Endocrine Abstracts

The occurrence of a pheochromocytoma (Pheo) or a paraganglioma (PGL) during pregnancy is extremely rare with a frequency of 0.002% of all pregnancies.

Because of the rarity of this association and the extremely variable clinical picture of Pheo/PGL, the diagnosis constitutes a real challenge for the clinicians. In fact, the differential diagnosis between Pheo/PGL and the more frequent gestational hypertension or (pre)eclampsia is very difficult. Paroxysmal or labile hypertension or hypertension in the first 20 weeks of pregnancy should alert the clinician to suspect a Pheo/PGL.

The occurrence of Pheo/PGL in pregnancy is a life-threatening event for both the fetus and the mother. If undiagnosed, fetal and maternal mortality amount to about 40-50% but can be decreased to 15 and 5% respectively by an early diagnosis and a proper treatment.

Diagnosis stems on laboratory results. The recommended test is the measurement of plasma or urinary metanephrines in view of their highest negative predictive value.

Tumor localization is performed by magnetic resonance imaging.

After diagnosis, the recommended treatment, to be continued until surgery, is with α-blockers, phenoxybenzamine or doxazosine. Surgical removal depends on the time of diagnosis. If early diagnosed, the best period for tumor removal is the second trimester. If lately diagnosed, the tumor may be removed simultaneously with caesarian section or after delivery. Laparoscopic adrenalectomy is the recommended procedure.

Because of the young age of pregnant women and the high rate of familial forms of Pheo/PGL, genetic analysis should be offered.

In view of the rarity and the complexity of this condition, pregnant women with a Pheo/PGL should be treated by a multidisciplinary team with high level of expertise in the field.