Endocrine Abstracts

Introduction: Adrenocortical carcinoma (ACC) is an uncommon aggressive malignancy. Despite surgical resection, recurrence rates are high. Two areas of contention currently involve treatment of ACC: the role of laparoscopic surgery in removal of large, potentially malignant tumours and the role of Mitotane. We present a case of a large adrenal tumour, later confirmed as ACC, where initially laparoscopic adrenalectomy was considered, and adjuvant Mitotane has been given despite R0 resection.

Case: A 25 years lady presented with clinical features of Cushing’s syndrome. Cortisol levels failed to suppress with a low-dose dexamethasone suppression test and ACTH was undetectable (<5 ng/l). An adrenal MRI revealed a 10 cm well-circumscribed right adrenal tumour and no metastatic disease.

The departmental default position in the absence of overt signs of malignancy or technically excessive size is a laparoscopic adrenalectomy. However, with a large tumour size, IVC displacement and effacement, and sinister tumour washout characteristics on imaging, open adrenalectomy was deemed appropriate.

Within 8 weeks of initial imaging, open adrenalectomy revealed significant tumour growth and a 17 cm mass was removed in its entirety (macro and microscopic R0 resection). Histopathology showed ACC. Postoperatively, Mitotane treatment was initiated.

Postoperative CT abdominal scan at 4 months is clear of recurrence and metastatic disease. She feels well and plans to marry.

Conclusion: Laparoscopic adrenalectomy is the gold standard approach to small functioning adrenal tumours. Whilst the feasibility of laparoscopically resecting large adrenal tumours is established, the role of minimal access approach for such tumours is controversial due to risk of incomplete resection and capsular breach. This case demonstrates how a combination of clinical and radiological features guide in optimal surgical management of such tumours.

Although, adjuvant mitotane postoperatively remains controversial, a retrospective study showed reduced risk of recurrence and better survival in appropriately resected adrenocortical carcinomas followed by mitotane therapy.