Erschienen in:
16.06.2023 | Research
Optimizing reirradiation for relapsed medulloblastoma: identifying the ideal patient and tumor profiles
verfasst von:
Maura Massimino, Sabina Vennarini, Francesca Romana Buttarelli, Manila Antonelli, Francesca Colombo, Simone Minasi, Emilia Pecori, Paolo Ferroli, Carlo Giussani, Marco Schiariti, Elisabetta Schiavello, Veronica Biassoni, Alessandra Erbetta, Luisa Chiapparini, Olga Nigro, Luna Boschetti, Francesca Gianno, Evelina Miele, Piergiorgio Modena, Loris De Cecco, Bianca Pollo, Francesco Barretta
Erschienen in:
Journal of Neuro-Oncology
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Ausgabe 3/2023
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Abstract
Background
First-line therapies for medulloblastoma(MBL) are obtaining higher survival-rates while decreasing late-effects, but treatment at relapse is not standardized. We report here the experience with MBL re-irradiation(re-RT), its timing and outcome in different clinical settings and tumor groups.
Methods
Patient’s staging/treatment at diagnosis, histotypes/molecular subgroups, relapse site/s, re-treatments outcome are reported.
Results
25 patients were included, with a median age of 11.4 years; 8 had metastases. According to 2016–2021 WHO-classification, 14 had SHH subgroup tumors(six TP53 mutated,one + MYC,one + NMYC amplification), 11 non-WNT/non-SHH (two with MYC/MYCN amplification).Thirteen had received HART-CSI, 11 standard-CSI, one HFRT; all post-radiation chemotherapy(CT), 16 also pre-RT. Median time to relapse (local-LR in nine, distant-DR in 14, LR + DR in two) was 26 months. Fourteen patients were re-operated, in five cases excising single DR-sites, thereafter three received CT, two after re-RT; out of 11 patients not re-operated, four had re-RT as first treatment and seven after CT. Re-RT was administered at median 32 months after first RT: focally in 20 cases, craniospinal-CSI in five. Median post-relapse-PFS/after re-RT was 16.7/8.2 months, while overall survival-OS was 35.1/23.9 months, respectively. Metastatic status both at diagnosis/relapse negatively affected outcome and re-surgery was prognostically favorable. PD after re-RT was however significantly more frequent in SHH (with a suggestive association with TP53 mutation, p = 0.050). We did not observe any influence of biological subgroups on PFS from recurrence while SHH showed apparently worse OS compared to non-WNT/non-SHH group.
Conclusions
Re-surgery + reRT can prolong survival; a substantial fraction of patients with worse outcome belongs to the SHH-subgroup.