EARLY PRESENTATION OF METASTATIC MEDULLARY CARCINOMA IN MULTIPLE ENDOCRINE NEOPLASIA, TYPE IIA - IMPLICATIONS FOR THERAPY

A girl 5 years 11 months of age, belonging to an extensive kindred wit h multiple endocrine neoplasia, type IIA (MEN IIA), was found to have multifocal medullary thyroid carcinoma with metastasis in one paraglan dular lymph node after positive findings on a calcium-pentagastrin sti mulation test. Her sister, 3 years 8 months of age, also had an elevat ed calcitonin level, and thyroidectomy revealed C-cell hyperplasia and a focus of medullary thyroid carcinoma. These two cases underscore th e need for prophylactic thyroidectomies in MEN IIA patients as young a s 5 years of age and strict yearly provocative screening beginning at age 1 year.