NEURO-ONCOLOGY (NEUROPLASMS) (M ROSENFELD, SECTION EDITOR)
First Online: 24 May 2012 DOI:
Cite this article as: von Hoff, K. & Rutkowski, S. Curr Treat Options Neurol (2012) 14: 416. doi:10.1007/s11940-012-0183-8 Opinion statement
The mainstay of medulloblastoma treatment is high-quality interdisciplinary collaboration in diagnosis, treatment, and aftercare by all involved disciplines. The first step in treatment of medulloblastoma is a maximal safe surgery, followed by thorough staging. Surgery should only be performed in experienced neurosurgical centers, with age-appropriate postoperative care. As optimal risk stratification is based on histopathological and neuroradiological assessments, these should be performed or confirmed by experienced specialists. Central review of histopathological subtype, as well as review of staging evaluations is highly desirable. For young children with desmoplastic/nodular (DMB), or extensive nodular medulloblastoma, craniospinal or any radiotherapy should be avoided. For young children with classic medulloblastoma (CMB), large cell, or anaplastic medulloblastoma (LC/A MB) optimized strategies with high-dose chemotherapy and autologous stem cell rescue with or without local radiotherapy are under investigation. For older clinical standard risk patients (without metastases, without postoperative residual tumor >1.5 cm
2) with CMB or DMB, craniospinal radiotherapy with 23.4 Gy and boost to the posterior fossa to 54 Gy, followed by maintenance chemotherapy can be regarded as a standard therapy besides other currently applied regimen, such as the use of intensified chemotherapy after irradiation. Older children with LC/A MB, metastatic medulloblastoma, and/or large residual tumor can be regarded as high-risk patients and should receive intensified treatment: intensified chemotherapeutic regimen before or after radiotherapy with increased dose (36-Gy CSI normofractionated, or 40-Gy hyperfractionated) is used. For treatment to be effective, quality control of radiotherapy is of high relevance. Information on long-term sequelae is essential and appropriate multidisciplinary follow-up and support, including rehabilitation and help for reintegration, is necessary. Whenever possible, patients should be included in prospective studies, and tumor material should be sampled to facilitate further research on medulloblastoma biology, which will significantly influence the stratification criteria and the introduction of targeted therapies in standard treatment recommendations in the future. Keywords Medulloblastoma Risk stratification Treatment Large-cell medulloblastoma Anaplastic medulloblastoma Chemotherapy Radiation Craniospinal Radiotherapy Metastases Desmoplastic/nodular medulloblastoma Classic medulloblastoma Medulloblastoma with extensive nodularity Surgery Residual tumor Posterior fossa syndrome Primitive neuroectodermal tumor PNET References and Recommended Reading Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
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