In:
Neuro-Oncology, Oxford University Press (OUP), Vol. 22, No. Supplement_3 ( 2020-12-04), p. iii393-iii393
Abstract:
Irradiation-sparing approaches are used in young children with medulloblastoma (MB) given the vulnerability of the developing brain to neurocognitive impairment. Limited data are available following relapse for these patients. We aimed to describe the management and outcomes of young children with MB who relapsed after initial treatment without craniospinal irradiation (CSI). METHODS International retrospective study including patients with MB diagnosed between 1995–2017, ≤ 72 months old, initially treated without CSI, who subsequently relapsed. RESULTS Data are available for 52 patients (32 male). Median age at initial diagnosis was 27 months (range, 6–72) with 24 being metastatic. Initial therapy included conventional chemotherapy alone or high-dose chemotherapy (HDC) in 21 and 31 subjects, respectively. Three received upfront focal irradiation. Molecular subgrouping, available for 24 tumors, included 9 SHH and 15 non-WNT/non-SHH. Median time to relapse was 13 months (range, 3–63). Relapse was local, disseminated or combined in 20, 15, and 16, respectively. Salvage therapy with curative intent was given in 42/52 patients, including CSI in 28 subjects (median dose 36Gy, 18–41.4) or focal irradiation in 5 others. Three received HDC only. At a median follow-up time of 46 months (range, 4–255), 25 (48%) were alive, including 7/9 SHH and 7/15 non-WNT/non-SHH. The 2- and 5-year OS was 67% and 56% (SE, 7%), respectively. Two of 3 patients with SHH who did not receive salvage radiotherapy are survivors. CONCLUSION A substantial proportion of young children who relapse following irradiation-sparing strategies can be salvaged. Neurocognitive and ototoxicity outcomes are being evaluated.
Type of Medium:
Online Resource
ISSN:
1522-8517
,
1523-5866
DOI:
10.1093/neuonc/noaa222.500
Language:
English
Publisher:
Oxford University Press (OUP)
Publication Date:
2020
detail.hit.zdb_id:
2094060-9
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