ABSTRACT
INTRODUCTION: Nephroblastoma (Wilms tumor - WT) is the most common solid tumor of kidney in children. We present treatment development of WT at the Department of Pediatric Hematology and Oncology, Charles University in Prague, 2nd Faculty of Medicine and University Hospital Motol (KDHO) in the Czech Republic over 30 years. Patients that were treated prior to access to the International Society of Pediatric Oncology (SIOP) protocols are considered to be the historical group, then we have patients treated according to SIOP 9, SIOP 93-01 and SIOP 2001 protocols as full participants of SIOP studies. PATIENTS AND METHODS: Between January 1980 and April 2009, we treated 330 patients with WT at KDHO: 91 patients in historical group (1980-1988), 94 pts in SIOP 9 (1988-1993), 80 pts in SIOP 93-01 (1994-2001) and 65 pts in SIOP 2001 (2002-2009). Overall survival (OS) and event-free survival (EFS) were analyzed by Kaplan-Meier test. RESULTS: The overall ten-year EFS was 81.2% and OS 87.6%. Fifty-eight patients from the 330 (17.6%) had metastases at diagnosis, EFS without metastatic process was 84.6% compared to 65.4% with metastasis presented at diagnosis (p = 0.0003), OS was 70.7% compared to 91.2% (p < 0.0001). One hundred and seventy patients (51.5%) were treated with preoperative chemotherapy and/or radiotherapy, whereas 158 patients (47.5%) underwent primary nephrectomy; EFS and OS did not differ: neoadjuvant vs primary nephrectomy EFS was 81.2% vs 80.9% (p = 0.85), OS 89.4% vs 85.4% (p = 0.38). Sixty (18%) patients experienced disease recurrence; OS after relapse was 33%. In the historical group, EFS and OS were 85.7% and 91.2%. In patients treated according to the SIOP 9 protocol, EFS and OS were 68.1% and 74.5%, resp. In patients treated according to SIOP 93-01, it was 83.6% and 93.7%, resp. and in patients treated according to 87 SIOP 2001, it was 7% and 95.4% (p = 0.001 and p = 0.0008), resp. CONCLUSION: WT is a well treatable disease. The aim for the future is to maintain the current very good survival while minimizing the treatment intensity.
Subject(s)
Kidney Neoplasms/mortality , Wilms Tumor/mortality , Child , Female , Humans , Kaplan-Meier Estimate , Kidney Neoplasms/therapy , Male , Neoplasm Recurrence, Local , Prognosis , Treatment Outcome , Wilms Tumor/therapyABSTRACT
A new PXO set of RIS represents a fixed F2 population derived from polydactylous (P) congenic strain SHR.Lx and oligodactylous (O) RI strain BXH2. The PXO strains were derived as a complementary set to current RIS (HXB, BXH) of the laboratory rat. All PXO strains are homozygous in the Lx allele and express different morphological phenotypes of the polydactyly-luxate syndrome (PLS) due to variable combinations of Lx modifying genes of either SHR or BN origin. The SDP is being built up by genotyping polymorphic microsatellite markers and several gene polymorphisms. The markers were ordered according to data from public mapping resources such as the Rat Genome Database (rgd.mcw.edu) and current SDP of the other RI strain sets (HXB, BXH). The resulting map corresponding to the common SDP of HXB, BXH RIS sets consists of 448 markers, from which 261 were proven to be polymorphic in the PXO set. The SDP of PXO strains with polymorphic markers arranged in approximately 5 cM intervals is ready for the association analysis and interval mapping in interconnection with the SDP of HXB/BXH strains.
