{"entity":{"id":"papillary-tumour-pineal-region","kind":"cancer","name":"Papillary tumour of the pineal region","aka":["PTPR","Papillary tumor of the pineal region"],"tldr":"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 and often given radiotherapy, and it has a marked tendency to come back locally.","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).\n\nHow 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.\n\nHow common: no incidence figure; 177 published cases in 14 years (Neurosurgery 2019).\n\nTreatment: gross total resection where possible, with adjuvant radiotherapy commonly given because of the recurrence rate; the best strategy is undefined and chemotherapy has no established role (Neurosurgery 2019).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Papillary_tumor_of_the_pineal_region","links":[{"label":"Brain Pathology 2016: papillary tumour of the pineal region, a distinct molecular entity (24 cases)","url":"https://doi.org/10.1111/bpa.12282"},{"label":"Neurosurgery 2019: papillary tumour of the pineal region, systematic review of 177 patients","url":"https://doi.org/10.1093/neuros/nyz062"}],"tags":["subtype-page","wave4","rare"],"related":["brain-tumours","pineal-parenchymal-tumours","pineoblastoma","ependymoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"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"],"standardOfCare":[{"setting":"All cases","approach":"Gross total resection where possible, with adjuvant radiotherapy commonly given for the recurrence risk; chemotherapy has no established role.","refs":["brain-tumours","ependymoma"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"brain-tumours"},"route":"/cancers/papillary-tumour-pineal-region/","neighbours":{"cancer":[{"id":"brain-tumours","kind":"cancer","name":"Brain and spinal cord tumours (all types)","route":"/cancers/brain-tumours/"},{"id":"ependymoma","kind":"cancer","name":"Ependymoma","route":"/cancers/ependymoma/"},{"id":"pineoblastoma","kind":"cancer","name":"Pineoblastoma","route":"/cancers/pineoblastoma/"},{"id":"pineal-parenchymal-tumours","kind":"cancer","name":"Pineocytoma and pineal parenchymal tumour of intermediate differentiation","route":"/cancers/pineal-parenchymal-tumours/"}]}}