FIRST EXPERIENCE WITH PROGNOSTIC FACTORS IN UNSELECTED NEUROBLASTOMA PATIENTS - THE AUSTRIAN NEUROBLASTOMA-87 STUDY

Between January 1987 and December 1993, 117 patients were registered i n the Austrian A-NB87 study. The male/female ratio was 1.18, with 50 p atients below the age of 1 year at diagnosis. Patients were assigned t o stage according to the result of primary surgery in localised diseas e. Age, ferritin and neuron specific enolase were used in addition in stage III disease for risk-adapted treatment. Adrenal or pelvic primar y tumour sites were mainly associated (81%) with advanced disease. The median observation time of the study is 3.5 years. The overall surviv al at 3 years was excellent in low stage disease and IVs patients, i.e . 100% for stage I and IIA (20 patients), 92% in stage IVs (13 patient s), 81% in stage IIIA (18 patients) and 69% in stage IIB (8 patients). Stage IV (38 patients) showed a survival rate of 51%, whereas stage I IIB (10 patients) had the worst outcome in this study, i.e. 20%, due t o treatment-related toxicity. Significant unfavourable prognostic fact ors were neuron specific enolase (NSE)> 100 ng/ml, ferritin > 300 mu g /ml and amplified MYCN. This study achieved a better survival rate in stage IV patients and a subgroup of stage III in comparison to our pre vious study (Padiatrie und Padologie 1986, 21, 269) and gives the basi s to further reduce treatment intensity in low-risk disease based on b iological factors. However, prognosis for high-risk cases was still po or in spite of a very aggressive treatment concept.