Bilateral pheochromocytoma in pregnancy heralding multiple endocrine neoplasia syndrome IIA - A case report

BACKGROUND: Multiple endocrine neoplasia syndrome type IIA (MEN IIA) has ra rely been encountered in pregnancy. CASE: A 22-year-old, nulliparous woman developed bilateral pheochromocytoma s during pregnancy. This finding aroused suspicion for MEN IIA, and close e ndocrinologic follow-up was arranged. Four years later, hyperparathyroidism developed, and the diagnosis was established. The patient underwent prophy lactic total thyroidectomy with parathy void exploration. CONCLUSION: This was the first case of MEN IIA in pregnancy in which the di agnosis was established prior to the development of medullary thyroid cance r, thereby allowing prophylactic thyroidectomy. Tile presence of bilateral neoplastic disease in young patients may be indicative of a hereditary pred isposition to malignancy. (J Reprod Med. 2001;46: 385-388).