Background: Adrenocortical carcinomas (ACC) are rare malignancies with an incidence of one to two per million every year. 60% of these tumors are functional, most of which are Cushing’s syndrome. ACC’s are generally a postoperative diagnosis, often detected by immunohistochemistry. Aldosterone producing ACC’s (APAC) are extremely rare out of these with an incidence less than 7%.
Aim: To report a rare case of aldosterone producing ACC.
Materials and methods: 24 year old gentleman presented with episodic lower limb weakness with headache of 3 months duration and was referred to us.
Results: A young man presented to us with lower limb weakness with headache and was found to be normotensive on evaluation. He had a vague mass palpable in the right upper quadrant on palpation. On further investigation, he was found to have hypokalemia, which persisted despite serial corrections. A detailed biochemical evaluation revealed elevated aldosterone levels and the ratio of plasma aldosterone concentration to plasma renin assay. 24 hour urine fractionated metanephrines and cortisol were within normal limits. Contrast imaging of the abdomen showed a right adrenal mass of 9.7x7.7cm, which was lipid poor with decreased adrenal wash out, suspicious of adrenal malignancy. He underwent a right open adrenalectomy revealing a large right adrenal tumor of 12x10cm with no surrounding invasion. Postoperative histopathology was suggestive of adrenocortical carcinoma with a Weiss score of 3. Complete surgical resection normalized his potassium levels postoperatively.
Conclusion: Hypokalemia evaluation can be laborious and can lead us to rare diagnosis. Normotensive patients with persistent hypokalemia should raise a suspicion for adrenal tumors. Aldosterone producing adrenocortical tumors are a possibility to be remembered in the workup for adrenal tumors. Complete surgical excision is the cornerstone of treatment with long term follow up with imaging and hormonal levels.