Adrenocortical pheochromocytoma diagnosed during pregnancy: a case report

Gestational hypertension, a common complication of pregnancy, remains one of the leading causes of maternal and perinatal fetal and neonatal mortality, which seriously compromises the health of mothers and infants [1, 2]. The etiology of very few patients is related to adrenal tumors. Adrenocortical carcinoma (ACC) is a rare malignant tumor with an incidence rate of about one in a million [3]. ACC is characterized by strong invasiveness, rapid disease progression, and poor prognosis. A large proportion of ACC patients may present local infiltration or distant metastasis at the time of diagnosis. Further, ACC in pregnancy is rather rare, and there are few reports on ACC in pregnancy.

A 26-year-old, gravida 2, para 1 (G2P1) woman at the 31st week of gestation was admitted to the hospital with a chief complaint of elevated blood pressure for 3 months. The woman occasionally suffered from dizziness and amaurosis when changing her posture during pregnancy. Regular antenatal examinations were performed, and there was no sweating or other special discomfort during pregnancy. The oral glucose tolerance test (OGTT) revealed 5.46-8.76-8.42mmol/L, and the patient was diagnosed with gestational diabetes mellitus and given diet and exercise interventions, but no regular blood glucose monitoring. The blood pressure was 106/78 mmHg at the 7th week of gestation, 127/94 mmHg at the 16th week of gestation, and 149/100 mmHg at the 25th week of gestation. However, the patient did not pay attention enough attention to it and also did not receive further diagnosis and treatment. Palpitation and chest tightness occurred once at 31 weeks + 2 days of gestation. The next day, the patient felt headache and dizziness, without blurred vision, and her blood pressure was 161/112 mmHg. Therefore, the patient was transferred to the local hospital. The presence of a solid mass in the right-side adrenal region was identified during the admission obstetric ultrasound evaluation. The Fig. 1 resulted to be highly suggestive of an adrenal pheochromocytoma. The woman presented with severe hypertension (164/109 mmHg), therefore, hydrochloride 100 mg (twice a day, po) was started twice a day. A course of corticosteroids for fetal lung maturation was also prescribed taking into account the high chance of premature delivery (31 weeks + 6 days of gestation). Considering the suspicion of pheochromocytoma and the presence of a severe hypertension the patient was referred to out tertiary obstetric care unit. At 33 weeks + 2 days of gestation, the patient was given detemir insulin hypoglycemic treatment due to poor blood glucose control. After admission, the patient had no dizziness, headache, chest tightness, sweating, abdominal distension, abdominal pain, vaginal bleeding, or other special discomforts. The patient’s vital signs, blood sugar, and consciousness symptoms were monitored, and relevant auxiliary examinations were carried out. The results of the blood biochemical examination are shown in Table 1. For imaging examinations, B-ultrasound of the urinary system indicated a cystic and solid space-occupying lesion in the right adrenal gland, considering the possibility of pheochromocytoma (Fig. 1); The third trimester growth scan has shown a fetus of 2095 g±, with a mild polihydramnios (deepest vertical pocket of amniotic fluid of 10.5 cm); magnetic resonance (MRI) of the urinary system showed a mass in the right adrenal gland with necrosis and hemorrhage, and pheochromocytoma was considered (Fig. 2); MRI of the brain showed that the pituitary gland was plump, and no other obvious abnormality was found (Fig. 3). Multi-disciplinary consultation was conducted to evaluate the patient’s condition comprehensively, and it was recommended to terminate the pregnancy at the 34th week of gestation after her condition gradually stabilized. After maternal hypertension and glucose control was obtained at 34 weeks + 1 day, considering her physical condition and disease state, in order to reduce the risk of continuing pregnancy, the patient and their family required an elective Caesarean section. No complication occurred during surgery and a healthy newborn was delivered. After the operation, the patient continued to receive nifedipine controlled-release tablets 30 mg (one a day, po) and labetalol 150 mg (once every 8 h, po) to control the blood pressure between 128–136/85–93 mmHg and also received infection prevention, blood pressure reduction, and symptomatic treatment. Five days after cesarean section the patient was discharged from the hospital with the stable condition, and she was instructed to continue to take oral nifedipine controlled-release tablets and labetalol to control blood pressure.

Two months after delivery, the patient underwent surgery of “excision of the right adrenal gland and space-occupying lesion + partial resection and repair of vena cava + stripping of right renal capsule” in our hospital. During the operation, a tumor about 10 cm in size was seen in the right retroperitoneum, with a fixed, position and hard texture, which was closely related to the right liver, kidney, and retrohepatic inferior vena cava. The tumor tissue was sent for pathological examination after the operation, and the results indicated an adrenal space-occupying grayish red nodular mass (10*7.5*7.5 cm), and the cut surface was grayish red and grayish yellow with medium texture, accompanied by hemorrhage and necrosis. A histopathologic description of cells is arranged in an acinar shape. (Fig. 4). Immunohistochemistry confirmed the diagnosis of right adrenocortical carcinoma, inhibin(+), Ki67(+, 40%), Vimentin(+) and demonstrated positive results for intravascular cancer embolus and capsule invasion (Fig. 5, Fig. 6 and Fig. 7).

Considering the patient’s condition, obstetrics and urology department recommended chemotherapy. After 3 months of follow-up, she has completed chemotherapy at another hospital. During the follow-up, she survived with no clinical evidence of disease recurrence.