A case of bilateral pheochromocytoma during pregnancy

A 32-year-old woman, primigravida, who was 12 weeks’ pregnant, initially consulted a practitioner with awareness of right abdominal discomfort. She was pointed out bilateral adrenal tumor by abdominal ultrasonography. She was diagnosed with pheochromocytoma by blood examination and consulted our hospital. There were no signs of preeclampsia. Her other past history were unremarkable. A physical examination showed a temperature of 35.7°C and a respiratory rate of 16 breaths/min. Her blood pressure was 129/90 mmHg and pulse rate was 86 beats/min. Her heart and breath sounds were normal. The size of her uterus was consistent with 12 weeks of gestation and fetal heart rate was 148 beats/min. There were no palpable masses in the thyroid, no uterine contractions, and no edema was detected. Major laboratory findings included a hematocrit of 38.8% and white blood cell count of 8,100/mm³, with 72.0% neutrophils and the platelet count was 391,000/mm³. The blood sugar level in the fasting was 87mg/dl. Serum free T3, free T4, and TSH levels were 3.07 pg/ml (normal, 2.30–4.00), 1.22 ng/dl (normal, 0.90–1.70), and 3.940 μunit/ml (normal, 0.500–5.000), respectively. Urinary albumin was negative. A 24-h urine sample demonstrated elevated metanephrine (14 mg/24 h; normal, 0.005–0.20 mg/24 h) and normetanephrine levels (12 mg/24 h; normal, 0.10–0.28 mg/24 h). An ultrasonogram showed a normal fetus, which was compatible with 12 weeks of gestation and solid masses. The crown-rump length was 59 mm and the biparietal diameter was 22 mm. Magnetic resonance imaging (MRI) confirmed bilateral adrenal masses with central necrosis (right, 4.9 × 4.4 × 4.2 cm; left, 7.3 × 6.1 × 7.5 cm), which were compatible with bilateral pheochromocytoma (Figure 1a, b). A tumor exhibiting relative non-uniform low signals on T1 MRI was observed in both adrenal glands. Alpha-adrenergic blockade with doxazosin mesylate 2 mg daily was initiated and blood pressure was maintained under 140/90 mmHg. Hydration was also started at 14 weeks of pregnancy. At 15 weeks’ gestation, exploratory laparotomy and bilateral adrenalectomy were performed. Noradrenaline was used by an anesthesiologist during an operation and maintained blood pressure. With regard to surgical findings, no hemorrhage or abnormal adhesions were observed in the abdominal cavity. A soft elastic tumor measuring 9 cm was palpable in the retroperitoneum at the inferior border of the pancreatic body (Figure 2a). The tumor was removed en bloc without leaving any remnants. After left adrenalectomy, right adrenal adrenalectomy was then performed (Figure 2b). The operative time was 167 minutes and the amount of bleeding was approximately 215 ml. The patient recovered uneventfully. The right tumor measured 5.5 × 4.5 × 3.5 cm, with a weight of 60 g (Figure 3a). The left tumor measured 9.0 × 8.5 × 5.5 cm, with a weight of 350 g (Figure 3b). The cut surface was a yellowish solid tumor, and areas of bleeding accompanied by necrosis in a branched pattern were observed. Histology of the masses confirmed pheochromocytoma of bilateral adrenal glands (Figure 4a, b). The postoperative course was uneventful but the patient also received daily prednisolone. Urinary metanephrine and normetanephrine levels were normalized on the 3rd day postoperation. Vital signs, the height of the uterus, fetal heart sounds, and urine protein levels were monitored accordingly. Postoperatively, the patient’s blood pressure was not controlled with any antihypertensive agents, with normalization of urinary catecholamine levels over time. The patient and her fetus were in good health. She did not show any signs of intrauterine growth retardation or pregnancy-induced hypertension. Therefore, she continued pregnancy as an outpatient. At 39 weeks and 1 day of gestation, a healthy male neonate weighing 2810 g was delivered by vaginal birth with Apgar scores of 8 and 9 at 1 and 5 min, respectively. She was discharged on the 4th postpartum day and was followed up. Her neonate was also discharged with a body weight of 2718 g on the 4th postpartum day. All date reported in the manuscript have been visualized and then approved by our University Hospital Ethics Committee and all procedures carried out on the patients were in compliance with the Helsinki Declaration. Moreover, the patient has given written explicit, express and unequivocal consent to publish her sensible date on our manuscript.

We present a case of bilateral pheochromocytoma during pregnancy that was diagnosed in the first trimester. Differentiating pheochromocytoma from pregnancy-induced hypertension is important. Early diagnosis and appropriate blood pressure management with medical treatment followed by surgical removal of the tumor results in good maternal and fetal outcomes.