The Neuroradiological Spectra of Adult and Pediatric Medulloblastoma Differ : Results from a Literature-based Meta-analysis.

PURPOSE: Current knowledge on the spectrum of the neuroradiological appearance of adult medulloblastoma is sparse. Due to the rarity of the disease, adult patients were generally diagnosed and treated similar to children; however, pediatric and adult medulloblastomas display substantial molecular differences that may influence the neuroradiological phenotype. This study therefore aimed at assessment of the neuroradiological spectrum of adult medulloblastoma in comparison to pediatric tumors. METHODS: All available publications on adult medulloblastoma published until June 2013 were screened for imaging data on single patients. A total of 109 patients were identified and compared to 118 pediatric patients described in 4 cohorts. RESULTS: The average age of the adult patients was 34.3 years. Most adult medulloblastomas (57.6 %) were localized laterally (vs. 14.4 % in pediatric patients). On T1-weighted sequences, only 41.1 % of all adult medulloblastomas appeared hypointense (vs. 89.3 %) and 69.6 % were hyperintense on T2 sequences (vs. 83 %). In contrast to pediatric patients only 53.3 % showed strong contrast enhancement (pediatric patients 77.1 %), while the contrast uptake of the remainder was described as subtle, moderate or lacking. Contrast enhancement was more often described as inhomogeneous in adults (35.5 % as compared to 15.2 % in children) and 26.4 % had cysts. CONCLUSION: Although the neuroradiological spectrum of medulloblastoma in adults was similar to children, an atypical presentation with inhomogeneous contrast enhancement, more hyperintense signal on T1 and a more hypointense signal on T2-weighted sequences was common. Given the rarity of the tumor, awareness of these differences constitutes a prerequisite to avoid delays in diagnostics.

Chemotherapy increases long-term survival in patients with adult medulloblastoma--a literature-based meta-analysis.

BACKGROUND: Adult medulloblastoma is a potentially curable malignant entity with an incidence of 0.5-1 per million. Valid data on prognosis, treatment, and demographics are lacking, as most current knowledge stems from retrospective studies. Surgical resection followed by radiotherapy are accepted parts of treatment regimes; however, established prognostic factors and data clarifying the role of chemotherapy are missing. METHODS: We investigated 227 publications from 1969-2013, with 907 identifiable, individual patients being available for meta-analysis. Demographic data, risk stratification, and treatment of these patients were similar to previous cohorts. RESULTS: The median overall survival (mOS) was 65 months (95% CI: 54.6-75.3) , the 5-year overall survival was 50.9% with 16% of the patients dying more than 5 years after diagnosis. Incomplete resection, clinical and radiological signs for brainstem infiltration, and abstinence from radiotherapy were predictive of worse outcome. Metastatic disease at tumor recurrence was identified as a new prognostic factor, while neither metastasis at initial diagnosis nor desmoplastic/classic histology was correlated with survival. Patients receiving chemotherapy first-line survived significantly longer (mOS: 108 mo, 95% CI: 68.6-148.4) than patients treated with radiation alone (mOS: 57 mo, 95% CI: 39.6-74.4) or patients who received chemotherapy at tumor recurrence. This effect was not biased by tumor stage or decade of treatment. Importantly, (neo)adjuvant chemotherapy also significantly increased the chance for long-term survival (>5 y) compared with radiotherapy alone or chemotherapy at tumor recurrence. CONCLUSIONS: This meta-analysis clarifies relevant prognostic factors and suggests that chemotherapy as part of first-line therapy improves overall survival and increases the proportion of patients with long-term survival.