7735 A Rare Case of Paraganglioma in Pregnancy

Abstract Disclosure: H. Kreit: None. J. Patel: None. N.L. Shah: None. K.B. Guttenberg: None. Introduction: Paragangliomas are rare neuroendocrine tumors that consist of an abnormal growth of chromaffin cells. Exceptionally rare is a paraganglioma in pregnancy. Case Report: A 30-year-old woman presented at 21 weeks gestation with a syncopal episode while ambulatory at work. She reported dizziness, palpitations, sweating, and dyspnea prior to this episode. In the Emergency Department, she was found to have supraventricular tachycardia (SVT) with a heart rate (HR) of 170 beats per minute that resolved after vagal maneuvers. Initial evaluation, including thyroid function tests and echocardiogram, was unremarkable. Symptoms were thought to be due pregnancy, and she was discharged home. She continued to report intermittent palpitations, sweating, and dizziness that resolved spontaneously. Due to her tachycardia and persistent symptoms, biochemical evaluation for pheochromocytoma was obtained at 30 weeks gestation. This was notable for elevated 24 hour urine normetanephrine (4462 mcg, normal 40-412 mcg), normal 24 hour urine metanephrine (85 mcg, normal 25-222 mcg), elevated plasma normetanephrine (1334.7 pg/mL, normal < 148 pg/mL), and normal plasma metanephrine (42.8 pg/mL, normal < 57 pg/mL). MRI abdomen and pelvis without contrast was unremarkable. She was readmitted to the hospital and started on doxazosin. This was followed by initiation of metoprolol and a high sodium diet. C-section was planned for 37 weeks gestation. One day prior to her scheduled C-section, she presented to the hospital with contractions. She was diagnosed with hypertensive emergency complicated by pulmonary edema. Emergent C-section was performed. MRI abdomen and pelvis with contrast subsequently revealed bilateral enhancing retroperitoneal masses, measuring 4.4 cm on the right and 3.7 cm on the left. Retroperitoneal masses were resected 3 months after delivery without complication. Pathology showed a left-sided paraganglioma and a right-sided paraganglioma of undetermined malignant potential. Genetic testing was recommended. Conclusion: Paraganglioma in pregnancy is very rare. If left unrecognized and untreated, it carries increased risk of mortality to both the fetus and mother. A multidisciplinary approach with close monitoring is crucial for management. Presentation: 6/1/2024