Treatment Outcome of Desmoplastic Medulloblastoma after Surgery and Chemo-Radiotherapy: Mansoura Experience,EMAN A. TOSON and MOHAMED A. MATTAR
Abstract
Objective: To investigate the treatment outcome and prognostic factors of desmoplastic medulloblastoma in patients who received postoperative craniospinal irradiation with or without chemotherapy.
Methods: This retrospective study was carried out between 2000 and 2009 by reviewing the records of 20 patients with histologically confirmed desmoplastic medulloblastoma (12 males and 8 females) who presented to Clinical Oncology & Nuclear Medicine Department.
Results: The median age was 17 years, (range 3-39) with male predominance (1.5-1). The most predominant symptoms was vomiting (80%)and headache (70%). The tumor location was lateral in 65% of cases and midline in 35%. Tumor resection was completed in 11 (55%) patients. All patients received craniospinal irradiation. The median dose to the posterior fossa was 54 Gy (range 49-56 Gy), 65% of patients received adjuvant chemotherapy. The median follow-up was 50 months (range 25-120) Five years overall survival and progression free survival rates were 70% and 65% respectively. Six patients developed relapse. Posterior fossa was the most common site of relapse (20%). Brain stem infiltration is correlated with poor prognosis (p=0.005). Spinal seeding at presentation is poor prognostic factor but in our study it did not reach statistical significance. Extent of surgical resection is a good prognostic factor (p=0.003). Age, sex, tumor location does not significantly influence the survival outcome. The most frequently reported adverse events include, sensory neuropathy, nausea, vomiting and febrile neutropenia.
Conclusion: Multimodality treatment approach with sur-gical resection followed by chemo-radiotherapy achieved high response rates with long overall survival in desmoplastic medulloblastoma patients. PFS was found to be significantly better with complete surgical resection and higher radiotherapy dose to the posterior fossa. Brain stem infiltration is correlated with poor prognosis.