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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.
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.
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).
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, Papillary tumour of the pineal region, 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)
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.
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Query for this cancer: (TITLE:"Pineoblastoma" OR ABSTRACT:"Pineoblastoma" OR TITLE:"Pineal embryonal tumour" OR ABSTRACT:"Pineal embryonal tumour" OR TITLE:"Pineoblastoma WHO grade 4" OR ABSTRACT:"Pineoblastoma WHO grade 4" OR TITLE:"Trilateral retinoblastoma pineoblastoma with bilateral retinoblastoma" OR ABSTRACT:"Trilateral retinoblastoma pineoblastoma with bilateral retinoblastoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Pineoblastoma, not a curated reading list.
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Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
Fatal if given intrathecally: label all syringes.
Dose by Calvert formula using GFR (see the calculators).
Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
Reactions around the moment a drug is given: chills, fever or breathlessness from antibodies (infusion reactions), true allergy (hypersensitivity, rarely anaphylaxis), and leakage of a damaging drug into tissue around the vein (extravasation).
During chemotherapy for triple-negative breast cancer a temperature over 37.5 C or below 36 C, shivering, or feeling unwell even with a normal temperature means ringing the hospital's 24-hour line straight away; breathing very fast, confusion, mottled skin or no urine in a day means 999.
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