Adrenal adenocarcinoma rarely presents with hyperaldosteronism (Conn’s syndrome), which usually presents as chronic hypertension with hypokalemia. In severe cases of hyperaldosteronism, profound weakness with bizarre aches and pains may occur acutely.
A 29-year-old woman presented to the emergency department with severe malaise, profound weakness and nausea plus chest and abdominal pains of fleeting duration. Investigation to rule out biliary tract disease included ultrasonography, which revealed a large left upper abdominal mass. Subsequent CT (Figs 1 and 2) and MRI demonstrated a massive adrenal tumour extending through the left renal vein into the vena cava and upward to the heart. Laboratory investigations demonstrated severe hypokalemia and a high serum aldosterone level with low renin levels.
Surgical management consisted of a sternotomy and abdominal approach with the aid of cardiopulmonary bypass to remove the entire mass, including the left kidney and intracaval extension of the tumour thrombus to the right atrium (Fig. 3). Her recovery was uncomplicated. Management has continued with observation and chemotherapy, although a metastatic pulmonary lesion has developed.
Adrenocortical adenocarcinoma is a lethal disease, requiring aggressive resection if possible. Chemotherapy and radiotherapy have given disappointing results, so recurrent disease is best managed by repeat resection also, if feasible. Unresectable disease has a uniformly poor outcome.
Footnotes
Section Editors: David P. Girvan, MD, and Nis Schmidt, MD
Submissions to Surgical Images should be sent to Dr. David P. Girvan, Victoria Hospital Corporation, PO Box 5375, Station B, London ON N6A 5A5 or to Dr. Nis Schmidt, Department of Surgery, St. Paul’s Hospital, 1081 Burrard St., Vancouver BC V6Z 1Y6, with a copy of the submitting letter to Dr. Jonathan L. Meakins, Rm. S10.34, Royal Victoria Hospital, 687 Pine Ave. W, Montreal QC H3A 1A1.