PREGNANCY AND PARAGANGLIOMA: A RARE BUT DANEGEROUS ASSOCIATION

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 37-82-Pheochromocytoma & Paraganglioma
Clinical
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-68
Sarah Varghese1, Amit Bhargava*2, Sameera Tallapureddy2 and Paul Tooran Labinson3
1University of Connecticut, Hartford, CT, 2University of Connecticut, Farmington, CT, 3CMG, West Hartford, CT
Pregnancy and paraganglioma : A Rare and dangerous association

Varghese, Sarah MD1; Bhargava, Amit MD1; Tallapureddy, Sameera MD1; Labinson Tooran Paul DO2.

Division of Endocrinology, University of Connecticut Health Center, Farmington CT 06030.1

Division of Endocrinology , Hartford Hospital , Hartford CT 06105.2

Background: Paraganglioma is a rare disease and its discovery during pregnancy is even rarer.  It is important to consider it and manage appropriately especially in pregnancy, as otherwise increased maternal and fetal mortality can be encountered.

Clinical case: A 20 year old Caucasian normotensive pregnant female at 16 weeks gestation with history of pancreatitis presented to the ER with recurrent episodes of abdominal pain. Work-up included an abdominal MRI resulting in an incidental finding of a 1.7 x 1.5 cm hyper vascular enhancing mass lesion possibly arising off of the inferior-most aspect of the left adrenal gland. Plasma metanephrines were normal at 35 (<57pg/ml). Plasma normetanephrines were elevated at 188 (<148pg/ml). The 24 hour urinary levels of normetanephrines were elevated at 635 (40-412 pg. /ml), however urinary metanephrines, cortisol and VMA levels were within normal limits. The patient was treated with a low dose alpha-blocker. She underwent an exploratory laparotomy at 24 weeks gestation with resection of a 1.5 cm extra-adrenal mass, the final pathology of which confirmed it to be a paraganglioma. She is currently being closely monitored during rest of her pregnancy and will also be undergoing genetic screening for familial paraganglioma.

Conclusion: The cases of paraganglioma diagnosed during pregnancy have been reported with typical signs and symptoms. Levels of catecholamine’s and their metabolites in a pregnant individual has been reported either to be similar or mildly increased when compared to a non- pregnant individual, thus making it difficult to diagnose on the basis of biochemical levels. Imaging modalities in pregnancy are restricted to MRI only. To the best of our knowledge, there have been no cases of paraganglioma reported in the literature of patients presenting with abdominal pain in early pregnancy as the only symptom. The etiology of the pain is not well understood. It is critical to have low index of suspicion and diagnose as early as possible in a pregnant patient to reduce the risks of morbidity and mortality, as reported cases in literature that underwent early surgery during the pregnancy had better outcomes for both mother and fetus.

Mannelli M, Bemporad D. Diagnosis and management of pheochromocytoma during pregnancy. J Endocrinol Invest. 2002;25(6):567–571. Grodski S, Jung C, Kertes P, Davies M, Banting S. Phaeochromocytoma in pregnancy. Intern Med J. 2006;36(9):604–606. Ahlawat SK, Jairi S, Kumari S, Varma S, Sharma BK. Pheochromocytoma associated with pregnancy: Case report and review of the literature. Obstet Gynecol Surv. 1999;54(11):728–737 Zuspan FP. Urinary excretion of epinephrine and norepinephrine during pregnancy. Journal of Clinical Endocrinology and Metabolism 1970 30 357–360.

Nothing to Disclose: SV, AB, ST, PTL

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