A case of bilateral pheochromocytoma during pregnancy

Kitayama et al. BMC Surgery A case of bilateral pheochromocytoma during pregnancy Kishu Kitayama 0 Shinichiro Kashiwagi 0 Ryosuke Amano 0 Satoru Noda 0 Go Ohira 0 Sadaaki Yamazoe 0 Kenjiro Kimura 0 Kae Hamamoto 2 Akihiro Hamuro 1 Masahiko Ohsawa 3 Naoyoshi Onoda 0 Kosei Hirakawa 0 0 Department of Surgical Oncology, Osaka City University Graduate School of Medicine , 1-4-3 Asahi-machi, Abeno-ku, Osaka , Japan 1 Department of Obstetrics and Gynecology, Osaka City University Graduate School of Medicine , 1-4-3 Asahi-machi, Abeno-ku, Osaka , Japan 2 Department of Metabolism and Molecular Medicine, Osaka City University Graduate School of Medicine , 1-4-3 Asahi-machi, Abeno-ku, Osaka , Japan 3 Department of Diagnostic Pathology, Osaka City University Graduate School of Medicine , 1-4-3 Asahi-machi, Abeno-ku, Osaka , Japan Background: Pheochromocytoma is a disease where catecholamines are secreted. If pheochromocytoma occurs during pregnancy, it can be difficult to diagnose because it is similar to pregnancy-induced hypertension. Furthermore, bilateral pheochromocytoma during pregnancy is even rarer than unilateral pheochromocytoma. Case presentation: A 32-year-old primigravida, who was 12 weeks' pregnant, was aware of right abdominal discomfort. Masses in both adrenal glands were observed by abdominal ultrasonography. She was diagnosed with pheochromocytoma. Bilateral adrenalectomy was undertaken at 15 weeks' gestation and she continued pregnancy. At 39 weeks' gestation, a healthy male neonate was delivered. She was discharged on the 4th postpartum day. Conclusions: 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. Pheochromocytoma; Pregnancy; Adrenal tumor; Bilateral; Adrenalectomy - Background Pheochromocytoma is an uncommon disease that exhibits a variety of sympathetic symptoms by secreting catecholamines [1,2]. Pheochromocytoma may also occur during pregnancy [3,4]. The symptoms of pheochromocytoma are similar to pregnancy-induced hypertension [5]. When pheochromocytoma occurs in pregnancy, maternal and fetal mortality is increased [6-8]. Therefore, early diagnosis and appropriate treatment are important because of these high risks. Moreover, bilateral pheochromocytoma during pregnancy is even rarer [9]. We report here an additional case of bilateral pheochromocytoma that was diagnosed in the first trimester. Case presentation 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 35.7C 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, 2.304.00), 1.22 ng/dl (normal, 0.901.70), and 3.940 unit/ml (normal, 0.5005.000), respectively. Urinary albumin was negative. A 24-h urine sample demonstrated elevated metanephrine (14 mg/24 h; normal, 0.0050.20 mg/24 h) and normetanephrine levels (12 mg/24 h; normal, 0.100.28 Figure 1 MRI findings: MRI shows bilateral adrenal masses with central necrosis. The right mass was 4.9 4.4 4.2 cm and the left mass was 7.3 6.1 7.5 cm, which were compatible with bilateral pheochromocytoma. A tumor exhibiting relative non-uniform low signals on T1 MRI was observed in both adrenal glands. Transverse plane (a). Coronal plane (b). 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 patients 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 Figure 2 Surgical findings: Soft elastic tumor measuring 9 cm was palpable in the retroperitoneum at the inferior border of the pancreatic bo (...truncated)