PEDIATRICS Vol. 52 No. 2 August 1973, pp. 192-196
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MEDULLARY CARCINOMA OF THE THYROID GLAND: THE COMPLETE SYNDROME IN A CHILD

Daniel L. Levin M.D.1, Charles Perlia M.D.1, and Armen H. Tashjian Jr. M.D.1

1 Departments of Pediatrics and Medicine, Rush Presbyterian-St. Luke's Medical Center, Chicago, Illinois, Laboratory of Pharmacology, Harvard School of Dental Medicine, and Department of Pharmacology, Harvard Medical School, Boston, Massachusetts

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.

Submitted on February 2, 1973
Accepted on April 10, 1973