XXXX with multilayered rosettes in @entity1 : the SFCE experience.

@entity1 @entity6196 @entity26534 @entity4969 @entity5 @entity8125
PURPOSES: The purpose of this study was to retrospectively study @entity5 with multilayered rosettes (ETMR), a rare new entity that gathers ETAN-TR ( @entity5 with abundant @entity8125 ), @entity6196 , and @entity6196 , in order to improve their descriptions and try to better define therapeutic modalities. METHODS: @entity1 with ETMR, ETAN-TR, @entity6196 , and @entity6196 treated in SFCE centres (Soci  t   Fran  aise de lutte contre les Cancers et les leuc  mies de l'Enfant et de l'adolescent) since 2000 were collected. Data were retrieved from clinical charts. RESULTS: Thirty-eight @entity1 were included in the analysis. Seventeen had an ETAN-TR, 13 had a @entity6196 , and 8 had an ETMR. No @entity6196 was included. The median age at diagnosis was 31  months (range, 2.8-141  months). The predominant @entity5 location was supratentorial (66  %); 18.4  % @entity1 had metastatic lesion. @entity26534 expression was positive in 11/11 @entity1 . Amplification of the locus 19q13.42 was positive in 10/12 @entity1 . Thirty @entity1 were treated according to the primitive @entity4969 of high risk (PNET-HR) protocol. The median time of follow-up was 0.9  years (range 0.1 to 15.3  years). The 1-year event-free survival (EFS) and overall survival (OS) were, respectively, 36  % CI 95  % (23-55) and 45  % CI 95  % (31-64). On multivariate analysis, complete surgical resection, radiotherapy, and high-dose chemotherapy were associated with a better overall survival with a relative risk of, respectively, 7.9 CI 95  % (2.6-23.5) p  <  0.0002, 41.8 CI 95  % (9.4-186) p  <  0.0001, and 3.5 CI 95  % (1.3-9.5) p  =  0.012. CONCLUSION: Prognosis of ETMR remains dismal despite multimodal therapy. @entity26534 immunostaining and 19q13.42 amplification should be systematically done to secure the diagnosis. Complete surgical resection, radiotherapy, and high-dose chemotherapy are associated with better outcome.
