Introduction: The adrenocortical carcinoma (ACC) is a primary malignant tumor developed in depends of the adrenal cortex, defined by a Weiss score ≥3. Despite the therapeutic progress, its prognosis is still severe.
Patients and methods: This retrospective descriptive study evaluated 4 patients with adrenal corticosteroid followed in the endocrinology department of Sfax - Tunisia between 2010 and 2018.
Results: There were 3 women and 1 man. The mean age at diagnosis was 48.7±13.5 years (34, 57, 63 and 41 years). The circumstances of discovery were, respectively: an adrenal incidentaloma, lumbar pain with cachexia, Cushings syndrome, and signs of hyperestrogenism in men. Clinical examination revealed respectively: asympotomatic, hirsutism, cushing syndrome and gynecomastia with decreased libido in men. The mean tumor size estimated by CT was: 13.5±5.7 cm. The evaluation of the extent of loco-regional recurrence and distant metastasis was positive in two cases with presence of liver and kidney metastases in one case, pulmonary liver metastases in an another case. All these patients were operated. The anatomopathological examination confirmed the diagnosis of adrenocortical carcinoma with a Weiss score ≥3 (from 3 to 9). In two cases, the tumor was classified stage II, for the rest it was classified stage III according to the classification of the ENSAT. In the postoperative period, one woman received a chemotherapy treatment and is currently on Lysodren. The clinical and biological evolution is good for two cases, stable for one case, dark for another case with appearance of secondary locations and peritoneal carcinomatosis.
Conclusion: The ACC is a rare agressive tumor with poor prognosis Therefore, every adrenal mass should be explored to not under estimate this fatal diagnosis. The standard treatment is surgery associated with Lysodren, to improve and increase survival.
18 - 21 May 2019
European Society of Endocrinology