Outcome and prognostic factors of desmoplastic medulloblastoma treated within a multidisciplinary treatment concept

BMC Cancer

Research Article

Wolfgang Wick¹  Timo Gaiser²  Olaf Witt³  Andreas E Kulozik³  Jürgen Debus⁴  Stefan Rieken⁴  Stephanie E Combs⁴  Angela Mohr⁴  Thomas Welzel⁴ 

[1] University Hospital of Heidelberg, Department of Neurooncology, Im Neuenheimer Feld 400, 69120, Heidelberg, Germany;University Hospital of Heidelberg, Department of Neuropathology, Im Neuenheimer Feld 220/221, 69120, Heidelberg, Germany;University Hospital of Heidelberg, Department of Pediatric Oncology, Hematology, Immunology, and Pneumonology, Im Neuenheimer Feld 430, 69120, Heidelberg, Germany;University Hospital of Heidelberg, Department of Radiation Oncology, Im Neuenheimer Feld 400, 69120, Heidelberg, Germany;

关键词: Overall Survival;    Adjuvant Chemotherapy;    Medulloblastoma;    Posterior Fossa;    Fourth Ventricle;   

DOI  :  10.1186/1471-2407-10-450

 received in 2009-12-09, accepted in 2010-08-23,  发布年份 2010

来源: Springer

【 摘 要 】

BackgroundDesmoplasia in medulloblastoma is often diagnosed in adult patients and was repeatedly associated with improved results. Today, all medulloblastoma patients receive intensive multimodal treatment including surgery, radiotherapy and chemotherapy. This study was set up to investigate treatment outcome and prognostic factors after radiation therapy in patients with desmoplastic medulloblastomas.MethodsTwenty patients treated for desmoplastic medulloblastoma in the Department of Radiation Oncology at the University of Heidelberg between 1984 and 2007 were included. Data were collected retrospectively. Tumor resection was performed in all patients. All patients underwent postsurgical radiotherapy (RT). Two patients underwent whole brain radiotherapy (WBRT), and 18 patients received craniospinal irradiation (CSI). In all patients, an additional boost was delivered to the posterior fossa. The median dose to the whole brain and the craniospinal axis was 35.2 Gray (Gy), and 54.4 Gy to the posterior fossa. Fourteen patients received chemotherapy, including seven who were treated with combined radiochemotherapy and twelve who received adjuvant chemotherapy. Statistical analysis was performed using the log-rank test and the Kaplan-Meier method.ResultsMedian follow-up was 59 months. Overall (OS), local (LPFS) and distant progression-free survival (DPFS) was 80%, 71.2%, and 83.3% at 60 months. Patients who suffered from local or distant relapses had significantly worse outcome. Five patients died from recurrent medulloblastoma. Treatment-associated toxicity was acceptable.ConclusionsMultimodal approaches with surgical resection followed by chemoirradiation achieved high response rates with long OS in desmoplastic medulloblastoma patients. Staging parameters expected to predict for poor prognosis did not significantly influence outcome. However, success of any first line regimen had strong impact on disease control, and remission was achieved in no patient with relapsing disease. Multimodal concepts must be evaluated in further clinical trials.

【 授权许可】

CC BY   
© Rieken et al; licensee BioMed Central Ltd. 2010