Management of Paraganglioma in the Third Trimester of Pregnancy

Presentation Number: SUN-0794
Date of Presentation: June 22nd, 2014

Foiqa Chaudhry* and Colleen Rose Digman
University of Florida, Gainesville, FL


BACKGROUND:  Paragangliomas are rare neuroendocrine tumors arising from extra-adrenal autonomic paraganglia.  Diagnosis and management during pregnancy presents unique challenges due to the paucity of data regarding management especially in the third trimester of gestation.


A 28 year old woman, G3P1011, underwent an MRI for evaluation of persistent back pain at 36 6/7 weeks gestation.  This revealed a right adrenal mass that was confirmed on abdominal MRI to be a 3.8 x 3.5 x 4 cm solid, round, smoothly marginated mass with heterogeneity and no significant signal drop out.  She had remained normotensive during the pregnancy but experienced increased palpitations, anxiety, and night sweats for 1 year prior to the current pregnancy.  No family history of endocrine disorders was elicited.  Patient’s laboratory workup revealed elevated 24 hr urine normetanephrine (NME), greater than 2 times upper limit of normal. [serum free NME 5.3 nmol/L (ref < 0.90), serum free metanephrine < 0.20 nmol/L (ref < 0.50), 24 hr urine metanephrine 52 mcg/24 hr (ref 30-180), 24 hr urine NME 744 mcg/24 hr (ref 103-390), 24 hr total metanephrine 796 mcg/24 hr (ref 145-510) and 24 hr VMA 2.8 mg/24 hr (ref < 8.0)].  Dexamethasone suppression test, aldosterone and renin levels were within normal range.  Given symptoms, imaging characteristics and laboratory data, it was concluded that the patient had a pheochromocytoma and was admitted to the high risk obstetrics service.  She was started on phenoxybenzamine and underwent an uncomplicated C-section at 37 6/7 weeks gestation.  On POD 5 from C-section, she underwent laparoscopic right adrenalectomy.  Pathology specimen revealed mass adjacent to normal appearing adrenal gland and cytology positive for CD56 (+) neuroendocrine cells.  Thus, the diagnosis was most consistent with paraganglioma.  Follow up imaging, laboratory testing and workup for associated genetic/hereditary conditions is pending.  The patient and baby continue to do well with resolution of palpitations and anxiety. 


Guidelines regarding management of pheochromocytoma/paraganglioma in the third trimester of pregnancy are lacking given rarity of condition.  This patient had been normotensive and tolerated mild orthostasis on alpha blockade with no adverse effects to the fetus/neonate.  She also underwent successful right laparoscopic adrenalectomy in the immediate postpartum period which resolved concerns of ongoing phenoxybenzamine use while breast feeding.   Though she was presumed to have a pheochromocytoma based on imaging and abnormal biochemistry, final pathology and immunohistochemistry confirmed a diagnosis of a paraganglioma.  Further workup for synchronous lesions as well as assessment for associated genetic conditions is required.


Nothing to Disclose: FC, CRD