Embryonal tumors with multilayered rosettes in children: the SFCE experience - Archive ouverte HAL Access content directly
Journal Articles Child's Nervous System Year : 2016

Embryonal tumors with multilayered rosettes in children: the SFCE experience

(1) , (2) , (3) , (4) , (5) , (6) , (1) , (7) , (8) , (9) , (1) , (10) , (11, 1) , (1) , (12)
1
2
3
4
5
6
7
8
9
10
11
12
Meryl Horwitz
  • Function : Author
  • PersonId : 981478
Christelle Dufour
  • Function : Author
  • PersonId : 981479
Anne-Isabelle Bertozzi
  • Function : Author
  • PersonId : 981481
Didier Scavarda
  • Function : Author
  • PersonId : 981482

Abstract

Purposes The purpose of this study was to retrospectively study embryonal tumors with multilayered rosettes (ETMR), a rare new entity that gathers ETAN-TR (embryonal tumor with abundant neuropil and true rosettes), ependymoblastomas, and medulloepitheliomas, in order to improve their descriptions and try to better define therapeutic modalities. Methods: Patients with ETMR, ETAN-TR, ependymoblastoma, and medulloepithelioma treated in SFCE centres (Société Française de lutte contre les Cancers et les leucémies de l'Enfant et de l'adolescent) since 2000 were collected. Data were retrieved from clinical charts. Results: Thirty-eight patients were included in the analysis. Seventeen had an ETAN-TR, 13 had a medulloepithelioma, and 8 had an ETMR. No ependymoblastoma was included. The median age at diagnosis was 31 months (range, 2.8–141 months). The predominant tumor location was supratentorial (66 %); 18.4 % patients had metastatic lesion. LIN28A expression was positive in 11/11 patients. Amplification of the locus 19q13.42 was positive in 10/12 patients. Thirty patients were treated according to the primitive neuroectodermal tumors 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. LIN28A 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.
Fichier principal
Vignette du fichier
horwitz.pdf (373.03 Ko) Télécharger le fichier
Origin : Explicit agreement for this submission

Dates and versions

hal-01368643 , version 1 (12-10-2016)

Licence

Attribution - NonCommercial - NoDerivatives - CC BY 4.0

Identifiers

Cite

Meryl Horwitz, Christelle Dufour, Pierre Leblond, Franck Bourdeaut, Cécile Faure-Conter, et al.. Embryonal tumors with multilayered rosettes in children: the SFCE experience. Child's Nervous System, 2016, 32 (2), pp.299-305. ⟨10.1007/s00381-015-2920-2⟩. ⟨hal-01368643⟩
678 View
10278 Download

Altmetric

Share

Gmail Facebook Twitter LinkedIn More