Roshankumar Patil1, Tejpal Gupta1, Madan Maitre1, Archya Dasgupta1, Ayushi Sahay2, Sridhar Epari2, Neelam Shirsat3, Abhishek Chatterjee1, Rahul Krishnatry1, Jayant Sastri Goda1, Aliasgar Moiyadi4, Vijay Patil5, Girish Chinnaswamy6, Nazia Bano7, Rakesh Jalali1. 1. Department of Radiation Oncology and ACTREC/TMH, Tata Memorial Center, Homi Bhabha National Institute (HBNI), Mumbai, India. 2. Department of Pathology Disease Management Group, ACTREC/TMH, Tata Memorial Center, Homi Bhabha National Institute (HBNI), Mumbai, India. 3. Neuro-Oncology Laboratory Disease Management Group, ACTREC/TMH, Tata Memorial Center, Homi Bhabha National Institute (HBNI), Mumbai, India. 4. Department of Neuro-Surgical Oncology, ACTREC/TMH, Tata Memorial Center, Homi Bhabha National Institute (HBNI), Mumbai, India. 5. Department of Medical Oncology, and ACTREC/TMH, Tata Memorial Center, Homi Bhabha National Institute (HBNI), Mumbai, India. 6. Department of Pediatric Oncology Disease Management Group, ACTREC/TMH, Tata Memorial Center, Homi Bhabha National Institute (HBNI), Mumbai, India. 7. Neuro-Oncology Disease Management Group, ACTREC/TMH, Tata Memorial Center, Homi Bhabha National Institute (HBNI), Mumbai, India.
Abstract
Purpose: Medulloblastomas, comprising 20%-25% of all primary brain tumors in children are much rarer in adulthood. Disease biology varies substantially across different age groups; however, owing to rarity, adults with medulloblastoma are traditionally treated using pediatric protocols. This is a retrospective audit of adolescent and adult medulloblastoma from a comprehensive cancer center. Methods: Data regarding demography, clinical presentation, imaging characteristics, histopathological features, molecular profiling, risk stratification, treatment details, and outcomes were retrieved from medical records. All time-to-event outcomes were analyzed using Kaplan-Meier method and compared with the log-rank test. Univariate and multivariate analysis of relevant prognostic factors was done with p value <0.05 being considered statistically significant. Results: A total of 162 patients ≥15 years of age with medulloblastoma were included. The median age was 25 years (range: 15-59 years) with leptomeningeal metastases seen in 31 (19%) patients at initial diagnosis. Following surgery, patients were treated with appropriate risk-stratified adjuvant therapy comprising of craniospinal irradiation plus boost with or without systemic chemotherapy. At a median follow-up of 50 months, 5-year Kaplan-Meier estimates of progression-free survival and overall survival were 53.5% and 59.5%, respectively. The addition of adjuvant systemic chemotherapy did not impact upon survival in standard-risk medulloblastoma. High-risk (HR) disease and anaplastic histology emerged as significant and independent predictors of poor survival on multivariate analysis. Conclusion: Medulloblastoma is a rare tumor in adolescents and adults with key differences in disease biology and resultant outcomes compared with the pediatric population. Contemporary management comprising maximal safe resection followed by appropriate risk-stratified adjuvant therapy provides acceptable survival outcomes.
Purpose: Medulloblastomas, comprising 20%-25% of all primary brain tumors in children are much rarer in adulthood. Disease biology varies substantially across different age groups; however, owing to rarity, adults with medulloblastoma are traditionally treated using pediatric protocols. This is a retrospective audit of adolescent and adult medulloblastoma from a comprehensive cancer center. Methods: Data regarding demography, clinical presentation, imaging characteristics, histopathological features, molecular profiling, risk stratification, treatment details, and outcomes were retrieved from medical records. All time-to-event outcomes were analyzed using Kaplan-Meier method and compared with the log-rank test. Univariate and multivariate analysis of relevant prognostic factors was done with p value <0.05 being considered statistically significant. Results: A total of 162 patients ≥15 years of age with medulloblastoma were included. The median age was 25 years (range: 15-59 years) with leptomeningeal metastases seen in 31 (19%) patients at initial diagnosis. Following surgery, patients were treated with appropriate risk-stratified adjuvant therapy comprising of craniospinal irradiation plus boost with or without systemic chemotherapy. At a median follow-up of 50 months, 5-year Kaplan-Meier estimates of progression-free survival and overall survival were 53.5% and 59.5%, respectively. The addition of adjuvant systemic chemotherapy did not impact upon survival in standard-risk medulloblastoma. High-risk (HR) disease and anaplastic histology emerged as significant and independent predictors of poor survival on multivariate analysis. Conclusion: Medulloblastoma is a rare tumor in adolescents and adults with key differences in disease biology and resultant outcomes compared with the pediatric population. Contemporary management comprising maximal safe resection followed by appropriate risk-stratified adjuvant therapy provides acceptable survival outcomes.
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