Won Kee Ahn, Seung Min Hahn, Hong In Yoon, Jeongshim Lee, Eun Kyung Park, Kyu Won Shim, Dong Seok Kim, Chang-Ok Suh, Se Hoon Kim, Chuhl Joo Lyu, Jung Woo Han
Cancer Res Treat. 2024;56(2):652-664. Published online November 30, 2023
Purpose The Korean Society of Pediatric Neuro-Oncology (KSPNO) conducted treatment strategies for children with medulloblastoma (MB) by using alkylating agents for maintenance chemotherapy or tandem high-dose chemotherapy (HDC) with autologous stem cell rescue (ASCR) according to the risk stratification. The purpose of the study was to assess treatment outcomes and complications based on risk-adapted treatment and HDC.
Materials and Methods Fifty-nine patients diagnosed with MB were enrolled in this study. Patients in the standard-risk (SR) group received radiotherapy (RT) after surgery and chemotherapy using the KSPNO M051 regimen. Patients in the high-risk (HR) group received two and four chemotherapy cycles according to the KSPNO S081 protocol before and after reduced RT for age following surgery and two cycles of tandem HDC with ASCR consolidation treatment.
Results In the SR group, 24 patients showed 5-year event-free survival (EFS) and overall survival (OS) estimates of 86.7% (95% confidence interval [CI], 73.6 to 100) and 95.8% (95% CI, 88.2 to 100), respectively. In the HR group, more infectious complications and mortality occurred during the second HDC than during the first. In the HR group, the 5-year EFS and OS estimates were 65.5% (95% CI, 51.4 to 83.4) and 72.3% (95% CI, 58.4 to 89.6), respectively.
Conclusion High intensity of alkylating agents for SR resulted in similar outcomes but with a high incidence of hematologic toxicity. Tandem HDC with ASCR for HR induced favorable EFS and OS estimates compared to those reported previously. However, infectious complications and treatment-related mortalities suggest that a reduced chemotherapy dose is necessary, especially for the second HDC.
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Ferroptosis Transcriptional Regulation and Prognostic Impact in Medulloblastoma Subtypes Revealed by RNA-Seq Christophe Desterke, Yuanji Fu, Jenny Bonifacio-Mundaca, Claudia Monge, Pascal Pineau, Jorge Mata-Garrido, Raquel Francés Antioxidants.2025; 14(1): 96. CrossRef
Purpose The purpose of this study is to investigate the long-term results and appropriateness of radiation therapy (RT) for medulloblastoma (MB) at a single institution. Materials and Methods We analyzed the clinical outcomes of 106 patients with MB who received RT between January 1992 and October 2009. The median age was 7 years (range, 0 to 50 years), and the proportion of M0, M1, M2, and M3 stages was 60.4%, 8.5%, 4.7%, and 22.6%, respectively. The median total craniospinal irradiation (CSI) and posterior fossa tumor bed dose in 102 patients (96.2%) treated with CSI was 36 Gy and 54 Gy, respectively. Results The median follow-up period in survivors was 132 months (range, 31 to 248 months). A gradual improvement in survival outcomes was observed, with 5-year overall survival rates of 61.5% in 1990s increasing to 73.6% in 2000s. A total of 29 recurrences (27.4%) developed at the following sites: five (17.2%) in the tumor bed; five (17.2%) in the posterior fossa other than the tumor bed; nine (31%) in the supratentorium; and six (20.7%) in the spinal subarachnoid space only. The four remaining patients showed multiple site recurrences. Among 12 supratentorial recurrences, five cases recurred in the subfrontal areas. Although the frequency of posterior fossa/tumor bed recurrences was significantly high among patients treated with subtotal resection, other site (other intracranial/spinal) recurrences were more common among patients treated with gross tumor removal (p=0.016). There was no case of spinal subarachnoid space relapse from desmoplastic/extensive nodular histological subtypes. Conclusion Long-term follow-up results and patterns of failure confirmed the importance of optimal RT dose and field arrangement. More tailored multimodal strategies and proper CSI technique may be the cornerstones for improving treatment outcomes in MB patients.
