BETHESDA, MD. — With timely diagnosis and treatment, the prognosis can be good for women with pheochromocytoma during pregnancy, Dr. Henri Timmers said at an international symposium on pheochromocytoma sponsored by the National Institutes of Health.
His conclusion, based on a literature review of more than 100 cases, also found that maternal morbidity is high when diagnosis is delayed, said Dr. Timmers, of the department of endocrinology, Radboud University Nijmegen Medical Center, Nijmegen, the Netherlands.
Pheochromocytoma affects an estimated 1/50,000 full-term pregnancies and is associated with high maternal and fetal morbidity and mortality. Pheochromocytoma during pregnancy is often missed because it can mimic preeclampsia, delaying diagnosis and appropriate treatment.
Dr. Timmers and associates found a total of 174 reports of histology-confirmed cases of pheochromocytoma during pregnancy, in a PubMed database search for case reports in English. The mean age of the women was 28 years, 7% had previously been diagnosed with pheochromocytoma and 17% had been previously diagnosed with hypertension; 61% had been pregnant before and 14% had been previously diagnosed with pregnancy-induced hypertension.
In 73% of cases, the diagnosis of pheochromocytoma was made before delivery, with the remainder diagnosed postpartum (17%) or postmortem (10%).
Nearly 90% of the patients were hypertensive, but in only 42% of the cases was presentation typical for pheochromocytoma, where hypertension is usually accompanied by headache, palpitations, or sweating. In 25% of the cases, presentation was a hypertensive emergency, which included cases of severe pulmonary edema, Dr. Timmers said.
In 31% of the cases (54 patients), the initial diagnosis was incorrect, with almost half of the incorrectly diagnosed patients presenting with a severe cardiovascular complication, such as heart failure; of these 24 patients, 10 patients died. Fetal and neonatal mortality was 22%. Overall maternal mortality was about 14%, but it was markedly higher in different subgroups: It was 38% among the women who were incorrectly diagnosed and 49% among those with a cardiovascular emergency.
The biochemical test with the greatest diagnostic sensitivity was urinary metanephrines, with a sensitivity of 98%; the lowest sensitivity was for plasma catecholamines (91%). The sensitivity of MRI was 95%.
Surgery was performed either before 24 weeks' gestation or during a cesarean section in 69 patients. Surgery was performed post partum in 68.
During the discussion, moderator Dr. William Manger, chairman of the National Hypertension Association, New York, said pheochromocytoma, though rare, is devastating and should be routinely considered in pregnant women with hypertension.