Spontaneous Rupture of Adrenal Pheochromocytoma

Abstract

A 39-year-old woman presented with a 2-year history of frequent episodes of headache, nausea and palpitations. Blood pressure had generally been stable but paroxysmally increased to 180/110 to 210/130 mm. Hg. At hospitalization serum adrenaline was 1,768 pg./ml., serum noradrenaline was 3,449 pg./ml., urine adrenaline was 1,170 g. per day and urinary vanillylmandelic acid was 31.5 mg. per day. Computerized tomography (CT) showed a left adrenal mass (fig. 1, A), and scintigraphy with iodine metaiodobenzylguanidine revealed a high accumulation in the mass. Based on these data left adrenal pheochromocytoma was diagnosed, and 0.5 mg. doxazosin mesylate was started to increase circulatory plasma volume. Subsequently, the patient experienced attacks that were more frequent and severe than before (fig. 2). Severe back pain, palpitations and vomiting persisted for a few minutes several times a day, and she was prescribed sublingual nifedipine and kept on bed rest. Two days after starting -blockade, the patient complained of sudden unbearable pain in the back and chest. Blood pressure increased to 300/180 mm. Hg and pulse rate was 140 beats per minute. Under the diagnosis of hypertensive crisis 5 mg. phentolamine was administered intravenously, and blood pressure decreased to 100/60 mm. Hg. Despite the blood pressure being well controlled, severe pain had steadily progressed from the time of onset. Ruptured pheochromocytoma was diagnosed by CT, which demonstrated a large hematoma in the right retroperitoneal space (fig. 1, B). After cardiac function was checked by electrocardiogram and ultrasonography, 4 units blood was transfused and volume supplementation was performed, while vasodilator phentolamine was administered under continuous monitoring of central venous pressure and direct blood pressure. Left adrenalectomy and drainage of the retroperitoneal hematoma were performed 7 hours after the onset of spontaneous rupture. The patient was placed under general anesthesia after good urine output was confirmed. A hemi-chevron incision was made and transperitoneal adrenalectomy was performed. Blood pressure was maintained at 90/50 to 110/80 mm. Hg and stabilized with phentolamine and alprostadil during the procedure. Hematoma volume was approximately 1,000 gm. and operative time was 120 minutes. Pathological diagnosis was adrenal pheochromocytoma (4 4 5 cm.). A laceration was found between the adrenal gland and tumor. Postoperatively blood pressure normalized without medication, and serum and urine catecholamines returned to normal.