Introduction: At times, it may be difficult to differentiate early stage, low-grade adrenocortical carcinoma from benign adrenal adenoma. Clinical Picture: A 53-year-old lady underwent right adrenalectomy for a 4-cm adrenocortical tumour causing Cushing’s syndrome. Histology revealed an adrenocortical adenoma. Sixteen years later, she presented with a 14-cm adrenal tumour, again on the right side. Treatment: She underwent surgical removal of the tumour. Histology confirmed adrenocortical carcinoma. Outcome: She died of metastatic disease 17 months later. Conclusions: This case highlights the importance of long-term, systematic follow-up of patients treated for benign adrenal adenomas, especially if the tumour size exceeds 4 cm.
Adrenocortical carcinoma is a rare tumour, with an annual incidence of 0.5 to 2 per 1 million people. The overall 5-year survival rate ranges from 16% to 38%.1-4 Recurrence, even after seemingly complete resection, is common, occurring in 23% to 85% of patients.3,5,6 The reported mean disease-free interval ranges from 1.2 years to 2.4 years. 2,6 To the best of our knowledge, the longest reported interval to local recurrence following surgical resection of a non-functioning adrenocortical carcinoma has been 16 years.7 We describe a patient who presented with an ipsilateral adrenocortical carcinoma 16 years following adrenalectomy for a functioning right adrenal tumour that had resulted in Cushing’s syndrome, and discuss its implications on the management of seemingly benign adrenal tumours in clinical practice.
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