EPEN-03. LONG-TERM FOLLOW-UP OF AIEOP 2ND SERIES OF CHILDREN AND ADOLESCENT WITH PRIMARY INTRACRANIAL (ST:SUPRATENTORIAL; PF: POSTERIOR FOSSA) EPENDYMOMA AND METHYLATION GROUPS RE-ANALYSES. (4th December 2020)

• Record Type: Journal Article
• Authors: Massimino, Maura; Buttarelli, Francesca Romana; Witt, Hendrik; Johann, Pascal; Minasi, Simone; Pfister, Stefan M; Pajtler, Kristian W; Antonelli, Manila; Barretta, Francesco; Modena, Piergiorgio; Gandola, Lorenza; Garrè, Maria Luisa; Bertin, Daniele; Mastronuzzi, Angela; Mascarin, Maurizio; Quaglietta, Lucia; Viscardi, Elisabetta; Sardi, Iacopo; Ruggiero, Antonio; Pollo, Bianca; Buccoliero, Annamaria; Boschetti, Luna; Biassoni, Veronica; Schiavello, Elisabetta; Chiapparini, Luisa; Erbetta, Alessandra; Giangaspero, Felice
• Is Part Of: Neuro-oncology. Volume 22(2020)Supplement 3
• Page Start: iii308
• Page End: iii308
• Publication Date: 2020-12-04
• Subjects: Brain Neoplasms -- Periodicals; Brain -- Tumors -- Periodicals; Brain -- Cancer -- Periodicals; Nervous system -- Cancer -- Periodicals; 616.99481
• DOI: 10.1093/neuonc/noaa222.144
• Languages: English
• ISSNs: 1522-8517

Abstract

Abstract: BACKGROUND: This 2002–2014 Italian prospective study stratified 160 patients by surgical resection (complete=NED/incomplete=ED) and centrally-reviewed grade. Grade2/NED patients received focal radiotherapy (RT) up to 59.4Gy, Grade3/NED received 4 courses of VEC(vincristine, etoposide, cyclophosphamide) after RT.ED patients received 1–4 VEC courses, second-look surgery, 59.4 Gy+8Gy boost on measurable residue. METHODS: We re-analyzed data at 115 months follow-up including methylation profile on available samples. RESULTS: Global PFS/OS at 5/10 years were 66/59% and 80/74%, respectively. Of the 64 relapsers at median 20 months, 53 died at median 37/18 months after diagnosis/relapse, respectively.10/64 relapsed after 5 years (66–126 months); 4 died, relapse was local in 8/10, metastatic 1, combined 1;5/10 patients were below age 3, 5 females, 8 PF tumors. Their survival post-relapse was not longer than earlier relapsers'. At univariable analysis, age over 3 years, female sex, complete surgery, grade 2, no shunt confirmed better PFS/OS. 66/95 analyzed tumors received a score >0.80 through the DNA methylation-based central nervous system tumor classifier: 41/8 as PFA/PFB, respectively, 14/17 ST as RELA-positive (3 scored for other molecular entities i.e. anaplastic PXA, LGG MYB, HGNET). Prognostic factors were equally distributed among PFA/PFB groups, 1 only group B patient relapsed locally at 96 months. CONCLUSIONS: Already published prognostic factors remained at long-term follow-up;6.2% patients had late relapses. OS after relapse was not better in late relapsers. Group B confirmed better prognosis but all patients had received «at least» adjuvant RT. Modern ependymoma trials need long follow-up to draw firm conclusions.