OR29-4 Pregnancy Outcomes in Women with Pheochromocytoma and Paraganglioma: An International Multi-Center Study

Background: Unrecognized or newly diagnosed pheochromocytoma (PHEO) or paraganglioma (PGL) during pregnancy may lead to adverse maternal and fetal outcomes. Recommendations for the management of women with PHEO or PGL (PPGL) during pregnancy are limited by the scarcity of published literature. Objective: To describe the presentation, management and outcomes of women with active PPGL during pregnancy and to identify predictors of unfavorable maternal and fetal outcomes. Methods: international retrospective multi-center study, 1968-2018. Results: Seventy-nine women with active PPGL had 89 pregnancies at a median age of 28 yrs (range, 16-46). At the time of pregnancy, 37 (42%) women had unilateral PHEO, 11 (12%) bilateral PHEO, 26 (29%) PGL, 8 (9)% multifocal disease and 7 (8%) metastatic PPGL. Of 51 patients evaluated for a predisposing mutation, 38 were positive (12 SDHB, 11 VHL, 8 RET, 7 other). Median tumor size was 6 cm (range, 1.9-29). Of 74 patients with available data on function, 65 (88%) had functioning PPGL. Of 89 pregnancies, only 13 (15%) patients had a known PPGL prior to pregnancy (8 with known metastases), while in 42 (47%) pregnancies, PPGL was diagnosed during pregnancy at a median gestation of 16 weeks (range, 2-38) and during 34 (38%) pregnancies, PPGL remained unrecognized until postpartum, diagnosed at a median of 9 weeks (range, 0-78) after delivery. PPGL surgery was performed in 72 women: 14 intrapartum at median gestation week 16 (range, 13-26) and 58 postpartum at a median of 36 weeks (range, 0-270) after pregnancy. Of 41 pregnancies with antepartum diagnosis of PHEO not treated with surgery, 25 (61%) were managed with medical therapy and 15 (39%) were closely monitored. Delivery was by C-section in 39 (44%) pregnancies (38 healthy babies, one death delivered at 22 weeks) and vaginal in 35 (39%) with no adverse maternal or fetal outcomes. Elective abortion was pursued in 5 (6%) pregnancies. Intrauterine fetal demise occurred in 6 (7%) pregnancies at a median of 21 weeks gestation (range, 11-28), all in women with unrecognized functioning PPGL. Adverse maternal outcomes occurred in 3 (3%) pregnancies, all in unrecognized functioning PPGL resulting in 1 death (at gestation week 38) and 2 severe cardiovascular complications (immediately postpartum). The most significant predictor of an unfavorable pregnancy outcome was postpartum diagnosis of PHEO, with an odds ratio of 16.6 (95% CI, 2-140, P=0.001), but not maternal age, tumor size, presence of metastases, antepartum surgery for PHEO, or known PHEO syndrome. Conclusion: The majority of pregnancies in women with active PPGL had excellent outcomes, even in women with metastatic or functional disease, especially when PPGL was diagnosed before or during pregnancy, allowing for appropriate management. However, unrecognized and untreated PPGL was associated with 16-fold higher risk of either maternal or fetal complications.