Abstract

Aim: We describe three chromaffin-cell tumors managed during pregnancy as well as systematically review case reports published from 2014 to 2018. Chromaffin-cell tumors are rare catecholamine-producing tumors that can arise from the adrenal medulla, where they are referred to as pheochromocytomas, or from extra-adrenal chromaffin tissue, referred to as paragangliomas. The incidence of chromaffin-cell tumors identified during pregnancy is extremely rare, with an incidence of 0.32 cases per 100,000 pregnancy years. Cases: We describe diagnosis and management during pregnancy of a 25-year-old with a 7.3 cm right pheochromocytoma, a 23-year-old with metastatic paraganglioma and SDHB mutation, and a 28-year-old with MEN2A and a left pheochromocytoma. We performed a systematic review of cases utilizing MEDLINE, EMBASE and Google Scholar with the terms (pheochromocytoma or paraganglioma) and (pregnancy or pregnant) within the timeframe 2014 through 2018 (searched on April 9th, 2018). We found that emergency cesarean section delivery (p < 0.05), maternal heart failure or pulmonary edema (p<0.05) and fetal or neonatal death (p < 0.05) were more common in women with a late or postpartum diagnosis of a chromaffin-cell tumor compared to women with diagnosis during or before pregnancy. Conclusion: Chromaffin-cell tumors are rare during pregnancy. However, morbidity is severe and requires an early diagnosis for the best possible outcomes. Hypertension during pregnancy is the most common presenting symptom of these catecholamineproducing tumors. Severe hypertension, labile hypertension or hypertension before 20 weeks, without proteinuria or lower extremity edema, should raise suspicion for a chromaffin-cell tumor. Management should consist of an experienced multidisciplinary team at a tertiary referral hospital to ensure the best outcomes.

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