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Childhood Central Nervous System Embryonal Tumors Treatment (PDQ®)

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Treatment Option Overview for CNS Embryonal Tumors

Risk Stratification for Medulloblastoma

Risk stratification is based on neuroradiographic evaluation for disseminated disease, cerebrospinal fluid (CSF) cytological examination, postoperative neuroimaging evaluation for the amount of residual disease, and age of the patient. Patients older than 3 years with medulloblastoma have been stratified into the following two risk groups:

  • Average risk: Children older than 3 years with tumors that are totally resected or near-totally resected (≤1.5 cm2 of residual disease) and no metastatic disease.[1]
  • High risk: Children older than 3 years with metastatic disease and/or subtotal resection (>1.5 cm2 of residual disease).[1] Metastatic disease includes neuroradiographic evidence of disseminated disease, positive cytology in lumbar or ventricular CSF obtained more than 10 days after surgery, or extraneural disease.[1] Children with tumors showing diffuse anaplasia, who otherwise would have been considered average risk, are assigned to the high-risk group.[2,3]

For younger children, in some studies for those younger than 3 years and for others younger than 4 or 5 years, similar separation into average-risk (no dissemination and ≤1.5 cm2 of residual disease) or high-risk (disseminated disease and/or >1.5 cm2 of residual disease) groups has been employed. Histologic findings of desmoplasia have also been used to connote a more favorable risk subgrouping, especially the medulloblastoma with extensive nodularity subgroup.

Assigning a risk group based on extent of resection and disease at diagnosis may not predict treatment outcome. Molecular genetic and histologic factors may be more informative.[4] Although molecular subdivisions will likely change risk characterization in the future, they are not yet used to assign treatment in North American prospective studies.

Table 3. Standard Treatment Options for Childhood Central Nervous System (CNS) Embryonal Tumors
Stage and/or Histopathologic ClassificationStandard Treatment Options
Newly diagnosed childhood medulloblastoma: 
 Children older than 3 years with average-risk medulloblastomaSurgery
Adjuvant therapy (radiation therapy and chemotherapy)
 Children older than 3 years with high-risk medulloblastomaSurgery
Adjuvant therapy (radiation therapy and chemotherapy)
 Children aged 3 years and youngerSurgery
Adjuvant chemotherapy
 
Newly diagnosed CNS primitive neuroectodermal tumor: 
 Children older than 3 yearsSurgery
Adjuvant therapy (radiation therapy and chemotherapy)
 Children aged 3 years and youngerSimilar to children aged 3 years and younger with medulloblastoma (surgery and adjuvant chemotherapy)
 
Newly diagnosed medulloepithelioma and ependymoblastomaSame as for children with high-risk medulloblastoma (surgery and adjuvant therapy) and children aged 3 years and younger with other embryonal tumors
 
Newly diagnosed pineoblastoma: 
 Children older than 3 yearsSurgery
Adjuvant therapy (radiation therapy and chemotherapy)
 Children aged 3 years and youngerBiopsy (for diagnosis)
Chemotherapy
High-dose, marrow-ablative chemotherapy with autologous bone marrow rescue or peripheral stem cell rescue
 
Recurrent childhood CNS embryonal tumors (treatment options not considered standard)Surgery
Radiation therapy
Chemotherapy
High-dose chemotherapy with stem cell rescue

References

  1. Zeltzer PM, Boyett JM, Finlay JL, et al.: Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: conclusions from the Children's Cancer Group 921 randomized phase III study. J Clin Oncol 17 (3): 832-45, 1999. [PUBMED Abstract]
  2. Giangaspero F, Wellek S, Masuoka J, et al.: Stratification of medulloblastoma on the basis of histopathological grading. Acta Neuropathol 112 (1): 5-12, 2006. [PUBMED Abstract]
  3. Eberhart CG, Kratz J, Wang Y, et al.: Histopathological and molecular prognostic markers in medulloblastoma: c-myc, N-myc, TrkC, and anaplasia. J Neuropathol Exp Neurol 63 (5): 441-9, 2004. [PUBMED Abstract]
  4. Taylor MD, Northcott PA, Korshunov A, et al.: Molecular subgroups of medulloblastoma: the current consensus. Acta Neuropathol 123 (4): 465-72, 2012. [PUBMED Abstract]
  • Updated: December 11, 2014