Résumé
Adrenocortical carcinoma is a rare malignant endocrine tumor which accounts for about 0.05% to 0.2% of all carcinomas. Three clinical patterns can be encountered. In 30% of the cases, a mass syndrome without any clinical evidence of hypersecretion is presented as abdominal pain, a palpable abdominal mass, abdominal distension, and weight loss. In 60% of the cases, an overt clinical syndrome of hypersecretion is of almost purely hypercortisolism in 30% of such cases, vilirization in 22%, feminization in 10%, hyperaldosteronism in 2.5%, and a mixed secretion in 35%. In the remaining 10% of the cases, an adrenal `incidentaloma' is found incidentally during evaluation of the other disease. About 70% to 80% of patients are diagnosed with an advanced stage (III or IV) and metastasis has occured in 20% to 40% of the patients at the time of presentation. Early surgery an with adrenalectomy is the only means of cure. The prognosis is poor with a 5-years survival rate of 16% to 34% due to initial diagnosis at an advanced stage. Recently, we experienced two cases of adrenocortical carcinomas. In case I, a 62-year-old female patient presented with a clinical syndrome of hypercortisolism and had an extensive local invasion of stage IV; in case II, a 49-year-old male patient complained of abdominal pain and distension without any clinical syndrome of hypersecretion and had stage IV liver metastasis.