@entity5 of the kidney (MRTKs), registered on recent SIOP protocols from 1993 to 2005: a report of the XXXX study group.

@entity1 @entity358 @entity5 @entity703
BACKGROUND: Survival data of @entity703 ( @entity703 ) registered in SIOP trials, advocating preoperative chemotherapy, are not available. AIM: To evaluate characteristics, response and survival of @entity703 @entity1 registered in recent SIOP protocols. METHODS: An evaluation of all @entity703 @entity1 treated from 1993 to 2005 (SIOP trials 93-01 and 2001) was performed. Data were obtained from study specific case record forms and entered centrally in a database. RESULTS: Hundred and seven @entity1 were identified (57 male), with a median age at diagnosis of 13 months (interquartile range 6-27 months), and a median follow-up time of 60 months. Left and right kidneys were equally affected. @entity5 stage distribution was stage I (6%), stage II (22%), stage III (43%), stage IV (22%) and stage V (3%). Stage IV @entity1 included 17 with @entity358 (8 lung-only) and 12 with multiple organ metastases (bone, brain and liver). Primary surgery was the upfront treatment approach in 22/107 @entity1 (21%), by which 19 @entity1 reached a complete remission (CR). Median difference in @entity5 volume before and after preoperative chemotherapy was 69 ml (interquartile range: 4.5-158.0, P < 0.0001), indicating marked chemosensitivity. The 5-year event-free survival (EFS) of the total group was 22% (95% CI: 15-33) and overall survival 26% (95% CI: 18-37). Most events (86%) occurred within the first 2 years after diagnosis. Younger age at diagnosis was an important adverse prognostic factors for survival. In contrast, @entity5 volume at diagnosis, nor volume reduction was associated with outcome. CONCLUSION: @entity703 has a poor outcome especially in young and advanced-stage disease @entity1 . Neither @entity5 volume at diagnosis, nor pre-operative chemosensitivity are prognostic factors for survival.
