ABSTRACT
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.
This article is available only as a PDF. Please click on “Download PDF” to view the full article.
