XXXX --a report from the Gesellschaft fur P  diatrische Onkologie und H  matologie (GPOH).

@entity1 @entity5 @entity12878 @entity3 @entity16557 @entity3969 @entity703
BACKGROUND: Surgery alone is the appropriate first-line treatment for @entity1 with @entity12878 ( @entity12878 ). Nevertheless, there are reports of local recurrences and @entity3 , especially in the cellular subtype. The authors evaluated the outcome of @entity1 with @entity12878 who were enrolled in either the International Society of Pediatric Oncology (SIOP) 93-01/GPOH or the SIOP 2001/GPOH @entity3969 Study and Trial. METHODS: In total, 50 @entity1 with @entity12878 were analyzed. Eleven @entity1 were suspected antenatally of having a @entity703 . The median age at diagnosis was 18.5 days. Central pathologic review was performed for all specimens. The median observation time was 4.2 years. RESULTS: Forty-five @entity1 underwent initial surgery. Five @entity1 older than 6 months received preoperative chemotherapy. Twenty-nine @entity5 were classic @entity12878 , and 21 @entity5 were cellular @entity12878 . Nine @entity1 had a Stage III @entity12878 , 5 of those @entity1 had @entity5 ruptures, and 8 had positive surgical margins. After they underwent nephrectomy, 40 @entity1 received no further treatment. For the entire group, event-free survival (EFS) (94%) and overall survival (OS) (95%) were excellent. @entity1 with a cellular @entity12878 , @entity1 with age 3 @entity16557 , and @entity1 with Stage III @entity12878 had lower EFS. Three @entity1 developed recurrent disease, and 2 of those @entity1 died. @entity3 to the brain, lung, and liver were observed in 1 @entity1 . CONCLUSIONS: Radical nephrectomy with accurate surgical-pathologic staging is the standard of care for @entity1 with @entity12878 . Nonetheless, a subgroup of @entity1 with @entity12878 (Stage III cellular @entity12878 in @entity1 age 3 @entity16557 ) tends to develop recurrences more often. Further prospective studies will be needed to verify this finding and should help determine whether these @entity1 may benefit from adjuvant therapy.
