Abstract
Purpose. To correlate the radiological aspects of metastases, the response to chemotherapy, and patient outcome in disseminated childhood medulloblastoma. Patients and Methods. This population-based study concerned 117 newly diagnosed children with disseminated medulloblastoma treated at the Institute Gustave Roussy between 1988 and 2008. Metastatic disease was assessed using the Chang staging system, their form (positive cerebrospinal fluid (CSF), nodular or laminar), and their extension (positive cerebrospinal fluid, local, extensive). All patients received preirradiation chemotherapy. Results. The overall survival did not differ according to Chang M-stage. The 5-year overall survival was 59% in patients with nodular metastases compared to 35% in those with laminar metastases. The 5-year overall survival was 76% in patients without disease at the end of pre-irradiation chemotherapy compared to 34% in those without a complete response (P = 0.0008). Conclusions. Radiological characteristics of metastases correlated with survival in patients with medulloblastoma. Complete response to sandwich chemotherapy was a strong predictor of survival.
Highlights
Medulloblastoma (MB) is the most common malignant brain tumor of childhood
Forty-seven (40%) children received sequential highdose chemotherapy (HDCT) with autologous stem cell transplantation (ASCT) followed by reduced dose craniospinal irradiation (CSI), and 22 patients (19%) were treated with sequential HDCT with ASCT followed by standard dose CSI
Chang’s, qualitative and quantitative classifications are significantly associated with early complete response rate after sandwich chemotherapy in univariate and multivariate analyses (Table 2). In this retrospective review of 117 children with disseminated medulloblastoma treated at a single institution, we found that the phenotype of metastasis had an impact on Overall Survival (OS)
Summary
Patients with disseminated medulloblastoma are classified according to Chang’s operative staging system [2], where the extent of metastasis is subdivided into M0 (no metastasis), M1 (presence of tumor cells in the CSF), M2 (nodular seeding in the cerebellar or cerebral subarachnoid space or in the third or lateral ventricle), M3 (metastasis in spinal subarachnoid space), and M4 (metastases outside the cerebrospinal axis). This sensitive operative system was initially determined from operative records and autopsy specimens and has been later adapted to modern imaging techniques such as MRI. We made the hypothesis that metastases could be further
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