Untreated pheochromocytoma in pregnancy drives maternal, fetal complications
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Unrecognized and untreated pheochromocytoma in women before or during pregnancy was associated with a substantially higher risk for maternal or fetal complications, according to data from a retrospective study.
“In women with known genetic predisposition to pheochromocytoma, appropriate counseling and monitoring should be instituted prior to planning the pregnancy,” Irina Bancos, MD, associate professor in the division of endocrinology at Mayo Clinic in Rochester, Minnesota, told Healio. “Optimal medical therapy with alpha-adrenergic blockade is key in any woman with pheochromocytoma during pregnancy. Multiple factors need to be considered to ensure the best possible management of a patient with concomitant pheochromocytoma and pregnancy. Evaluation by a multidisciplinary team may be necessary, and access to an adrenal endocrinologist with experience in managing pheochromocytoma is very important.”
Risk with unrecognized disease
In a retrospective study published in The Lancet Diabetes & Endocrinology, Bancos and colleagues analyzed data from the International Pheochromocytoma and Pregnancy Registry on 186 women with pheochromocytoma or paraganglioma with 197 pregnancies between 1980 and 2019. The researchers also conducted a systematic review of seven studies published between 2005 and 2019 that reported on 63 pregnancies from 55 patients. Inclusion criteria were pregnancy after 1980 and a diagnosis of pheochromocytoma or paraganglioma before or during pregnancy or within 12 months postpartum. Primary outcomes were maternal or fetal death and maternal severe cardiovascular complications of catecholamine excess. The researchers used logistic regression analysis to evaluate potential variables associated with these outcomes.
Within the cohort, diagnosis of pheochromocytoma or paraganglioma was made before pregnancy in 37 (15%) cases, during pregnancy in 134 (54%) cases, and after delivery in 78 (31%) cases.
Among 144 women evaluated for genetic predisposition for pheochromocytoma, 95 (66%) were positive.
Unrecognized pheochromocytoma or paraganglioma during pregnancy (OR = 27; 95% CI, 3.5-3,473.1), abdominal or pelvic tumor location (OR = 11.3; 95% CI, 1.5-1,440.5) and catecholamine excess at least 10 times the upper limit of the normal range (OR = 4.7; 95% CI, 1.8-13.8) were associated with adverse outcomes.
For patients diagnosed during pregnancy, alpha-adrenergic blockade therapy was associated with fewer adverse outcomes, with an OR of 3.6 (95% CI, 1.1-13.2) compared with no alpha-adrenergic blockade. Surgery during pregnancy was not associated with better outcomes compared with no surgery (OR = 0.9; 95% CI, 0.3-3.9).
Bancos said appropriate case detection and counseling for premenopausal women at risk for pheochromocytoma or paraganglioma could prevent adverse pregnancy-related outcomes.
“There are clear, actionable measures that, if instituted, will prevent pregnancy in a woman with untreated pheochromocytoma or significantly improve both maternal and fetal outcomes in women with diagnosed pheochromocytoma during pregnancy,” Bancos said. “For the first category, the actions include appropriate monitoring and counseling in women with known genetic predisposition to pheochromocytoma. In the second category, these include early diagnosis of pheochromocytoma during pregnancy, early intervention with medical therapy, and access to the adrenal endocrinologist for individualized advice and management.”
Future research, pregnancy recommendations
Bancos said the data show that alpha-adrenergic blockade was associated with improved outcomes, but the exact regimen, choice of medication, intensity of therapy and blood pressure goals are not completely clear.
“The role and the regimens for other medications, such as beta-adrenergic blockade, calcium channel blockers, are also unclear,” Bancos said. “We are currently designing a prospective study to better answer these questions.”
Bancos said some patients in the cohort were able to have a successful vaginal delivery; however, given the retrospective design of the study and institutional protocols at some centers recommending cesarean section, researchers cannot make strong recommendations for individualizing the mode of delivery.
“We have been surprised by a considerable number of patients with metastatic pheochromocytoma who had a successful pregnancy without any adverse outcomes,” Bancos said. “Likely, lower burden of disease, a more indolent course, and being treated by an expert team contributed to this success. Future studies need to understand in more depth the safety of pregnancy in these cases.”
For more information:
Irina Bancos, MD, can be reached at bancos.irina@mayo.edu.
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