MEDULLARY CARCINOMA OF THE THYROID GLAND: THE COMPLETE SYNDROME IN A CHILD

[摘要] The history of a 16-year-old boy with medullary carcinoma of the thyroid, bilateral pheochromocytomas, diffuse visceral ganglioneuromas, peculiar facies, "Marfanoid" habitus, and profuse diarrhea is presented. Concentrations of calcitonin in serum were markedly elevated (54 to 61 ng/ml).Metastatic tumor nodules contained high calcitonin concentrations (220 MRC units/gm). Serum parathyroid hormone levels (0.38 to 0.42 ng/ml) were near the upper limit of the normal range, and the patient was normocalcemic. Immunoassays revealed elevated concentrations of prostaglandins of the ABE series (8 to 12 ng/ml) in the peripheral serum. This syndrome may result from a disorder of a single cell system–the chromaffin cell system–and may be considered a systemic chromaffinomatosis.

[学科分类] 儿科学