Metastatic Medullary Thyroid Carcinoma in Young Children with Mucosal Neuroma Syndrome

Publisher Website
Google Scholar
Abstract
Multiple endocrine neoplasia, type 2 b (MEN 2b) is a disorder characterized by a distinct phenotype and a predisposition for medullary thyroid carcinoma (MTC) and pheochromocytoma. Two siblings aged 3 and 6 yr with MEN 2b, who had elevated plasma calcitonin levels suggesting the presence of MTC, are described. Microscopic foci of MTC were found in their thyroid glands and the glands were removed. In the younger child, a metastatic focus was present in a cervical lymph node. Of 12 previously reported children with MEN 2b and MTC who were < 10 yr old at diagnosis, 5 had metastases. One of the present patients is the youngest described with this complication. The high frequency of metastases in very young patients with this syndrome has not been emphasized previously. These findings indicate the need for early diagnosis of MEN 2b and the importance of thyroidectomy at the earliest possible age when MTC is suspected by calcitonin screening tests.

This publication has 6 references indexed in Scilit: