Loading
4 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Pineoblastoma is a rare, aggressive brain tumour of the pineal gland, mostly in children, made of primitive cells like those of medulloblastoma. It is treated with surgery, radiotherapy to the whole brain and spine in children old enough, and chemotherapy; survival is lowest in infants, in whom radiotherapy is limited by age, and the subgroups found in 2020 now guide treatment.
Pineoblastoma is the grade 4 embryonal tumour of the pineal gland in the WHO central nervous system classification (NCI PDQ). DNA methylation profiling of 72 core cases from the Rare Brain Tumor Consortium defined five molecular subgroups: groups 1 and 2 almost exclusively carried homozygous loss-of-function alterations in the microRNA biogenesis genes DICER1, DROSHA and DGCR8, and the subgroups differed in age, metastasis and survival (Acta Neuropathologica 2020). The pooled cohort of 178 cases from the Children's Oncology Group and institutional series found age the strongest determinant of outcome, with sex-specific differences, and noted that no pineoblastoma-specific trial has ever been run (Neuro-Oncology Advances 2022). Pineoblastoma with bilateral retinoblastoma is the trilateral retinoblastoma of RB1 carriers.
How it differs from its parent: it is treated on the medulloblastoma and CNS embryonal tumour protocols (craniospinal irradiation and chemotherapy) rather than as a glioma, it seeds the spinal fluid, and it is one of the DICER1 syndrome tumours, so germline testing and family screening follow the diagnosis.
| Setting | Approach | Guideline |
|---|---|---|
| All cases | Maximal safe resection, craniospinal irradiation with boost in older children, platinum-based chemotherapy; high-dose chemotherapy with stem cell rescue to spare radiotherapy in infants, as on the CNS embryonal tumour summary. | not mapped |