There is no clear consensus on the treatment of pheochromocytomas diagnosed during pregnancy, with most data limited to small case studies. Medical therapy is a vital aspect of treatment, regardless of if surgical intervention occurs during pregnancy; alpha-adrenergic blockade is essential and beta-adrenergic blockade may be required 10 to 14 days later. Antihypertensive drugs during pregnancy can cause fetal complications including hypoxia and intrauterine growth retardation, placental ischemia and placental abruption. These risks need to be balanced with the potential maternal complications from uncontrolled hypertension, which include hypertensive crisis, pulmonary edema, and cardiac and cerebral complications.7
Numerous case reports suggest an optimal window for surgical resection during the second trimester. If the diagnosis is made early in pregnancy or mid-pregnancy, surgery is recommended. The use of alpha-receptor blockade medications can decrease BP and control for expansion of blood volume.
In general, phenoxybenzamine is the drug of choice during pregnancy (pregnancy class C). However, the drug crosses the placenta and can result in transient hypotension in the neonate.9 Beta-blockade can be initiated after alpha-blockade to control for tachycardia.
According to case reports in the literature, if diagnosed early in pregnancy, appropriate medical treatment should be initiated until surgery can occur. If diagnosed later in pregnancy, medical treatment should be initiated and surgery deferred until delivery.9
Surgery is the definitive treatment for pheochromocytoma; however, the timing must be coordinated with respect to trimester of pregnancy and fetal status. Patients require postoperative assessment as well as close monitoring due to the possibility of incomplete resection of tumor and recurrence or development of a malignant adrenal mass. In addition, genetic screening is recommended to assess for hereditary causes of pheochromocytoma, including multiple endocrine neoplasia II and von Hippel-Lindau syndrome.8
In summary, the overall management of pheochromocytoma begins with a high clinical suspicion for the condition, appropriate assessment and diagnosis, with medical treatment and surgical resection timed according to the gestational age of the baby.
In early pregnancy, fetal outcome is improved with both medical treatment and tumor resection; after the second trimester, elective Cesarean section is preferred closer to delivery with improved maternal and fetal outcomes.9 With appropriate diagnosis and treatment, there is a significant reduction in maternal and fetal mortality associated with this rare diagnosis.
- Kamoun M, Mnif MF, Charfi N et al. Adrenal diseases during pregnancy: pathophysiology, diagnosis and management strategies. Am J Med Sci. 2014;347(1):64-73.
- Girling J, Martineau M. Thyroid and other endocrine disorders in pregnancy. Obstetrics, Gynaecology and Reproductive Medicine. 2010;20(9):265-271.
- Lenders JW. Pheochromocytoma and pregnancy: a deceptive connection. Eur J Endocrinol. 2012;166(2):143-150.
- Keeley E. Endocrine causes of hypertension in pregnancy: when to start looking for zebras. Semin Perinatol. 1998;22(6):471-484.
- Salazar-Vega JL, Levin G, Sansó G et al. Pheochromocytoma associated with pregnancy: unexpected favourable outcome in patients diagnosed after delivery. J Hypertension.2014; 32(7):1458-1463.
- Kamari Y, Sharabi Y, Leiba A et al. Am J Hypertens. Peripartum hypertension from pheochromocytoma: a rare and challenging entity. 2005;18(10):1306-1312.
- Dong D, Li H. Diagnosis and treatment of pheochromocytoma during pregnancy. J Matern Fetal Neonatal Med. 2014[Epub ahead of print].
- Oliva R, Angelos P, Kaplan E, Bakris G. Pheochromocytoma in pregnancy: a case series and review. Hypertension. 2010;55(3):600-606.
- Ahlawat SK, Jain S, Kumari S, Varma S, Sharma BK. Pheochromocytoma associated with pregnancy: case report and review of the literature. Obstet Gynecol Surv. 1999;54(11):728-737.