appropriate management of adrenal Cancer
Adrenocortical carcinoma (ACC) is a rare (1–2 per million) and aggressive cancer with a poor prognosis. At the time of diagnosis, approximately 25% of patients have nodal involvement, and 20% have distant metastases. Roughly 60% of ACCs are functioning tumors, and the mean size of tumors at the time of diagnosis is > 10 cm. The overall 5-year survival rate is around 25% and depends largely on the stage at diagnosis. Patients undergoing complete resection of small tumors (< 5 cm) without local invasion (stage 1) have a 5-year survival of 66%, whereas patients with metastases or invasion into other organs (stage 4) have a median survival of < 12 months. The only chance for cure is surgery, which should be offered to all patients who do not have metastases and who have a reasonable surgical risk. The operation for ACC should be performed with an open approach, and when a malignant tumor is suspected, a minimally invasive approach should be converted to an open approach. Surgery should also be considered for young patients with an isolated, easily resectable metastasis. Despite limited response rates, patients with stage 3 or 4 disease are frequently offered adjuvant therapy with mitotane (+/− cytotoxic chemotherapy) and/or radiotherapy because of the high recurrence rate (up to 85%). Mitotane is an adrenocorticolytic agent indicated for suspected incomplete resection and tumors with a high proliferative rate (Ki67 > 10%).
