Bijnierschorscarcinoom bij kinderen

In this manuscript two case histories of children with adrenal carcinoma are described, followed by a review of the literature. Adrenal carcinoma is a very rare diagnosis in childhood, encomprising only 0.2 to 0.4% of all childhood malignancies. The delay in diagnosis is often long, even when symptoms of hormonal production are present. Discrimination between benign and malignant tumors is difficult on histological grounds; tumor size is the most important factor in predicting the metastatic spread and survival. Treatment of adrenal carcinoma consists of surgical resection. The experience with adjuvant chemotherapy in childhood adrenal carcinoma is very limited. Based upon the limited amount of data, mitotane is the drug of first choice in case of metastasis. The role of radiotherapy seems limited. The overall survival rate is about 50%.