Unexpected Discovery: A Case of Adrenocortical Carcinoma

Introduction: Adrenocortical carcinoma (ACC) is a rare disease that could present in an unusual pattern. We present a case of a patient with rapid onset, severe hypokalemia and worsening blood pressure (BP) leading to diagnosis of ACC. Case Description: 67-year-old female, with history of hypertension (well controlled on Amlodipine 5mg daily) presented with gradual onset generalized muscle weakness for 10 days. Her blood pressure (BP) had been poorly controlled over the past 2 weeks and was 180/110 mmHg at presentation. She denied drug abuse, diuretics, or liquorice use. Lab testing (image 1) revealed severe hypokalemia with EKG changes (elongated QT interval and U wave). She was managed medically and discharged on oral potassium supplements in addition to multiple BP medications (Telmisartan 80 mg, Amlodipine 10 mg, Spironolactone 25 mg). Further evaluation in the office revealed elevated Aldosterone/PRA ratio (image 1) raising suspicion for primary hyperaldosteronism (PHA). A CT of the abdomen with adrenal protocol revealed a lobulated right adrenal nodule with adenomatous thickening. Adrenal venous sampling lateralized to the right adrenal gland, and she was referred for a laparoscopic right adrenalectomy. Adrenal gland histology revealed a high grade myxoid ACC. The patient was started on cisplatin, etoposide and mitotane. Discussion: ACC is a rare and potentially aggressive malignancy. The incidence of ACC worldwide is 0.5 to 2 per 1 million people annually. It could present with hormonal changes from hyperaldosteronism, hyperandrogenism or hypercortisolism, non-specific symptoms or as an incidental finding. It could also be part of some familial cancer syndromes. A high index of suspicion is therefore needed when it presents in an unusual manner such as was seen in our patient with rapid onset hypokalemia and worsening HTN suggestive of hyperaldosteronism. Treatment is with surgery and adjuvant chemotherapy/radiation therapy. It has a high risk of recurrence.