Gliomatosis cerebri in children: A poor prognostic phenotype of diffuse gliomas with a distinct molecular profile.
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Nussbaumer Gunther, Benesch Martin, Grabovska Yura, Mackay Alan, Castel David, Grill Jacques, Alonso Marta M, Antonelli Manila, Bailey Simon, Baugh Joshua N, Biassoni Veronica, Blattner-Johnson Mirjam, Broniscer Alberto, Carai Andrea, Colafati Giovanna Stefania, Colditz Niclas, Corbacioglu Selim, Crampsie Shauna, Entz-Werle Natacha, Eyrich Matthias, Friker Lea L, Frühwald Michael C, Garrè Maria Luisa, Gerber Nicolas U, Giangaspero Felice, Gil-da-Costa Maria J, Graf Norbert, Hargrave Darren, Hauser Peter, Herrlinger Ulrich, Hoffmann Marion, Hulleman Esther, Izquierdo Elisa, Jacobs Sandra, Karremann Michael, Kattamis Antonis, Kebudi Rejin, Kortmann Rolf-Dieter, Kwiecien Robert, Massimino Maura, Mastronuzzi Angela, Miele Evelina, Morana Giovanni, Noack Claudia M, Pentikainen Virve, Perwein Thomas, Pfister Stefan M, Pietsch Torsten, Roka Kleoniki, Rossi Sabrina, Rutkowski Stefan, Schiavello Elisabetta, Seidel Clemens, Štěrba Jaroslav, Sturm Dominik, Sumerauer David, Tacke Anna, Temelso Sara, Valentini Chiara, van Vuurden Dannis, Varlet Pascale, Veldhuijzen van Zanten Sophie E M, Vinci Maria, von Bueren André O, Warmuth-Metz Monika, Wesseling Pieter, Wiese Maria, Wolff Johannes E A, Zamecnik Josef, Morales La Madrid Andrés, Bison Brigitte, Gielen Gerrit H, Jones David T W, Jones Chris, Kramm Christof M
Аннотация
METHODS: We conducted a multinational retrospective study of 104 children and adolescents with GC providing comprehensive clinical and (epi-)genetic characterization.
RESULTS: Median overall survival (OS) was 15.5 months (interquartile range, 10.9-27.7) with a 2-year survival rate of 28%. Histopathological grading correlated significantly with median OS: CNS WHO grade II: 47.8 months (25.2-55.7); grade III: 15.9 months (11.4-26.3); grade IV: 10.4 months (8.8-14.4). By DNA methylation profiling (n = 49), most tumors were classified as pediatric-type diffuse high-grade glioma (pedHGG), H3-/IDH-wild-type (n = 31/49, 63.3%) with enriched subclasses pedHGG_RTK2 (n = 19), pedHGG_A/B (n = 6), and pedHGG_MYCN (n = 5), but only one pedHGG_RTK1 case. Within the pedHGG, H3-/IDH-wild-type subgroup, recurrent alterations in EGFR (n = 10) and BCOR (n = 9) were identified. Additionally, we observed structural aberrations in chromosome 6 in 16/49 tumors (32.7%) across tumor types. In the pedHGG, H3-/IDH-wild-type subgroup TP53 alterations had a significant negative effect on OS.
CONCLUSIONS: Contrary to previous studies, our representative pediatric GC study provides evidence that GC has a strong predilection to arise on the background of specific molecular features (especially pedHGG_RTK2, pedHGG_A/B, EGFR and BCOR mutations, chromosome 6 rearrangements).