Adrenocortical carcinoma (ACC) is a rare neoplasm with poor prognosis. Patients present signs of hormone excess: virilisation, Cushings syndrome or only enlarged abdominal mass. Incidentally ACC can be also detected in the ultrasonography. Some of non-hypersecretory ACCs can produce non-bioactive hormones steroid precursors or not very big amount of them and sometimes patients present subclinical Cushings symptoms. Surgery and adjuvant radiotherapy and chemotherapy with Mitotane is the treatment of choice.
Aim: The aim of the study was to analyse clinical features, hormonal test results and prognosis in patients with secreting and non- secreting adrenal cancers.
Patients: The course of disease of 22 patients: 6 men and 16 women median age 45.5 year was analysed. Clinical examination, the imaging studies and hormonal assays were performed. Seventeen patients underwent surgical treatment. Mitotane was administered in 16 patients.
Results: Virilization was diagnosed in 3 patients, overt or subclinical Cushings syndrome in 12 patients. Fourteen patients were classified as the I-st or II-nd stage, 8 as the III rd or IV th stage. The total and the asymptomatic survival time was longer in non-secreting patients than in secreting (53.86 vs 32.6 and 53.42 vs 13.9 months respectively). The secretory tumours were bigger than non-secretory (10.34 vs 6.15 cm). Due to ACC progress 10 patients died among which only one had non-secretory tumour. The mean time of observation was 13.7 months. Three patients with advanced disease and 9 patients with complete remission are still alive. In all patients with long-term remission, the mean diameter of tumour was 5.7 cm, and the mean time of observation is 76.6 months.
Conclusion: The poor prognostic factors in ACC are: size of tumour, presence of local and distant metastases and hormonal activity. Chemotherapy with mitotane prolong life of patients, but is less effective in advanced disease with excessive steroid hormone secretion.
03 - 07 May 2008
European Society of Endocrinology