We registered 170 relapses in 1392 children with nephroblastoma in the SIOP/GPOH trials. The study aimed to evaluate prognostic factors for outcome in relapsed patients. Age, gender, initial stage, metastatic disease, local stage, histology, time to relapse and tumour volume were analysed for their prognostic relevance. Overall survival after relapse was 48% (median follow-up 5 years). Relapses were local in 28%, metastatic in 57% and combined in 15%. The median age of the cohort was 4.5 years whereas patients in complete continuous remission were significantly younger (3.1 years, p=0.001). Patients with initial stage I and II showed a significantly better prognosis than children with stage III (57 vs. 31%, p=0.008). Patients with high-risk tumours had a much poorer prognosis than those with intermediate and low-risk tumours (58 vs. 31%, p=0.003). Children with recurrence within 6 months after diagnosis had a poorer outcome than children relapsing later on (54 vs. 22%, p=0.0001). The tumour volume initially and after preoperative chemotherapy did not have any influence on outcome. Patients with isolated distant metastasis had a significantly better outcome than those with local and combined relapses (p=0.001). In conclusion, factors for poor prognosis after relapse are early relapse, local stage III, high-risk histology and combined relapse.
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Pediatr Blood Cancer
April 2018
Kiel Pediatric Tumor Registry, Department of Pediatric Pathology, Christian-Albrechts-University Kiel, Kiel, Germany.
Background: Congenital mesoblastic nephroma (MN) is a rare pediatric renal tumor representing approximately 5% of all pediatric renal tumors. Three different types of MN are distinguished histologically: classical, cellular, and mixed. A frequent genetic alteration is the translocation t(12;15) resulting in a fusion of the ETV6 gene on 12p13 and the NTRK3 gene on 15p15 that occurs almost exclusively in cellular MN.
View Article and Find Full Text PDFKlin Padiatr
May 2014
Saarland University Hospital, Pediatric Hematology and Oncology, -Homburg/Saar, Germany.
Background: Treatment of stage V nephroblastoma is less established and more complex than in unilateral nephroblastoma.
Methods: Retrospective analysis of 121 consecutive patients with stage V nephroblastoma registered from January 1989 to May 2005. Registration, prospective data collection and treatment were carried out within the framework of 3 consecutive SIOP/GPOH-nephroblastoma-trials.
PLoS One
March 2011
In Silico Oncology Group, Institute of Communication and Computer Systems, School of Electrical and Computer Engineering, National Technical University of Athens, Athens, Greece.
The development of computational models for simulating tumor growth and response to treatment has gained significant momentum during the last few decades. At the dawn of the era of personalized medicine, providing insight into complex mechanisms involved in cancer and contributing to patient-specific therapy optimization constitute particularly inspiring pursuits. The in silico oncology community is facing the great challenge of effectively translating simulation models into clinical practice, which presupposes a thorough sensitivity analysis, adaptation and validation process based on real clinical data.
View Article and Find Full Text PDFStrahlenther Onkol
August 2009
University of Saarland, Hospital for Paediatric Oncology and Haematology, Building 9, 66421 Homburg/Saar, Germany.
Oncol Rep
August 2008
Asklepios Hospital, Pediatric Haematology and Oncology, D-53757 Sankt Augustin, Germany.
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