[Malignant pheochromocytoma in childhood and adolescence--clinical case and review of the literature].

In regard to the diagnostic and therapeutic dilemma concerning rare neoplasms in childhood, the presented paper includes the comprehensive description of a malignant pheochromocytoma in a 14-year-old girl on the one hand and the detailed analysis of a collected series of 21 equal cases in pediatric literature on the other hand. In our patient there was in particular a lack of hypertension in spite of proven hypercatecholaminemia, an initially good effect of the post-operative aggressive chemotherapy according to the neuroblastoma study NBL 79 (4b) resulting in a transient reduction of the pulmonary metastases, furthermore a diminuation of the total tumour mass induced by the last applied treatment with the radiopharmaceutical 131I-metaidiobenzylguanidine. The analysis of 21 case reports revealed a peak between 10 and 15 years of age, a striking prevalence of the female sex in all age groups, and a high frequency of metastases (75%) at the time of diagnosis. Two thirds of the primary tumours arose in extra-adrenal sites, about one third occurred in the adrenal gland. The regional lymph nodes, the bone system and the lungs were the main locations of metastatic lesions. The association with a group of other conditions, particularly hypoxemic congenital heart malformations has proved to be a remarkable feature, but not only in the malignant forms.(ABSTRACT TRUNCATED AT 250 WORDS)