SUN-LB060 Paraganglioma in Pregnancy: Challenges to Treatment

Background: Paragangliomas are rare neuroendocrine tumors that arise from extra-adrenal chromaffin cells derived from the embryonic neural crest. Sympathetic paragangliomas secrete catecholamines and present with episodic hypertension and tachycardia. Paragangliomas in pregnancy are extremely rare. Here we describe a complicated case of a paraganglioma in pregnancy that highlights the special considerations taken during this state. Case Presentation: A 17-year-old pregnant female presented with chest pain and dyspnea on exertion at 32 weeks of gestation secondary to hypertensive urgency. Further history revealed several paternal family members with tumors and resistant hypertension. A work-up for secondary causes of hypertension was performed. Pertinent studies included plasma normetanephrines >50 nmol/L (0-0.89 nmol/L) and normal plasma metanephrines. Plasma fractionated catecholamines revealed a norepinephrine level of 18,977 pg/mL (85-1250 pg/mL), a dopamine level of 83 pg/mL (0-20 pg/mL), and a normal epinephrine level. MRI revealed an 8.0 cm hyperintense left infrarenal mass consistent with a paraganglioma. The patient was started on increasing doses of phenoxybenzamine followed by metoprolol to reduce her blood pressure and heart rate, keeping placental perfusion in mind. At 35 weeks of gestation she underwent successful cesarean delivery immediately followed by surgical resection of the tumor. Pathology revealed a 9.1 cm mass with nests of tumor cells, prominent vascularity, and foci of necrosis. A periaortic lymph node infiltrated by tumor indicated malignancy. Immunostaining was positive for neuron specific enolase, chromogranin, and synaptophysin. The patient briefly required vasopressors post-operatively, but she and the neonate recovered well with intensive monitoring. Genetic testing revealed a mutation in the succinate dehydrogenase B gene, increasing her risk for recurrent malignant paragangliomas and necessitating genetic testing of her child. Conclusions: Paragangliomas discovered during pregnancy are extraordinarily rare and present several challenges to management. While alpha and beta blockade should be initiated to correct volume depletion and avoid hemodynamic lability, caution must be taken to avoid excessive reductions in blood pressure and heart rate that may precipitate uteroplacental insufficiency and intrauterine growth restriction. Cesarean delivery is preferred over vaginal delivery to reduce the risk of precipitating hypertensive crisis. Furthermore, phenoxybenzamine crosses the placenta and neonates should be monitored in the NICU for hypotension in the first few days of life. Lastly, as approximately one quarter of all paragangliomas are associated with a genetic mutation, it is imperative to discuss genetic testing with the patient and the implications of a positive result for both the patient and offspring. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. Abstracts presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.