Brain tumors in infancy and early childhood

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By Roger Packer MD

Brain tumors in infancy and early childhood are also known as or subsume Congenital brain tumors, Connatal brain tumors, Infantile brain tumors, and Primitive brain tumors. -ed.

Approximately 10% to 15% of all childhood brain tumors will arise in the first 2 years of life. These tumors, which may be congenital, range from benign complex lesions that can be difficult to classify to highly malignant tumors that are often unresponsive to treatment. Embryonal tumors often arise in children younger than 2 years of age, and in the past decade an increasing incidence of the atypical teratoid/rhabdoid tumor has been reported. Although outcome is far from optimal, increasing evidence indicates that some subgroups of childhood infantile brain tumors have a somewhat better prognosis. This is especially true for the desmoplastic medulloblastoma. Because of the potential deleterious effects of radiation therapy on the immature nervous system, patients who are not cured by surgery are often treated with chemotherapy rather than radiation therapy. There is some evidence that intensification of chemotherapy has increased the likelihood of survival in some subsets of patients, especially infants with localized medulloblastoma. Molecularly targeted therapy is being explored in this patient population, as is the use of new radiation techniques utilizing more localized (conformal) radiotherapy.

Key Points

  • Although uncommon in children less than 2 years of age, primary central nervous system tumors still comprise almost 15% of all childhood brain tumors.
  • Many infantile brain tumors are congenital, may be large at diagnosis, and can be difficult to classify.
  • Treatment options for infantile brain tumors may be limited because of the immaturity of the nervous system and resultant risk of treatment-related neurotoxicity.
  • Primary infantile brain tumors range from the highly immature or primitive (medulloepitheliomas, AT/RT, etc.) to the large, but often very mature (desmoplastic infantile ganglioglioma); likewise, treatment and prognosis vary greatly.