Chromaffin-cell tumors in pregnancy: A case series and systematic review

Aim: We describe three chromafin-cell tumors managed during pregnancy as well as systematically review case reports published rom 2014 to 2018. Chromafin-cell tumors are rare catecholamine-producing tumors that can arise rom the adrenal medulla, where they are reerred to as pheochromo-cytomas, or rom extra-adrenal chromafin tissue, reerred to as paragangliomas. The incidence o chromafin-cell tumors identified during pregnancy is extremely rare, with an incidence o 0.32 cases per 100,000 pregnancy years. Cases: We describe diagnosis and management during pregnancy o 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 let pheochromocytoma. We perormed a systematic review o cases utilizing MEDLINE, EMBASE and Google Scholar with the terms (pheochromocytoma or paraganglioma) and (pregnancy or pregnant) within the timerame 2014 through 2018 (searched on April 9th, 2018). We ound that emergency cesarean section delivery (p < 0.05), maternal heart ailure or pulmonary edema (p<0.05) and etal or neonatal death (p < 0.05) were more common in women with a late or postpartum diagnosis o a chromafin-cell tumor compared to women with diagnosis during or beore pregnancy. Conclusion: Chromafin-cell tumors are rare during pregnancy. However, morbidity is severe and requires an early diagnosis or the best possible outcomes. Hypertension during pregnancy is the most common presenting symptom o these catecholamine-producing tumors. Severe hypertension, labile hypertension or hypertension beore 20 weeks, without proteinuria or lower extremity edema, should raise suspicion or a chromafin-cell tumor. Management should consist o an experienced multi-disciplinary team at a tertiary reerral hospital to ensure the best outcomes.