Case Presentation:
A 66 year‐old man presented for evaluation of severe back pain, fatigue and lower extremity weakness for one month. Further questioning elucidated a two month history of worsening dyspnea both with exertion and at rest, orthopnea, paroxysmal nocturnal dyspnea and lower extremity edema. Review of systems revealed a 14 kg weight gain in one month, new‐onset urinary incontinence and persistent hypokalemia despite aggressive daily supplementation. Eight years prior, right adrenalectomy was performed for an incidental 5 cm tumor. Pathology was reportedly benign. Physical examination was remarkable only for obesity, multiple purple abdominal striae and profound bilateral lower extremity edema. Laboratory studies were notable for hypokalemia (2.6 mEq/L). CT scan of the abdomen revealed a 9.4 × 6.7 cm heterogeneous mass and small satellite nodules in the liver, along with smaller masses in the right adrenal fossa and behind the liver causing IVC compression. Ultrasound‐guided needle biopsy of the liver revealed metastatic adrenal cortical carcinoma. Concurrent with the liver biopsy, serum corticotropin level was decreased, morning cortisol and plasma renin activity elevated, and aldosterone level normal. 24hour urine testing revealed elevated free cortisol, cortisone, and aldosterone. Subsequently, the patient's right adrenal surgical tissue was obtained and showed histologic findings identical to that of the metastatic liver mass.
Discussion:
Incidental adrenaloma is a common finding on imaging studies. The distinction between benign and malignant tumors is generally based on size, radiographic characteristics and histologic features. Tumor morphology is the best predictor of metastatic risk, but even after adequate biopsy, the distinction remains difficult. Due to greater risk of malignancy, all tumors larger than 5 cm should be surgically resected, even if not hormonally active. Adrenal cortical carcinoma is rare, with an incidence of 1‐2 cases per million per year. Presentation is often delayed and is varied due to differing hormonal effects and mass effects. Despite surgical resection, which is the only curative option, nearly 80% of patients have recurrence. There is no data to suggest that repeated imaging after resection can detect recurrent malignancy at a resectable stage. In this case, the recurrence occurred eight years after adrenalectomy. The patient died two months after discharge.
Conclusion:
Our case highlights two important principles of inpatient medicine: the importance of reviewing outside pathology slides, even when the biopsy is remote and pathology is presumed to be favorable, and the importance of keeping rarer tumors, such as adrenal cortical carcinoma, in the differential diagnosis of patients with evidence of hormonally‐active adrenal masses.
Author Disclosure Block:
D. Roberts, None; A. Bacani, None.