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Pheochromocytoma in Pregnancy


Learning Objectives

  • Describe the clinical presentation of pheochromocytoma in pregnancy.

  • Discuss appropriate management of pheochromocytoma in pregnancy.

Pheochromocytoma is a catecholamine-secreting tumor that arises from chromaffin cells of the adrenal medulla or the sympathetic ganglia.

Clinical Presentation

  • Symptoms ( Fig. 24.1 ):

    • Severe hypertension—often difficult to control. Patient may have paradoxical supine hypertension with normal blood pressure in the sitting or erect position. This is because the gravid uterus can compress the tumor in the supine position and trigger catecholamine release

    • Paroxysmal or persistent hypertension

    • Headaches

    • Palpitations

    • Sweating

    • Panic attack-type symptoms

    Fig. 24.1, Symptoms of pheochromocytoma.

  • Presents similarly to hypertensive disorders of pregnancy, but pheochromocytoma rarely has proteinuria and in pheochromocytoma the hypertension persists after childbirth ( Table 24.1 )

    Table 24.1
    When to Suspect Pheochromocytoma in a Pregnant Patient
    Intermittent labile blood pressures
    Alternating hyper- and hypotension
    Hypertension that worsens with beta-blockers
    Paroxysmal symptoms of anxiety, diaphoresis, headache, palpitations, etc.
    Family history of pheochromocytoma
    Café-au-lait macules

Diagnosis

  • Initial diagnosis is made by measurement of blood and/or urine catecholamines and their metabolites. As in nonpregnant women, the diagnosis is usually based upon the results of 24-hour urinary fractionated metanephrines and catecholamines and plasma fractionated metanephrines. However, clinicians must be aware that treatment with labetalol may give false positive results to this test

  • The localization of the neoplasm includes the use of ultrasound, MRI, and CT scan

    • MRI examination (without gadolinium) has high sensitivity and specificity in pregnancy

    • Ultrasound is convenient with no harm to the fetus and is often the first method to discover pheochromocytoma during pregnancy. The disadvantage is poor sensitivity, especially for small adrenal pheochromocytomas and extra-adrenal paragangliomas

    • CT scan examination has a high sensitivity for the diagnosis of pheochromocytoma. However, its use in pregnancy is limited due to concerns for fetal exposure to radiation

    • Nuclear examination such as 131I iodo benzyl guanidine (MIBG) scintigraphy and octreotide are specific and sensitive diagnostic methods but are not appropriate during pregnancy

      Pheochromocytoma Simulation

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