Paraganglioma Presenting in Pregnancy: A Mimic of Pre-Eclampsia

The new presentation of pheochromocytoma (PCC) or paraganglioma (PGL) in pregnancy is very rare and can be life-threatening for mother and child.

Case Description :

A 26-year-old G3P2 woman at 34 weeks gestation presented to the ED for new onset hypertension. She was otherwise healthy with two prior uncomplicated pregnancies. She denied a family history of hypertension or endocrinopathies. On presentation, her blood pressure was 200/130 mmHg, associated with headaches, dry heaves, diaphoresis, and palpitations. Routine labs were significant for proteinuria. She was diagnosed with pre-eclampsia and started on labetalol and magnesium. She was admitted and had an urgent cesarean-section at 34 weeks and 2 days, delivering a healthy baby boy. The diagnosis of pre-eclampsia came into question when, six weeks post-partum, the patient’s hypertension and symptoms persisted. Additional testing revealed profoundly elevated plasma normetanephrines to 19,950 pg/mL (normal: 18-101 pg/mL). MRI of the chest, abdomen, and pelvis showed multiple retroperitoneal para-aortic masses and hepatic and vertebral lesions, consistent with metastatic PGL. Genetic testing revealed an SDHB gene mutation. The patient was started on alpha-methyldopa in addition to labetalol, and has had close blood pressure monitoring ever since. Discussion of further therapies is ongoing.

Discussion :

We present the case of a young patient with rare SDHB metastatic PGL without any known family history of PGL/PCC who first presented with symptoms during pregnancy. It is unclear if the onset of symptoms and catecholamine release were related to the physical stress of late pregnancy, or unfortunate timing in the natural course of her disease. She was initially misdiagnosed as pre-eclampsia due to the overlapping features of new-onset hypertension late in pregnancy accompanied by headache and proteinuria. It is impractical to routinely screen for PGL/PCC in all pregnant patients diagnosed as pre-eclampsia due to the rarity of these tumors and the harm from many false-positive results. Therefore, it is incumbent on the provider to have a high degree of suspicion for PGL/PCC with clinical features that are unusual for pre-eclampsia, such as intermittent palpitations, diaphoresis, orthostatic hypotension, or elevated blood sugars. The early detection of PGL/PCC greatly reduces maternal and fetal mortality. Alpha-blockers and cesarean delivery are key to avoiding hypertensive crises from unopposed alpha-mediated vasoconstriction and vaginal delivery, respectively. Fortunately, our patient delivered a healthy baby and did not have any additional pregnancy complications despite the delay in her diagnosis.