# Pineoblastoma

Source: https://onco.cc/cancers/pineoblastoma/  
OnCo record `pineoblastoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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; infants do worst, and molecular subgroups discovered in 2020, including ones linked to the DICER1 gene, are starting to guide treatment.

## Summary

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.

How common: no incidence figure; 178 cases in 30 years across the pooled series (Neuro-Oncology Advances 2022).

Treatment: maximal safe resection, craniospinal irradiation with a boost in children over about three years, and platinum-based chemotherapy, with high-dose chemotherapy and stem cell rescue used to defer or replace radiotherapy in infants, as the PDQ embryonal tumour summary describes; molecular subgroup and DICER1 status are being built into current protocols.

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: Pineal embryonal tumour; Pineoblastoma (WHO grade 4); Trilateral retinoblastoma (pineoblastoma with bilateral retinoblastoma)
- Tags: subtype-page; wave4; rare
- Group: paediatric
- Burden: Rare: the Rare Brain Tumor Consortium registry gathered 91 patients from 29 centres (Acta Neuropathologica 2020) and a pooled North American and Australian cohort held 178 cases over 30 years (Neuro-Oncology Advances 2022).
- Subtypes: Pineal pineoblastoma, DICER1- or DROSHA-altered (molecular groups 1 and 2; DICER1 syndrome); Pineoblastoma, MYC or FOXR2-activated group; Pineoblastoma, RB1-altered (trilateral retinoblastoma); Pineoblastoma in infants (worst outcome; radiotherapy-sparing protocols)
- Biomarkers: DNA methylation subgroup; DICER1, DROSHA and DGCR8 alterations; germline DICER1 testing; RB1 status; Spinal MRI and cerebrospinal fluid cytology

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/pineoblastoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/pineoblastoma/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/pineoblastoma/#what-it-is [4 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/pineoblastoma/#finding-it [4 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/pineoblastoma/#treating-it [1 setting, 1 decision with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/pineoblastoma/#evidence
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/pineoblastoma/#science [1 target]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/pineoblastoma/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/pineoblastoma/#living-with-it [8 questions, 6 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/pineoblastoma/coming/ [4 medicines]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/pineoblastoma/data/ [11 connected records]

## Standard of care

- 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. ([Brain and spinal cord tumours (all types)](https://onco.cc/cancers/brain-tumours/), [Medulloblastoma](https://onco.cc/cancers/medulloblastoma/), [Cisplatin](https://onco.cc/drugs/cisplatin/), [Carboplatin](https://onco.cc/drugs/carboplatin/), [Vincristine](https://onco.cc/drugs/vincristine/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Pineoblastoma
- NCI PDQ: childhood medulloblastoma and other CNS embryonal tumours treatment: https://www.cancer.gov/types/brain/patient/child-cns-embryonal-treatment-pdq
- Acta Neuropathologica 2020: pineoblastoma molecular subgroups, Rare Brain Tumor Consortium registry: https://doi.org/10.1007/s00401-019-02111-y
- Neuro-Oncology Advances 2022: paediatric pineoblastoma, pooled outcomes of 178 North American and Australian cases: https://doi.org/10.1093/noajnl/vdac056

## Connected records

- cancers: [Brain and spinal cord tumours (all types)](https://onco.cc/cancers/brain-tumours/), [Choroid plexus carcinoma](https://onco.cc/cancers/choroid-plexus-carcinoma/), [Medulloblastoma](https://onco.cc/cancers/medulloblastoma/), [Papillary tumour of the pineal region](https://onco.cc/cancers/papillary-tumour-pineal-region/), [Pineocytoma and pineal parenchymal tumour of intermediate differentiation](https://onco.cc/cancers/pineal-parenchymal-tumours/), [Pleuropulmonary blastoma (types I, Ir, II and III)](https://onco.cc/cancers/pleuropulmonary-blastoma-dicer1/), [Retinoblastoma](https://onco.cc/cancers/retinoblastoma/)
- targets: [DICER1](https://onco.cc/targets/dicer1/)
- drugs: [Carboplatin](https://onco.cc/drugs/carboplatin/), [Cisplatin](https://onco.cc/drugs/cisplatin/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Vincristine](https://onco.cc/drugs/vincristine/)

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JSON: https://onco.cc/api/v1/entities/pineoblastoma.json