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

Abstract

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.

Evolution of cerebral microbleeds after cranial irradiation in medulloblastoma patients

“…2,3 Current overall survival rates at 5 years range from 50% to 80%. [2][3][4][5] Neurotoxicity from cancer therapy remains an important issue in patient management and is in particular a concern in long-term survivors.…”

Extraneural metastases of medulloblastoma: Desmoplastic variants may have prolonged survival

“…This suggests that desmoplastic variant is an independent prognostic factor for improved survival. This was suggested by previous studies in children and young adults with CNS disease [5,11], but had not previously been demonstrated in patients with extraneural metastases.…”

Multi-nodular Desmoplastic Medulloblastoma in a Child: A Rare Radiological Physiognomy

“…These lesions are usually located within the posterior fossa normally around the vermis of the cerebellum [1]. These neoplasms are presumed to originate from neuroepithelial cells layers surrounding the roof of the fourth ventricle [8]. Desmoplastic variant of the neoplasms is a particular subtype that often has eccentric origin and may initiate a desmoplastic reaction within the overlying meninges [1,9].…”