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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.
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; the best strategy is undefined and chemotherapy has no established role (Neurosurgery 2019).
Rare: a systematic review found 177 published patients between 2003 and 2017, mean age 33 and 53 percent male (Neurosurgery 2019).
Gliomas infiltrate along white matter and can cross the corpus callosum, medulloblastoma sits in the cerebellum, and CNS lymphoma favours deep periventricular tissue; none spread through lymph nodes.
No conventional lymphatics: gliomas spread along white matter tracts and, rarely, through cerebrospinal fluid; medulloblastoma can seed the spine.
Same organ: Lactotroph pituitary neuroendocrine tumour (prolactinoma), Somatotroph pituitary neuroendocrine tumour (acromegaly), Corticotroph pituitary neuroendocrine tumour (Cushing disease and silent corticotroph tumour), Gonadotroph pituitary neuroendocrine tumour (non-functioning adenoma), Thyrotroph pituitary neuroendocrine tumour (TSH-secreting), Pineocytoma and pineal parenchymal tumour of intermediate differentiation, Pineoblastoma, Choroid plexus carcinoma, Glioma & glioblastoma, Primary CNS lymphoma, Medulloblastoma, Paediatric low-grade glioma, Diffuse midline glioma, H3 K27-altered (including DIPG), Atypical teratoid/rhabdoid tumour (ATRT), Ependymoma, Craniopharyngioma, Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma, Brain and spinal cord tumours (all types), Astrocytoma, IDH-mutant (grades 2 to 4), Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, Paediatric high-grade glioma (excluding diffuse midline glioma), Meningioma, Brain metastases (secondary brain tumours), Vestibular schwannoma (acoustic neuroma), Central nervous system germ cell tumours (germinoma and non-germinomatous), Spinal cord tumours (intramedullary and intradural), WNT-activated medulloblastoma, SHH-activated medulloblastoma, Group 3 and group 4 medulloblastoma (non-WNT/non-SHH)
Gross total resection where possible, with adjuvant radiotherapy commonly given for the recurrence risk; chemotherapy has no established role.
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