Adrenocortical Carcinoma: An Update

Adrenocortical carcinoma (ACC) is a rare tumor that affects about 0.72 persons per one million population.[1]. Only about 30% of these malignancies are confined to the adrenal gland at the time of diagnosis.[2]. Approximately 60 percent of ACCs are sufficiently secretory to present clinical syndrome of hormone excess. Adults with hormone-secreting ACCs usually present with Cushing s syndrome alone (45 percent), or a mixed Cushing s and virilization syndrome, with overproduction of both glucocorticoids and androgens (25 percent). Fewer than 10 percent present with virilization alone, but the presence of virilization in a patient with an adrenal neoplasm suggests an ACC rather than an adenoma.[3]Most patients with nonfunctioning tumors present with clinical manifestations related to tumor growth (ie, abdominal or flank pain) or with an incidentally found adrenal mass detected on radiographic imaging performed for a different reason; constitutional symptoms (weight loss, anorexia) are frequent in this clinical context. At initial presentation, approximately 50 percent of adult patients with ACC have relatively advanced disease stage.Based on the eighth edition of the TNM staging system and by the ENSAT (the European Network for the Study of Adrenal Tumors) classification, the five-year, disease-specific survival rates in 416 adult cases reported to the German ACC registry were [4]:●Stage I – Confined to the adrenal gland without local invasion or distant metastases; greatest tumor dimension ≤5 cm (T1N0M0): 82 percent●Stage II – Same as stage I but with tumor size > 5 cm without risk factors (T2N0M0): 61 percent●Stage III – Tumor of any size with at least one of the following factors: tumor infiltration in surrounding tissues (T3), tumor invasion into tumor thrombus in the vena cava or renal vein (T4), positive lymph nodes (N1) but no distant metastases: 50 percent●Stage IV – Distant metastases: 13 percent Recently, the European Society of Endocrinology (ESE) in collaboration with the ENSAT published a guideline for healthcare providers involved in the care of patients with ACC, i.e., endocrinologists, oncologists, surgeons, radiologists, nuclear medicine physicians, radio-oncologists, pathologists, specialists in general internal medicine and nurse specialists.[5]This lecture is a review of updated management of ACC based on the last published data in the literature.