Phaeochromocytoma in pregnancy is an extremely rare condition. Early recognition can largely decrease maternal and fetal mortality rates. As symptoms of phaeochromocytoma are similar to those of other more common causes of hypertension during pregnancy, timely diagnosis is a challenge. There is no consensus in literature about the specific treatment nor about the optimal delivery timing or route. Case of a 32 years old woman with a single pregnancy of 30 weeks and 3 days admitted in an obstetrics department because of a paroxistic hypertension. She had been asymptomatic until the first trimester of gestation when she started with paroxysmal episodes of migraine, palpitations and precordial pain associated with hypertensive crises (systolic blood pressure 190-200 mmHg). The patient had no relevant personal medical history except a previous pregnancy and delivery without complications. Proteinuria was absent, therefore excluding a preeclampsia. Renal ultrasound showed no renal asymmetry or vascular alterations but identified a heterogeneous 44×42×41 mm mass in the left adrenal gland. Abdominal MRI confirmed the 40 mm nodule in the left adrenal gland with hyperintense and homogeneous central area in T1 and T2 and solid peripheral area hypointense in T1 and hyperintense in T2. Normal Echocardiogram. 24h hölter: sinus rhythm and multiple periods of sinus arrhythmia. Ambulatory blood pressure monitoring: non dipper profile. Fetal ultrasound: normal fetal circulation and fetus in centile 36. Analytically: metanephrine (urine): 27 ug/24h (64301 ug/24h) and normetanephrine (urine): 4086 ug/24h (162527 ug/24h). Diagnosis of phaeochromocytoma was established. She was initially medicated with nifedipine 30mg 2id and, at the 33rd week the patient started alpha-blockade and at the 34th week beta-blockade. We chose fenoxibenzamin (maximum 30 mg/day) and propranolol (30 mg/d) respectively. A Caesarean-section was performed at 37 weeks of gestation. Mother and child had no complications. Specifically the child had no hypotensive period. After delivery the patient kept fenoxibenzamin 40 mg/d and propranolol 30 mg/d until the left laparoscopic adrenalectomy was performed (9 days after delivery). The surgery ran without intercurrences and she stopped antihypertensive medication with good tolerance and stable arterial blood pressure. The histology confirmed a phaeochromocytoma with a PASS score of 4. One month after surgery the patient had normal plasmatic values of metanephrine (13.9 pg/ml <65) and normetanephrine (82.3 pg/ml<196). Phaeochromocytoma during pregnancy is a rare condition and as such should be treated in specialized centers with a multidisciplinary team. Important issues that need special attention include pretreatment, timing of surgery and route and timing of delivery.
18 - 21 May 2019
European Society of Endocrinology