Pheochromocytoma and pregnancy: a rare but dangerous combination

To review the literature on pheochromocytoma in pregnancy (PIP) published during the 11-year period 1998 through 2008.

We searched MEDLINE data sources from 1998 through 2008 using the search terms "pheochromocytoma" and "pregnancy" and reviewed case reports of PIP published in English.

We identified 54 case reports in MEDLINE data sources, of which 51 reports including 60 cases were reviewed. In comparison with the previous decade, a decreased rate of antenatal diagnosis (from 83% to 70%) and increased maternal and fetal mortality (from 4% to 12% and from 10% to 17%, respectively) were observed. Prematurity was present in 53% of the infants of mothers with antenatally diagnosed pheochromocytoma who gave birth to a live infant. Hypertension was the most common manifestation (88%), with 33% of patients having antepartum hypertensive crises. Urinary catecholamines (64%) and metanephrines (40%) were the most commonly used tests, whereas urinary and plasma metanephrines were the most sensitive tests (100%). Ultrasonography had poor sensitivity (54%), especially during the third trimester. Magnetic resonance imaging was 100% sensitive for adrenal tumors but had only 50% sensitivity for extra-adrenal tumors. A syndromic or familial presentation was seen in 18% of patients, and 32% of patients had bilateral or extra-adrenal tumors.

In comparison with the previous decade, a decreased rate of antenatal diagnosis and increased maternal and fetal mortality were observed. Prematurity was a major morbidity associated with PIP. Hypertensive crises were more common during pregnancy. Urinary and plasma metanephrines had the highest sensitivity to detect PIP. Genetic screening should be offered to all pregnant women with pheochromocytoma.