Array-based karyotyping of 260<unk> s resulted in the following clinical subgroups based on cytogenetic profiles: * Poor prognosis: gain of 6q or amplification of MYC or MYCN * Intermediate: gain of 17q or an i(17q) without gain of 6q or amplification of MYC or MYCN * Excellent prognosis: 6q and 17q balanced or 6q deletion Transcriptional profiling shows the existence of four main subgroups (Wnt, Shh, Group 3, and Group 4). * Very good prognosis: WNT group, CTNNB1 mutation * Infants good prognosis, others intermediate: SHH group, PTCH1/SMO/SUFU mutation, GLI2 amplification, or MYCN amplification * Poor prognosis: Group 3, MYC amplification, photoreceptor/GABAergic gene expression * Intermediate prognosis: Group 4, gene expression of neuronal/glutamatergic, CDK6 amplification, MYCN amplification Survival The historical cumulative relative survival rate for all age groups and histology follow-up was 60%, 52%, and 47% at 5 years, 10 years, and 20 years, respectively. Patients diagnosed with a<unk>  or PNET are 50 times more likely to die than a matched member of the general population. The most recent population-based (SEER) 5-year relative survival rates are 69% overall: 72% in children (1–9 years) and 67% in adults (20+ years). The 20-year survival rate is 51% in children. Children and adults have different survival profiles, with adults faring worse than children only after the fourth year after diagnosis (after controlling for increased background mortality). Before the fourth year, survival probabilities are nearly identical. Long-term sequelae of standard treatment include hypothalamic-pituitary and thyroid dysfunction and intellectual impairment. The hormonal and intellectual deficits created by these therapies causes significant impairment of the survivors. In current clinical studies, the patients are divided into low-, standard- and high-risk groups: * Depending on the study, healing rates of up to 100% are achieved in the low-risk group (usually WNT-activated). The current efforts are therefore moving in the direction of reducing the intensity of the therapy and thus the negative long-term consequences while confirming the high healing rates. * In the HIT-SIOP PNET 4 study, in which 340 children and adolescents of the standard-risk group between the ages of four and 21 from several European countries participated, the 5-year survival rate was between 85% and 87% depending on the randomization. Around 78% of the patients remained without relapse for 5 years and are therefore considered to be cured. After a relapse, the prognosis was very poor. Despite intensive treatment, only four of 66 patients were still alive 5 years after a relapse. * A US study involved 161 patients between the ages of three and 21 with a high-risk profile. Depending on the randomization, half of the patients additionally received carboplatin daily during the radiation. The 5-year survival rate of patients with carboplatin was 82%, those without 68%. The European SIOP PNET 5 study is currently taking place and will run until April 2024, in which an attempt is made to confirm the promising results with carboplatin during irradiation in the standard risk group.
Medulloblastoma
Sialoblastoma
Pineoblastoma
Ameloblastoma
Hemangioblastoma
Glioblastoma
Neurothekeoma
Subependymoma