasms will require surgical resection.