Medullary carcinoma of the thyroid gland in association with pheochromocytoma has become a well-recognized syndrome in recent years. In addition, parathyroid adenomas, a peculiar facies, "marfanoid" habitus, multiple mucosal neuromas, and easily visible corneal nerves may be present.1,2
An increased propensity toward multicentric foci of independent chromaffin tumor development results in a high incidence of bilateral pheochromocytoma in this discorder. There is also a greater number of chromaffin tumors within each adrenal gland than is seen in the sporadic or familial cases of pheochromocytoma that are not associated with thyroid carcinoma.3,4 In spite of the tendency toward multicentricity, the finding of extra-adrenal pheochromocytomas has not, to our knowledge, been previously reported in this syndrome.
Our patient had medullary carcinoma of the thyroid and bilateral intra-adrenal pheochromocytomas. In addition, two extra-adrenal pheochromocytomas were found at separate operations.
First Admission, Aug 19, 1963.
—A 16-year-old white girl was
Marks AD, Channick BJ. Extra-adrenal Pheochromocytoma and Medullary Thyroid Carcinoma With Pheochromocytoma. Arch Intern Med. 1974;134(6):1106-1109. doi:10.1001/archinte.1974.00320240140020