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Management of a pregnant patient with MEN2A syndrome and recurrent pheochromocytoma
Abstract Number: T-61
Abstract Type: Case Report/Case Series
Pheochromocytomas are rare catecholamine-producing endocrine tumors that produce paroxysmal HTN, headache, sweating and palpitations (1). Pregnancy complicated by undiagnosed pheochromocytoma is associated with up to a 50% maternal and fetal mortality rate (2). Early detection and appropriate management of this condition can be life-saving.
A 22 year old female G1P0 with MEN2A syndrome and pheochromocytoma presented at 39 weeks gestation for elective primary Cesearean delivery. The patient had a prior left adrenalectomy for pheochromocytoma and thyroidectomy for medullary thyroid cancer.
At 13 weeks gestation, a recurrent pheochromocytoma was diagnosed based on plasma free metanephrines in the upper range of normal and a right adrenal mass on MRI. The patient was admitted at 36 weeks gestation for initiation of alpha blockade and then underwent scheduled Cesarean delivery at 39 weeks.
Neuraxial anesthesia was utilized for the Cesarean delivery. Prior to epidural placement, an arterial line and a central line were placed. Neuraxial anesthesia was then titrated with 2% lidocaine to achieve a surgical block. Modifications in surgical technique included vacuum assistance for delivery without fundal pressure, in situ uterine closure, and avoidance of fundal massage. The patient was hemodynamically stable and did not require vasoactive medications. She was admitted to the ICU for invasive blood pressure monitoring. She was discharged on postpartum day 4.
Due to their rarity, pheochromocytomas can be missed or misdiagnosed as gestational hypertension or pre-eclampsia (Table 1). Definitive therapy is surgical resection, but timing in pregnancy requires individualized treatment. In the first 24 weeks of gestation, the patient should be stabilized on medication, and resection can be considered. After 24 weeks, this condition is usually best managed medically. Cesarean delivery (with or without simultaneous tumor removal) is generally recommended, as vaginal delivery may cause mechanical irritation of the pheochromocytoma (3). Cesarean delivery can be safely performed under neuraxial or general anesthesia. A general anesthetic would be favored if there was a plan for simultaneous pheochromocytoma resection or if there was significant hemodynamic instability.
1. Lenders J. Eur J Endocrinol 2012;166:143-150
2. Darr R, et al. Ther Adv Endocrinol Metab 2012;3(1):11–26
3. Oliva R, et al. Hypertension 2010;55:600-606