(1.06) Pheochromocytoma in Pregnancy

Pheochromocytomas are catecholamine-secreting neuroendocrine tumors arising from chromaffin cells within the adrenal medulla that occur in 0.002% of pregnancies. These tumors are life-threatening not only to mother, but also fetus via vasoconstrictive effects on uteroplacental vasculature especially when they are untreated.

Case(s) Description :

   We present a 33 year old pregnant African American Female with hypertension, type 2 diabetes mellitus, and sickle cell trait who presented to our Emergency Room (ER) with complaints of headaches, chest pain, palpitations, tremors, and fatigue. She had multiple similar presentations to  and treated for hypertensive emergencies. In addition, years prior she had imaging showing an unidentified abdominal mass. Presently, she was admitted for hypertensive urgency with an ultrasound verifying an intrauterine pregnancy at 11 weeks 5 days. MRI showed a 3.7cm right para-adrenal lesion most consistent with extra-adrenal neoplasm with evaluation revealing plasma norepinephrine levels of 2141.8 pg/mL [12.66 nmol/L] (reference range 0.00-150.6 pg/mL; 0.00-0.89 nmol/L) and 24-hour urine studies demonstrated normetanephrine  levels of 1739 μg/24hr [9492 nmol/24hr] (reference range 95-650 ug/24hr; 518.5-3547.9 nmol/24hr) and norepinephrine of 322 μg/24hr [1903.3 nmol/day] (reference range 14-120 ug/24hr; 82.7-709.3 nmol/day). This confirmed the diagnosis of pheochromocytoma. She was treated with phenoxybenzamine, but later switched to doxazosin and metoprolol for better BP control prior to surgery. Patient underwent laparoscopic right adrenalectomy at 18 weeks 1 day gestation without intraoperative complications. Pathology confirmed a 4.2cm pheochromocytoma with moderate differentiation due to a Grading of Adrenal Pheochromocytoma and Paragangliomascore score of 5. She was discharged home with metoprolol 25mg twice a day. However, she presented to ER 5 and 7 days after discharge with complaints of nausea and denied admission for further blood pressure control. She again presented to our ER 3 days after her unplanned, urgent cesarean section due to hypertension emergency. She again left against medical advice from the Emergency Room.

Discussion :

Pheochromocytoma in pregnancy have significant impact on mother and fetus. In cases of pheochromocytoma diagnosed prior to birth, fetal mortality is 9.5% to 15%. However, delayed detection of pheochromocytoma has a mortality rate of 29%.  There are overlapping symptoms of pregnancy, pre-eclampsia, gestational hypertension and pheochromocytoma, thus having a high index of suspicion for pheochromocytoma is imperative. In our case, patient had multiple ER visits for hypertension crises with a known abdominal mass on imaging prior. For non-pregnant patients, treatment consists of alpha-blockade, then beta-blockade if necessary followed by surgery. For pregnant patients, adrenalectomy is ideally performed in 2nd trimester. Our patient and her child tolerated procedure well with no complications, but it appeared her hypertension did not resolve. She was lost to follow up since her operation.