Paraganglioma in Pregnancy: A Case Series and Review of the Literature

Abstract

Pregnancies complicated by a pheochromocytoma or paraganglioma are very rare, being estimated to occur in 0.007% of all pregnancies. Both the well-being of the mother and fetus need to be considered, and management can be challenging. The optimal management of women with a pheochromocytoma or paraganglioma in pregnancy is not well established. The objective of the study was to assess whether there is a difference in fetal or maternal mortality between pheochromocytomas and paragangliomas in pregnancy. We present an experience of eight pregnancies in four SDHB germline mutation-positive women with sympathetic paragangliomas, followed by a systematic review of the literature to compare the outcome of paragangliomas with that of pheochromocytomas occurring in pregnancy. In our case series, favorable fetal and maternal outcomes were seen in all eight pregnancies. From the systematic review, maternal and fetal mortality were lower in women with paragangliomas, at 3.6% and 12% respectively, compared with 9.8% and 16% in women with pheochromocytomas. Pregnant women with paragangliomas may be at a lower risk of adverse outcome than those with pheochromocytomas, but both maternal and fetal mortality rates are still higher than that of the general obstetric population.