# Papillary tumour of the pineal region

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

## TL;DR

Papillary tumour of the pineal region is a rare brain tumour of young adults arising near the pineal gland from cells of the embryonic subcommissural organ. It usually presents with raised pressure from blocked spinal fluid, is removed surgically at a centre that does pineal surgery and often given radiotherapy, and it recurs locally more often than it seeds the spine.

## Summary

The WHO central nervous system classification lists papillary tumour of the pineal region as a neuroepithelial tumour of the pineal region, grade 2 or 3, thought to derive from the subcommissural organ; molecular studies of 24 cases found loss of chromosome 10 in all 13 examined, losses of chromosomes 3 and 22q and gains of 8p and 12 commonly, a methylation profile that separates it from ependymoma and pineal parenchymal tumours, two methylation subgroups with the more methylated one tending to shorter progression-free survival, and overexpression of SPDEF, a subcommissural organ gene (Brain Pathology 2016). In the review of 177 patients, intracranial hypertension and hydrocephalus dominated the presentation, 82 percent had surgery, and gross total resection was among the factors associated with survival at 36 months (Neurosurgery 2019).

How it differs from its parent: a distinct entity of the pineal region that mimics ependymoma and pineal parenchymal tumours histologically, diagnosed reliably only by methylation profiling, and marked by frequent local recurrence rather than spinal seeding.

How common: no incidence figure; 177 published cases in 14 years (Neurosurgery 2019).

Treatment: gross total resection where possible, with adjuvant radiotherapy commonly given because of the recurrence rate; with only 177 published cases there is no randomised evidence to set a best strategy, and chemotherapy has no established role, so management is agreed by a neuro-oncology multidisciplinary team (Neurosurgery 2019).

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: PTPR; Papillary tumor of the pineal region
- Tags: subtype-page; wave4; rare
- Group: central nervous system
- Burden: Rare: a systematic review found 177 published patients between 2003 and 2017, mean age 33 and 53 percent male (Neurosurgery 2019).
- Subtypes: Papillary tumour of the pineal region, methylation group 1 (less methylated); Papillary tumour of the pineal region, methylation group 2 (more methylated; shorter progression-free survival); Recurrent papillary tumour of the pineal region (local; repeat surgery or radiotherapy)
- Biomarkers: Loss of chromosome 10 (all cases examined); DNA methylation class distinguishing it from ependymoma and pineal parenchymal tumours; SPDEF and cytokeratin expression; Extent of resection

## 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/papillary-tumour-pineal-region/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/papillary-tumour-pineal-region/#overview
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/papillary-tumour-pineal-region/#what-it-is [3 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/papillary-tumour-pineal-region/#finding-it [4 biomarkers]
- Treatment (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/papillary-tumour-pineal-region/#treating-it [1 setting]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/papillary-tumour-pineal-region/#evidence
- Biology and targets (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/papillary-tumour-pineal-region/#science
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/papillary-tumour-pineal-region/where-you-are/
- Decisions and support (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/papillary-tumour-pineal-region/#living-with-it [7 questions]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/papillary-tumour-pineal-region/coming/
- 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/papillary-tumour-pineal-region/data/

## Standard of care

- All cases: Gross total resection where possible, with adjuvant radiotherapy commonly given for the recurrence risk; chemotherapy has no established role. ([Brain and spinal cord tumours (all types)](https://onco.cc/cancers/brain-tumours/), [Ependymoma](https://onco.cc/cancers/ependymoma/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Papillary_tumor_of_the_pineal_region
- Brain Pathology 2016: papillary tumour of the pineal region, a distinct molecular entity (24 cases): https://doi.org/10.1111/bpa.12282
- Neurosurgery 2019: papillary tumour of the pineal region, systematic review of 177 patients: https://doi.org/10.1093/neuros/nyz062

## Connected records

- cancers: [Brain and spinal cord tumours (all types)](https://onco.cc/cancers/brain-tumours/), [Ependymoma](https://onco.cc/cancers/ependymoma/), [Pineoblastoma](https://onco.cc/cancers/pineoblastoma/), [Pineocytoma and pineal parenchymal tumour of intermediate differentiation](https://onco.cc/cancers/pineal-parenchymal-tumours/)

---
JSON: https://onco.cc/api/v1/entities/papillary-tumour-pineal-region.json