TREATMENT AND PROGNOSIS IN 11 ADULTS WITH WILMS-TUMOR (NEPHROBLASTOMA)

11 adults with a histologically proven diagnosis of Wilms' tumour at age 17 - 52 years (average 27.4 years; median 27.5 years) and first treated between 1980 and 1990 are reported from eight hospitals. 7 of the cases were histologically subtyped: 5 were of the ,,standard type" (,,low-risk"-morphology), one had the anaplastic-sarcomatous (,,high- grade") variant and one was a cystic nephroma. Staging and treatment were done according to actual pediatric Wilms' tumour protocols. 3 patients had stage 1, 3 stage II a, 2 stage III and 2 stage IV disease; the cystic nephroma was stage I. Radical tumour operations were possible in 9 / 11 patients (stage I-III); in 2 stage III-patients after preoperative chemotherapy. Two patients did not receive chemotherapy (one stage I standard type and concomitant tuberculosis of the lungs; one cystic nephroma). Nine patients had chemotherapy with either vincristine + actinomycin D (VCR + ACD) or ACD + VCR + adriamycin (ADR) or additional ifosfamide or cyclophosphamide. 6 patients received local radiotherapy (30-40 Gy). After a follow-up of 1-7 years, 4 patients have died (3 because of tumour and/or metastases after 4-16 months, one of cardiomyopathy 7 years after first treatment), and 7 are alive. Of the 7, 5 are recurrence-free after 2-6 2/12 years, one after successful treatment of a solitary liver metastasis one year later, and one patient is surviving her stage IV-disease one year after diagnosis and treatment initiation with possible residual disease. This is the first comprehensive report on Wilms' tumours in adults from Germany. Based on previous experience drawn from case reports and the literature, it can be concluded that the treatment and prognosis of this rare malignancy in adults has improved remarkably throughout the past years using pediatric Wilms' tumour protocols. Long-term disease-free survival in 50 % of the cases is a realistic goal of treatment at the current time.