Current opinion in pediatrics · 2009
Diagnosis and management of pediatric endocrine neoplasms.
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Iqbal Corey W, Wahoff David C
Аннотация
PURPOSE OF REVIEW: To guide the clinician in the diagnostic evaluation of endocrine neoplasms, to distinguish between benign and malignant and determine when surgical referral is indicated.
RECENT FINDINGS: 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.
SUMMARY: 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.
RECENT FINDINGS: 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.
SUMMARY: 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.