Abstract
We herein report a case of multiple endocrine neoplasia (MEN) type-2B treated by total thyroidectomy at the age six years. A girl, who had presented constipation since her infancy, was showing hypertrophic changes in her eyelids and lip, and developed multiple nodules on her tongue bas well. Although neither physical examination nor imaging tests revealed definite tumors in her neck, we diagnosed her as having MEN2B by a positive result of the calcium gluconate stimulation test, and the existence of RET gene mutation. She underwent total thyroidectomy that included cervical lymph node dissection, and bilateral medullary thyroid carcinomas without metastasis were histopathologically demonstrated. The postoperative course was non-eventful, indicating zero serum calcitonin level without recurrence so far. Although MEN2B in a child is extremely rare, early detection is possible by characteristic physical and clinical findings. When examining patients with constipation, it is important to check carefully whether they have the initial signs of MEN2B.
