MULTIPLE ENDOCRINE NEOPLASIA TYPE-2-B - A CASE-REPORT WITH LIGHT AND ELECTRON-MICROSCOPIC AND IMMUNOHISTOCHEMICAL CORRELATION

Purpose: To describe a case of multiple endocrine neoplasia type two B (MEN IIB) where ocular and systemic clinicopathological findings are correlated, in association with light and electron microscopic and imm unohistochemical findings. Methods: A 23-year-old man presented with m ucosal neuromas of the lips, tongue and eyelids, a Marfanoid habitus a nd prominent corneal nerves. These findings led to the diagnosis of mu ltiple endocrine neoplasia type two B. The patient subsequently develo ped phaeochromocytoma and metastatic medullary thyroid carcinoma (MTC) which led to his demise. Correlation of light and electron microscopi c and immunohistochemical findings with the systemic and ocular findin gs is presented to emphasise the aggressiveness of MTC in MEN IIB. Cli nicopathological correlation was obtained by examining the eyes post m ortem. Results and conclusions: Three new findings in MEN IIB have bee n established by this study. The enlarged corneal nerves can now be re garded as ganglioneuromas. Medullary thyroid carcinoma metastases were found in the choroid. Light and electron microscopic examination of t he eye showed ganglioneuromas of the nerves in the limbus, trabecular meshwork, uveal tract and posterior ciliary nerves; this finding may a ccount for the glaucoma occasionally seen in patients with MEN IIB.