–Craniospinal irradiation with age-dependent dosing

–Cranial dose 40 Gy, with an additional 10to 15-Gy involved-field radiotherapy

–Spinal irradiation dose 30–35 Gy

–Reduced irradiation after chemotherapy with the same results: 80% 3 year event-free survival in children with average risk disease

–Adverse sequelae after irradiation: growth retardation, neuroendocrine deficits, psychosocial disturbances, neurocognitive deficiencies

•Chemotherapy:

–Highly chemosensitive tumors, especially in small children (below the age of 3 years), in patients with brain stem infiltration, or in those with subtotal tumor resection

–Combination chemotherapy with cisplatin or carboplatin, vincristine, cyclophosphamide, etoposide, high-dose methotrexate with leucovorin rescue, according to schemas in cooperative group protocols

Patients with high-risk disease include those with presence of metastatic disease or >1.5 cm residual disease; for such patients, preand/or postsurgery radiotherapy/ chemotherapy, or surgery–radiotherapy–chemotherapy are used; event-free survival is between 55% and 80%.

9.12.4.6 Prognosis

•Five-year survival rate: 70–85%

•Unfavorable prognostic factors: large tumor, metastases (cerebral fluid with tumor cells), age less than 4 years, less than 90% tumor resection, chromosome deletion 17p, c-MYC amplification

•Favorable prognosis: small tumor mass, age over 4 years, total or subtotal tumor resection (more than 90% or less than 1.5 cm3 of residual total mass)

9.12.5Atypical Teratoid Rhabdoid Tumors (ATRT)

•Highly aggressive tumor

•Mostly in children <2 years of age

•Frequently with metastatic disease at diagnosis

•Alterations in gene on chromosome 22q11 with inactivation of SMARCB 1 gene (in 85%)

•Treatment: ATRA-directed intensive therapies

•Event-free survival: 14–21%

9.12.6Pineal Tumors

9.12.6.1 Frequency

• Two percent of brain tumors