Atypical presentation of pheochromocytoma as part of multiple endocrine neoplasia IIa in pregnancy.

Abstract

Pheochromocytoma in pregnancy is extremely dangerous, especially when unrecognized, and can present alone or as part of a multineoplastic syndrome. Hypertension, its hallmark, is not present in all cases, particularly in women with multiple endocrine neoplasia type IIa. We report a gravida with undiagnosed multiple endocrine neoplasia IIa who presented initially with peripartum cardiomyopathy but was diagnosed with an underlying pheochromocytoma. Once recognized and treated with appropriate alpha-adrenergic blockade, her condition reversed quickly. Physicians should be aware that pheochromocytoma can present as cardiovascular collapse rather than just hypertension.