Multiple endocrine neoplasia type IIb: A clinicopathological report

Van Zyl J.A. ; Muller A.G.S. ; Rossouw D.J. ; Van Velden D.J.J. (1990)

Article

The clinicopathological findings in a 12-year-old boy with multiple endocrine neoplasia type IIb (MEN-IIb) are presented. He demonstrated the characteristics of a Marfanoid habitus and had mucosal neuromas of the lips, tongue, eyelids, and cornea. The enlarged right thyroid lobe showed an increased thallium-201 chloride uptake, and the serum calcitonin level was markedly raised. A total thyroidectomy was performed. Histopathological examination showed an infiltrating medullary carcinoma of the thyroid (MCT) and metastatic spread to supraclavicular lymph nodes. Special stains, immunocytochemistry, and electron microscopy revealed mucin-containing follicular structures lined by calcitonin-positive and thyroglobulin-negative tumour cells. Ultrastructurally the mucosal neuromas consisted of proliferating perineurial and Schwann cells. The serum calcitonin level fell below 300 pmol/L, and his stools returned to normal postoperatively. Because the incidence of MCT in MEN-IIb is virtually 100%, a total thyroidectomy in high risk patients-even before clinical evidence of malignant tumours-may be the only hope of cure.

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