The Great Masquerader: Paraganglioma Diagnosed in the Third Trimester of Pregnancy

Background: Paragangliomas are rare neuroendocrine tumors located outside the adrenal gland. Diagnosis is often delayed because clinical symptoms mimic preeclampsia or gestational hypertension. Controversy exists on immediate or interval tumor removal diagnosed in the third trimester. Guidelines lack regarding surgical cesarean delivery technique and obstetrical considerations for tumor excision at time of delivery.

Case presentation: A 17-year-old at 30 weeks gestation presented with hypertensive emergency and diagnosed with preeclampsia. Further evaluation revealed a paraganglioma. She was treated with alpha blockade prior to a planned cesarean delivery at 34 weeks with concomitant tumor excision. The procedure was complicated by aortic laceration, uterine atony, profound hypotension and DIC.

Conclusion: Not all cases of severe hypertension are preeclampsia. Secondary causes of hypertension in an adolescent during pregnancy should be considered. Preoperative management with alpha blockade presents a challenge for treatment of hemorrhagic shock and DIC. A multidisciplinary approach is essential to reduce morbidity. Women diagnosed with paraganglioma and their neonates should be offered genetic counseling.

Author(s): Vi Duong*, Alison Gambou, Rebecca Chornock, Sara Iqbal and Elizabeth Coviello

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