Coexistence of MEN 2A and papillary thyroid carcinoma and a recurrent pheochromocytoma 23 years after surgery: report of a case and a review of the Japanese literature.

A 59-year-old woman who had undergone bilateral partial adrenalectomy 23 years previously was referred to our hospital because of neck masses and a left adrenal tumor. Fine needle aspiration biopsy of the neck tumor and elevated levels of calcitonin and CEA revealed medullary thyroid carcinoma. Slightly elevated levels of urinary normetanephrine and metanephrine, and results of a clonidine suppression test, CT, magnetic resonance imaging, and 131I-metaiodobenzylguanidine scintigraphy of the abdomen indicated that the left adrenal tumor was a recurrent pheochromocytoma in the residual adrenal gland. After total thyroidectomy, bilateral medullary thyroid carcinomas and papillary thyroid carcinoma were recognized pathologically. Although the coexistence of bilateral pheochromocytoma and medullary thyroid carcinoma is suggestive of multiple endocrine neoplasia (MEN), association of MEN type 2A (MEN 2A) with papillary thyroid carcinoma may have occurred incidentally. A retrospective analysis of 9 cases of pheochromocytoma associated with papillary thyroid carcinoma reported in the Japanese literature demonstrated a male-to-female ratio of 1:8, and an average patient age of 56.2 years. The present case indicates that association between pheochromocytoma and thyroid carcinoma may not always involve medullary thyroid carcinoma.