{"entity":{"id":"pineal-parenchymal-tumours","kind":"cancer","name":"Pineocytoma and pineal parenchymal tumour of intermediate differentiation","aka":["Pineocytoma","Pineal parenchymal tumour of intermediate differentiation","PPTID","Pineal parenchymal tumours"],"tldr":"Pineocytoma and pineal parenchymal tumour of intermediate differentiation are rare brain tumours of the pineal gland in adults, sitting between the benign end and the aggressive pineoblastoma. Pineocytoma is cured by surgery; the intermediate tumour recurs and spreads through the spinal fluid more often, so radiotherapy is usually added after surgery.","summary":"The WHO classification of central nervous system tumours grades the pineal parenchymal tumours from pineocytoma (grade 1) through pineal parenchymal tumour of intermediate differentiation (grade 2 or 3) to pineoblastoma (grade 4); the intermediate tumour is defined molecularly by recurrent small in-frame insertions in KBTBD4 and the absence of the DROSHA deletions or DICER1 mutations of pineoblastoma (Acta Neuropathologica 2019). In the pooled analysis of 127 patients with the intermediate tumour, median age was 33 (range 4.5 to 75), the MIB-1 labelling index a median of 7 percent (range 1 to 30), and adjuvant radiotherapy was widely used with surgery (Radiotherapy and Oncology 2016).\n\nHow it differs from its parent: the brain tumours page groups all central nervous system tumours; these are adult tumours of one small midline gland, presenting with hydrocephalus and eye movement disorders, staged with spinal imaging and cerebrospinal fluid because of the risk of seeding, and diagnosed now by methylation profiling and KBTBD4 testing.\n\nHow common: under 1 percent of central nervous system tumours (Radiotherapy and Oncology 2016).\n\nTreatment: pineocytoma by complete resection alone; the intermediate tumour by resection followed by radiotherapy, focal for grade 2 and craniospinal where the grade or spinal fluid findings warrant, with chemotherapy reserved for recurrence; the papillary tumour of the pineal region and pineoblastoma have their own pages.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Pineocytoma","links":[{"label":"Radiotherapy and Oncology 2016: pineal parenchymal tumour of intermediate differentiation, individual patient data of 127 cases","url":"https://doi.org/10.1016/j.radonc.2016.10.025"},{"label":"Acta Neuropathologica 2019: KBTBD4 insertions differentiate PPTID from pineoblastoma","url":"https://doi.org/10.1007/s00401-019-01990-5"}],"tags":["subtype-page","wave4","rare"],"related":["brain-tumours","pineoblastoma","papillary-tumour-pineal-region","cns-germ-cell-tumours","choroid-plexus-carcinoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"central nervous system","burden":"Pineal parenchymal tumours are under 1 percent of central nervous system tumours; the pooled analysis of the intermediate type found 127 published patients, median age 33, with 1.6 women to each man (Radiotherapy and Oncology 2016).","subtypes":["Pineocytoma (WHO grade 1; pineal, cured by resection)","Pineal parenchymal tumour of intermediate differentiation, low grade (grade 2; KBTBD4-mutated)","Pineal parenchymal tumour of intermediate differentiation, high grade (grade 3; higher MIB-1 index, spinal seeding)"],"biomarkers":["KBTBD4 in-frame insertion","MIB-1 (Ki-67) labelling index","DNA methylation class","Spinal MRI and cerebrospinal fluid cytology for seeding"],"standardOfCare":[{"setting":"Pineocytoma","approach":"Complete resection alone.","refs":["brain-tumours"]},{"setting":"Intermediate differentiation","approach":"Resection followed by radiotherapy, focal or craniospinal by grade and staging; chemotherapy for recurrence.","refs":["brain-tumours","pineoblastoma"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"brain-tumours"},"route":"/cancers/pineal-parenchymal-tumours/","neighbours":{"cancer":[{"id":"brain-tumours","kind":"cancer","name":"Brain and spinal cord tumours (all types)","route":"/cancers/brain-tumours/"},{"id":"cns-germ-cell-tumours","kind":"cancer","name":"Central nervous system germ cell tumours (germinoma and non-germinomatous)","route":"/cancers/cns-germ-cell-tumours/"},{"id":"choroid-plexus-carcinoma","kind":"cancer","name":"Choroid plexus carcinoma","route":"/cancers/choroid-plexus-carcinoma/"},{"id":"papillary-tumour-pineal-region","kind":"cancer","name":"Papillary tumour of the pineal region","route":"/cancers/papillary-tumour-pineal-region/"},{"id":"pineoblastoma","kind":"cancer","name":"Pineoblastoma","route":"/cancers/pineoblastoma/"}]}}