Pheochromocytoma and Paraganglioma in Pregnancy: a New Era

Curr Cardiol Rep. 2021 May 7;23(6):60. doi: 10.1007/s11886-021-01485-4.


Purpose of review: Pheochromocytoma and paraganglioma (PPGL) in pregnancy is a rare entity and management of these patients is fraught with uncertainty. Our objective is to review current literature and discuss diagnosis and management of these patients.

Recent findings: Outcomes of PPGL in pregnancy have improved in recent years. The greatest risk for adverse maternal and fetal outcomes is the diagnosis of PPGL after delivery. Alpha- and beta-adrenergic blockade is well tolerated and is associated with less adverse outcomes. Antepartum surgery is not associated with improved maternal or fetal outcomes. Biochemical testing and cross-sectional imaging should be performed prior to conception for patients with a known germline variant associated with PPGL.

Conclusions: Medical therapy should be initiated when PPGL is diagnosed in pregnancy. Antepartum surgery should be reserved for special circumstances. Case detection testing in high-risk patients can identify PPGL before pregnancy.

Keywords: Alpha-adrenergic blockade; Beta-adrenergic blockade; Paraganglioma; Pheochromocytoma; Pregnancy.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adrenal Gland Neoplasms* / diagnostic imaging
  • Adrenal Gland Neoplasms* / surgery
  • Female
  • Humans
  • Paraganglioma* / diagnostic imaging
  • Paraganglioma* / surgery
  • Pheochromocytoma* / diagnostic imaging
  • Pheochromocytoma* / surgery
  • Pregnancy