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Childhood Central Nervous System Embryonal Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Newly Diagnosed Childhood Medulloblastoma

Treatment Modalities

Surgery

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Surgery is considered a standard part of treatment for histologic confirmation of tumor type and as a means to improve outcome. Total or near-total resections are considered optimal, if they can be performed safely.[1]

Postoperatively, children may have significant neurologic deficits caused by preoperative tumor-related brain injury, hydrocephalus, or surgery-related brain injury.[2][Level of evidence: 3iC] A significant number of patients with medulloblastoma will develop cerebellar mutism syndrome. Symptoms of cerebellar mutism syndrome include the following:

  • Delayed onset of speech.
  • Suprabulbar palsies.
  • Ataxia.
  • Hypotonia.
  • Emotional lability.

The etiology of cerebellar mutism syndrome remains unclear, although cerebellar vermian damage and/or disruption of cerebellar-cortical tracts has been postulated as the possible cause for the mutism.[3,4]; [5][Level of evidence: 3iC] In two Children's Cancer Group studies evaluating children with both average-risk and high-risk medulloblastoma, the syndrome has been identified in nearly 25% of patients.[4,5,6]; [7][Level of evidence: 3iiiC] Approximately 50% of patients with this syndrome manifest long-term, permanent neurologic and neurocognitive sequelae.[5,7]

Radiation therapy

Radiation therapy to the primary tumor site is usually in the range of 54 Gy to 55.8 Gy. This is usually given with a 1 cm to 2 cm margin around the primary tumor site, preferably by conformal techniques. For all medulloblastomas in children older than 3 or 4 years at diagnosis, craniospinal radiation therapy is given at doses ranging between 23.4 Gy and 36 Gy, depending on risk factors, such as extent of disease at diagnosis. Chemotherapy is routinely administered during and after radiation therapy.

For children younger than 3 years, efforts are made to omit or delay radiation, given the profound impact of radiation at this age. Children of all ages are susceptible to the adverse effects of radiation on brain development. Debilitating effects on neurologic/cognitive development, growth, and endocrine function have been frequently observed, especially in younger children.[8,9,10,11,12] The use of proton-beam therapy to reduce toxicity is under investigation.

Chemotherapy

Chemotherapy, usually given during and after radiation therapy, is a standard component of treatment for older children with medulloblastoma and other embryonal tumors. Chemotherapy can be used to delay and sometimes obviate the need for radiation therapy in 20% to 40% of children younger than 3 to 4 years with nondisseminated medulloblastoma.[13,14]; [12][Level of evidence: 3iiiC]

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