Robotic Paraganglioma Resection in a Pregnant Patient

Presentation Number: SAT 388
Date of Presentation: April 1st, 2017

Margaret L Burks1, Shichun Bao*1 and Carmen Solorzano2
1Vanderbilt University Medical Center, Nashville, TN, 2Vanderbilt University


Background: Catecholamine secreting tumors in pregnancy (paragangliomas or pheochromocytomas) are rare, with an incidence of 0.007%; however, they represent one of the most life threatening medical conditions to a mother and fetus (1). Early recognition is important, as maternal and fetal mortality rates decline substantially with timely management. A high degree of suspicion is required as signs and symptoms can mimic those of more common conditions of pregnancy like pre-eclampsia. We present a case of a paraganglioma in a 33-year-old pregnant female which required robotic resection during the second trimester of pregnancy.

Clinical Case: The patient was seen in the emergency room prior to the index pregnancy for nausea, vomiting and intermittent, left lower quadrant abdominal pain. A CT scan of the abdomen and pelvis showed a 4.8 x 4.3 cm heterogeneously enhancing mass near the left adrenal gland and renal hilum. She reported episodic sweating for one year, palpitations weekly, and feeling anxious. Laboratory results were concerning for a norepinephrine secreting tumor. She had normal plasma metanephrines of 0.46 nmol/L (normal <0.49 nmol/L), but markedly elevated plasma normetanephrines of 7.12 nmol/L (normal <0.89). 24-hour urine epinephrine and dopamine were normal (<6 mcg/24 hrs and 218 mcg/24 hrs, respectively). Urine norepinephrine was elevated to 604 mcg/24 hrs (normal <90 mcg/24 hrs) and urine catecholamines were elevated to 610 mcg/24 hrs (normal <115 mcg/24 hrs). Chromogranin A level was elevated to 940 ng/mL (normal 0-95 ng/mL). Cortisol, DHEAS, renin, and aldosterone levels were normal. Soon after evaluation, the patient became pregnant. Her systolic and diastolic blood pressure were elevated during this time to 140 mmHg systolic and 100 mmHg diastolic. She was referred to endocrine surgery for resection. At 12 weeks of gestation, doxazosin was started. The mass was thought to be a paraganglioma, and removal was felt to be safest during the second trimester. Doxazosin dose was up titrated prior to surgery for blood pressure control. At 18 weeks of pregnancy, she underwent a robotic left retroperitoneal paraganglioma resection with excellent maternal and fetal outcomes.

Conclusion: Catecholamine secreting tumors in pregnancy are rare but must be recognized and treated to reduce maternal and fetal complications. Biochemical testing with 24-hour urine metanephrines and catecholamines is the same as for non-pregnant patients (1). Despite no formal guidelines for management of these patients, it is generally recommended tumors be removed either before 24 weeks of gestation or after delivery. Second trimester operative intervention is considered safest due to decreased risk of spontaneous abortion and less anatomic distortion (1). Medical management prior to surgery is approached similarly to non-pregnant patients. 


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