Adrenal cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Prognostic Role of Overt Hypercortisolism in Completely Operated Patients with Adrenocortical Cancer

“…Adrenocortical carcinoma (ACC) is a rare tumor characterized by a dismal prognosis with less than 50% of patients surviving more than 5 years after diagnosis (1). Complete surgical resection of ACC offers the best chance for prolonged survival, particularly in patients diagnosed at an early stage and with low proliferating tumors (1); however, a significant number of patients without objective and biochemical evidence of residual disease after surgery are destined to relapse (2-4).…”

Aldosterone-Producing Adrenocortical Carcinoma with Co-Secretion of Cortisol and Estradiol: A Case Report

“…Due to the variable hypercortisolemia and the rapid development of adrenocortical carcinoma, clinical features of Cushing's syndrome are often incomplete or even missing (atypical or subclinical Cushing's syndrome). 8 Preoperative preparation therefore entails glucocorticoid coverage in patients with Cushing's or subclinical Cushing's syndrome. Preparation is also needed for patients with tumors secreting metanephrines, therefore it is important to exclude a pheochromocytoma prior to surgery.…”

“…A Ki67 index more than 10% indicates high-risk adrenocortical carcinoma and would require more aggressive treatment after surgery such as adjuvant therapy with mitotane. 8 The patient's tumor had a Ki67 index of 5-10% which is classified as low-risk adrenocortical carcinoma.…”

“…8 The TNM classification is used in the assessment of disease stage. Stages I and II are described as localized tumors with a size of 5 cm or smaller and larger than 5 cm, respectively.…”

“…Laparoscopic adrenalectomy may be done in patients with small tumors of less than 8 cm without preoperative evidence for invasiveness and suspected to be only potentially malignant. 8 For patients with complete tumor resection, regular follow-up every 3 months with abdominal and thoracic CT scans and monitoring of initially elevated hormones are recommended. Interval follow-up may then be increased after 2 years and should be continued for at least 10 years thereafter.…”