Summary:
The article reports on eight children with adrenal cortex carcinoma. The most frequent symptom was androgenous adrenogenital syndrome-. in six children, in one the tumour manifested itself by Cushing's syndrome and in another it was endocrinologically mute and manifested itself by acute abdominal symptoms. Various diagnostic possibilities are discussed. Ultrasonography, possibly supplemented by a CT scan, appears as most convenient. A comprehensive endocrinological examination is indicated only in case of diagnostic uncertainty. Long-term monitoring of urine 17-hydroxysteroids and 17-keto-steroids is of great importance for early detection of relapse and metasthases. Operation is number one therapeutic measure. Possibilities to treat generalisation are very limited. There can be much better results than reported in literature if diagnosis is made at an early stage and adequate treatment is speedily introduced. In our set of eight patients there was only one case of death.