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Remote Supratentorial Recurrent Medulloblastoma: Case Study and Literature Review Asimina Dominari, Elias Antoniades, Antonio Capiccelo, Emmanuil Hatzipantelis, Nikolaos Foroglou Asian Journal of Neurosurgery.2022; 17(02): 286. CrossRef
Sequential improvement in paediatric medulloblastoma outcomes in a low-and-middle-income country setting over three decades Johann Riedemann, Anthony Figaji, Alan Davidson, Clare Stannard, Komala Pillay, Tracy Kilborn, Jeannette Parkes South African Journal of Oncology.2021;[Epub] CrossRef
Multifocal recurrence of medulloblastoma: a long follow-up case study Benny Zulkarnaien, Edwin Suharlim, Eka Susanto, Soehartati Argadikoesoema Gondhowiardjo Medical Journal of Indonesia.2020; 29(1): 93. CrossRef
Radiotherapy Advances in Paediatric Medulloblastoma Treatment L. Padovani, G. Horan, T. Ajithkumar Clinical Oncology.2019; 31(3): 171. CrossRef
Craniospinal irradiation using helical tomotherapy for central nervous system tumors Sanziana R.I. Schiopu, Gregor Habl, Matthias Häfner, Sonja Katayama, Klaus Herfarth, Juergen Debus, Florian Sterzing Journal of Radiation Research.2017;[Epub] CrossRef
Highly Conformal Craniospinal Radiotherapy Techniques Can Underdose the Cranial Clinical Target Volume if Leptomeningeal Extension through Skull Base Exit Foramina is not Contoured D.J. Noble, T. Ajithkumar, J. Lambert, I. Gleeson, M.V. Williams, S.J. Jefferies Clinical Oncology.2017; 29(7): 439. CrossRef
Long-term toxic effects of proton radiotherapy for paediatric medulloblastoma: a phase 2 single-arm study Torunn I Yock, Beow Y Yeap, David H Ebb, Elizabeth Weyman, Bree R Eaton, Nicole A Sherry, Robin M Jones, Shannon M MacDonald, Margaret B Pulsifer, Beverly Lavally, Annah N Abrams, Mary S Huang, Karen J Marcus, Nancy J Tarbell The Lancet Oncology.2016; 17(3): 287. CrossRef
PURPOSE The present study explores the expression rate of p53 mutation and the correlation between the expression of p53 protein and prognostic factors in medulloblastoma/ PNET (primitive neuroectodermal tumor). MATERIALS AND METHODS We studied retrospectively 24 patients with medulloblastoma/ PNET, who were admitted in Dong-A University Hospital, Pusan National University Hospital and Inje University Pusan Paik Hospital from 1988 to 1995. Detection of p53 mutations was made by immunohistochemical staining of p53 protein on paraffin- embedded tissues. The correlation between the expression of p53 protein and prognostic factors was evaluated by the Spearman correlation analysis. RESULTS p53 protein was expressed in 6 of 24 patients (25%). In 20 patients who could be evaluated for metastasis, 16 patients of M0, 1 patient of M1 and 3 patients of M2 were grouped by M stage, and the expression of p53 was detected in 1 of 16 M0 group (6.3%) and 3 of 3 M2 group (100%). p53 expression was significantly related to the M stage of medulloblastoma/PNET (r=0.73, p<0.001). The detection of p53 was not significantly associated with T stage, cellular differentiation and the relapse rate of medulloblastoma/ PNET. CONCLUSION The immunohistochemical detection rate of p53 protein in medulloblastoma/ PNET was 25%. The expression of p53 protein was significantly related to the M stage, with higher expression rate in M2 group of medulloblsatoma/PNET.