Treatment Options

There are no standard treatment options for recurrent childhood CNS embryonal tumors. For more information, see the Treatment for Recurrent Childhood CNS Atypical Teratoid/Rhabdoid Tumor section in Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment.

For most children, treatment is palliative, and disease control is transient in patients previously treated with radiation therapy and chemotherapy, with more than 80% of patients progressing within 2 years.[3]; [7][Level of evidence C1] The temporal and spatial patterns of relapse differ between molecular subgroups. Patients with group 4 medulloblastomas present with delayed relapse compared with patients in other subgroups. In children who develop relapsed disease after radiation-sparing strategies, patients with group 4 and SHH medulloblastomas have higher rates of local relapse (42% and 39%, respectively), compared with patients with group 3 disease (17%).[8] For young children, predominantly those younger than 3 years at diagnosis who were never treated with radiation therapy, longer-term control with reoperation, radiation therapy, and chemotherapy is possible.[5,8-12]

Treatment approaches may include the following: