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High-dose treatment for malignant rhabdoid tumor of the kidney: No evidence for improved survival-The Gesellschaft für Pädiatrische Onkologie und Hämatologie (GPOH) experience
- Source :
- Pediatric Blood & Cancer. 65:e26746
- Publication Year :
- 2017
- Publisher :
- Wiley, 2017.
-
Abstract
- Background Malignant rhabdoid tumor of the kidney (MRTK) is the most aggressive childhood renal tumor with overall survival (OS) rates ranging from 22% to 42%. Whether high-dose chemotherapy with autologous stem-cell transplantation (HDSCT) in an intensive first-line treatment offers additional benefit is an ongoing discussion. Methods A retrospective analysis of all 58 patients with MRTK from Austria, Switzerland, and Germany treated in the framework of consecutive, prospective renal/rhabdoid tumor studies SIOP9/GPO, SIOP93-01/GPOH (where SIOP is International Society of Pediatric Oncology and GPOH is German Society of Pediatric Oncology and Hematology), SIOP2001/GPOH, and European Rhabdoid Tumor Registry from 1991 to 2014. Results Median age at diagnosis was 11 months. Fifty percent of patients had metastases or multifocal disease at diagnosis (Stage IV). Local stage distribution was as follows: not done/I/II/III—1/6/11/40. Fifteen (26%) patients underwent upfront surgery. Thirty-seven (64%) patients achieved a complete remission, 17 (29%) relapsed, 34 (59%) died of disease progression, and two (3%) died of treatment-related complication. Mean time to the first event was 3.5 months. Two-year EFS/OS (where EFS is event-free survival) for the whole group was 37 ± 6%/38 ± 6%. Metastases/multifocal disease, younger age, and local stage III were associated with significantly inferior survival. Eleven (19%) patients underwent HDSCT (carboplatin + thiotepa, n = 6; carboplatin + etoposide + melphalan, n = 4; others, n = 1); 2-year OS in this group was 60 ± 15% compared to 34 ± 8% in the non-HDSCT group (P = 0.064). However, the time needed from radiologic to histologic diagnosis, stem-cell harvest, and HDSCT must also be taken into account to avoid selection bias by excluding the highest risk group with early progression (
- Subjects :
- Male
0301 basic medicine
Malignant rhabdoid tumor
medicine.medical_treatment
2720 Hematology
Pediatrics
Gastroenterology
0302 clinical medicine
Antineoplastic Combined Chemotherapy Protocols
Medicine
Prospective Studies
Registries
Stage (cooking)
Child
Kidney
Hematology
Age Factors
Perinatology
Kidney Neoplasms
and Child Health
Survival Rate
medicine.anatomical_structure
Oncology
Vincristine
Child, Preschool
030220 oncology & carcinogenesis
Dactinomycin
2730 Oncology
Female
medicine.medical_specialty
Adolescent
610 Medicine & health
Disease-Free Survival
03 medical and health sciences
Internal medicine
Humans
2735 Pediatrics, Perinatology and Child Health
High dose treatment
Rhabdoid Tumor
Chemotherapy
business.industry
Infant, Newborn
Infant
Surgery
Transplantation
030104 developmental biology
10036 Medical Clinic
Pediatrics, Perinatology and Child Health
business
Complication
Subjects
Details
- ISSN :
- 15455009
- Volume :
- 65
- Database :
- OpenAIRE
- Journal :
- Pediatric Blood & Cancer
- Accession number :
- edsair.doi.dedup.....056027e34ce0b67a7653b105b241e91e