Pheochromocytoma and pregnancy: the epitome of high risk

Past review has shown that one half of the fetuses and nearly that many mothers died of pheochromocytoma if the tumor was not detected in the mother before delivery. The rarity of the occurrence adds to the potential danger because experience with diagnosis and management is unusual. However, the diagnosis is straightforward when considered because pregnancy does not alter diagnostic levels of catecholamines. Management must be individualized and can be done safely.

The cases of five patients with pheochromocytomas during pregnancy are presented here. Diagnosis was made by urinary catecholamine measurement. Localization methods included caval catheterization and ultrasonography. Blood pressure control was done with alpha- and beta-adrenergic blockers. Treatment strategy was planned by a team of internists, obstetricians, surgeons, and anesthesiologists.

All five mothers and three fetuses survived. The two fetal deaths were planned terminations. Two mothers and one fetus had multiple endocrine neoplasia type 2B syndrome. One mother had two recurrences, one of which was involved with the pregnancy.

Pregnancy does not alter urinary catecholamine levels to confuse the diagnosis of pheochromocytoma. Localization by ultrasonography and magnetic resonance imaging is safe to the fetus. alpha- And beta-adrenergic blockers are well tolerated. Every effort should be made to save a normal fetus when the tumor is first discovered during the third trimester. Timing of excision is a decision best done by team planning.