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Pheochromocytoma and Paraganglioma Treatment (PDQ®)

Health Professional Version

Pheochromocytoma During Pregnancy

Pheochromocytoma diagnosed during pregnancy is extremely rare (0.007% of all pregnancies).[1,2] However, this situation deserves mention because women with hereditary conditions that increase the risk of developing pheochromocytoma are often also of child-bearing age, and the outcome of undiagnosed pheochromocytoma during pregnancy can be catastrophic.

Diagnosis

Prenatal diagnosis clearly results in decreased mortality for both mother and neonate.[3] Prior to 1970, a prenatal diagnosis of pheochromocytoma was made in only approximately 25% of cases, and the mortality rate for both mother and neonate was around 50%.[4,5] The prenatal diagnosis rate rose to greater than 80% through the 1980s and 1990s, and decreased maternal and neonatal mortality rates were 6% and 15%, respectively.[4,6]

The diagnosis of pheochromocytoma should be suspected in any pregnant woman who develops hypertension in the first trimester, paroxysmal hypertension, or hypertension that is unusually difficult to treat.[2,7] Normal pregnancy does not affect catecholamine levels.[8] Thus, the usual biochemical tests are valid. Magnetic resonance imaging is the localization method of choice because it does not expose the fetus to ionizing radiation.

Treatment

Phenoxybenzamine use is safe in pregnancy, but beta-adrenergic blockers should be initiated only if needed because their use has been associated with intrauterine growth retardation.[9,10] Resection of the tumor can often be performed safely during the second trimester, or tumor resection can be combined with cesarean section when the fetus is ready to be delivered. Case reports have documented successful outcomes in the rare circumstance when surgical resection was delayed until a short time after vaginal delivery.[11] The successful management of pheochromocytoma in pregnancy depends on careful monitoring and the availability of an experienced team of specialists.

References

  1. Harrington JL, Farley DR, van Heerden JA, et al.: Adrenal tumors and pregnancy. World J Surg 23 (2): 182-6, 1999. [PUBMED Abstract]
  2. Sarathi V, Lila AR, Bandgar TR, et al.: Pheochromocytoma and pregnancy: a rare but dangerous combination. Endocr Pract 16 (2): 300-9, 2010 Mar-Apr. [PUBMED Abstract]
  3. Freier DT, Thompson NW: Pheochromocytoma and pregnancy: the epitome of high risk. Surgery 114 (6): 1148-52, 1993. [PUBMED Abstract]
  4. Mannelli M, Bemporad D: Diagnosis and management of pheochromocytoma during pregnancy. J Endocrinol Invest 25 (6): 567-71, 2002. [PUBMED Abstract]
  5. Schenker JG, Granat M: Phaeochromocytoma and pregnancy--an updated appraisal. Aust N Z J Obstet Gynaecol 22 (1): 1-10, 1982. [PUBMED Abstract]
  6. Ahlawat SK, Jain S, Kumari S, et al.: Pheochromocytoma associated with pregnancy: case report and review of the literature. Obstet Gynecol Surv 54 (11): 728-37, 1999. [PUBMED Abstract]
  7. Keely E: Endocrine causes of hypertension in pregnancy--when to start looking for zebras. Semin Perinatol 22 (6): 471-84, 1998. [PUBMED Abstract]
  8. Jaffe RB, Harrison TS, Cerny JC: Localization of metastatic pheochromocytoma in pregnancy by caval catheterization. Including urinary catecholamine values in uncomplicated pregnancies. Am J Obstet Gynecol 104 (7): 939-44, 1969. [PUBMED Abstract]
  9. Butters L, Kennedy S, Rubin PC: Atenolol in essential hypertension during pregnancy. BMJ 301 (6752): 587-9, 1990. [PUBMED Abstract]
  10. Montan S, Ingemarsson I, Marsál K, et al.: Randomised controlled trial of atenolol and pindolol in human pregnancy: effects on fetal haemodynamics. BMJ 304 (6832): 946-9, 1992. [PUBMED Abstract]
  11. Junglee N, Harries SE, Davies N, et al.: Pheochromocytoma in Pregnancy: When is Operative Intervention Indicated? J Womens Health (Larchmt) 16 (9): 1362-5, 2007. [PUBMED Abstract]
  • Updated: October 24, 2013