A 23-year experience with malignant renal tumors in infancy and childhood

A retrospective analysis of 77 children treated between 1974 and 1996 was u ndertaken to evaluate morbidity and the evolution of therapy. A Wilms' tumo r (WT) was present in 73 children. 74% of patients (pats.) with WT survived (54 of 73 pats.). Histological specimens of 67 patients were re-evaluated, including 4 children with non-WT histology. Among patients with Wilms' tum ors (WT), nephroblastoma (NB) of intermediate risk predominated (73 %; 46 o f 63 pats.). Low-risk tumors occurred in 5 of 63 children (8%; mesoblastic nephroma 3, cystic partially diff. NE 1, completely necrotic NE ?). High-ri sk WT were diagnosed in 12 of 63 patients (19%) (NB with anaplasia 10, clea r cell sarcoma 1, malignant rhabdoid tumor 1). Nephrogenic rests were prese nt in 14 cases. We observed 3 children of school age with renal carcinoma a nd one patient with an intrarenal neuroblastoma. WT histology was the most important factor determining prognosis (p=0.018). The risk for relapses was 2.6-fold higher in patients with high-risk WT compared to the standard ris k group. Stages were re-evaluated according to SIOP 93-01. Comparing relaps e-free survival of stages I, II and iii, respectively, there was a reduced survival rate for stage III (p=0.019). According to the SIOP/GPOH protocol in 1989, the regimen was switched from primary surgery to preoperative chem otherapy without biopsy in 1989 (If pats.). Compared to earlier years, surv ival improved (n.s.). In 3 patients preoperative diagnosis by means of imag ing failed. During preoperative chemotherapy a venous occlusive disease of the liver occurred in 2 patients. Preoperative chemotherapy led to an impre ssive tumor shrinkage in the majority of patients, 2 patients of the preope rative group died (focal anaplastic MB). Long-term morbidity was analysed i n 49 patients and included radiation-induced scoliosis (35), chest-wall def ormity (3), congestive cardiomyopathy after relapse (1) and arterial hypert ension (2). Over the years there was a trend to reduce frequency and dose o f irradiation. Prognosis of WT is excellent but unfavorable histology thigh risk) predicts a poor prognosis. in our experience, reduction of tumor volume due to preo perative chemotherapy facilitates tumor removal by surgery and may prevent tumor spillage and the deleterious effects of radiation in young children. Surgery without delay is necessary if the diagnosis is unclear or the tumor fails to respond to preoperative chemotherapy.