Loading
Germ cell tumours of the brain grow near the pineal gland or above the pituitary in teenagers. The commonest kind, germinoma, is so sensitive to radiation and chemotherapy that most patients are cured; the other kinds need stronger chemotherapy and radiotherapy, and doctors measure two proteins in the blood and spinal fluid to tell them apart and to follow treatment.
Central nervous system germ cell tumours arise in the midline, in the pineal region (more often in boys) and suprasellar region (with diabetes insipidus and hormone deficits), sometimes at both sites (bifocal). WHO 2021 lists germinoma, embryonal carcinoma, yolk sac tumour, choriocarcinoma, mature and immature teratoma, teratoma with somatic-type malignancy and mixed germ cell tumour; clinically they divide into germinoma and non-germinomatous germ cell tumours (NGGCT). Alpha-fetoprotein and beta-hCG in serum and cerebrospinal fluid are diagnostic and prognostic: marked elevation indicates NGGCT and can spare biopsy, while normal or mildly raised hCG with typical imaging leads to biopsy to confirm germinoma. Staging requires spinal MRI and cerebrospinal fluid cytology. Klinefelter and Down syndromes raise risk, and KIT and RAS pathway mutations are frequent.
Germinoma is exquisitely radiosensitive. Craniospinal irradiation alone cured more than nine in ten patients with localised disease in SIOP CNS GCT 96 (five-year event-free survival above 90 percent), and the same trial showed that carboplatin, etoposide and ifosfamide followed by focal radiotherapy gave similar survival but more relapses in the ventricles; SIOP CNS GCT II and the Children's Oncology Group's ACNS1123 therefore adopted chemotherapy followed by reduced-dose whole-ventricular irradiation with a tumour boost, the current standard for localised germinoma, with craniospinal irradiation kept for disseminated disease. NGGCT is treated with intensive platinum-based chemotherapy (cisplatin or carboplatin with etoposide and ifosfamide), second-look surgery for residual masses, which often prove to be teratoma, and then radiotherapy; ACNS0122 used craniospinal irradiation after chemotherapy with good results, and attempts to reduce to whole-ventricular fields in ACNS1123 were tempered by spinal relapses. High-dose chemotherapy with stem cell rescue is used at relapse.
The agenda is reducing late effects without losing cure: lowering radiation dose and volume, proton therapy, and defining which NGGCT patients can safely avoid craniospinal irradiation. Long-term survivors need endocrine replacement, neurocognitive support and fertility counselling. International harmonisation of the SIOP and COG approaches, which historically differed on the role of chemotherapy for germinoma, is progressing through joint trials.
A few percent of childhood and adolescent brain tumours in Western countries and several times commoner in East Asia; peak age is the second decade with a male excess, and germinoma is one of the most curable brain tumours.
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, 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), Spinal cord tumours (intramedullary and intradural), WNT-activated medulloblastoma, SHH-activated medulloblastoma, Group 3 and group 4 medulloblastoma (non-WNT/non-SHH)
Nothing recorded yet.
Nothing recorded yet.
Background: Alpha-fetoprotein (AFP). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Platinum-based chemotherapy (carboplatin and etoposide, with ifosfamide in the SIOP schedule) followed by reduced-dose whole-ventricular irradiation with a tumour boost (SIOP CNS GCT II, ACNS1123); craniospinal irradiation alone remains an alternative in adults.
Craniospinal irradiation with boosts, with or without chemotherapy.
Intensive cisplatin or carboplatin, etoposide and ifosfamide chemotherapy, second-look surgery for residual disease, then craniospinal or whole-ventricular irradiation depending on stage and response (ACNS0122, ACNS1123, SIOP CNS GCT II).
Complete surgical resection; growing teratoma after chemotherapy is also managed surgically.
High-dose chemotherapy (thiotepa-based) with autologous stem cell rescue, with re-irradiation where possible.
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Response-adapted, reduced-dose whole-ventricular radiotherapy after chemotherapy is now a standard for localised germinoma in North American protocols.
Every brain tumour page on this site uses these names and grades; a tumour called glioblastoma before 2021 may now be an IDH-mutant astrocytoma with a different outlook and treatment.
Whole-ventricular irradiation with a boost is a reasonable option after a good response to chemotherapy, but the spinal failure pattern shaped the next trial and keeps craniospinal irradiation as an alternative.
Chemotherapy followed by whole-ventricular irradiation with a tumour boost, rather than craniospinal irradiation, is the standard for localised germinoma, sparing children the neurocognitive and endocrine cost of wider fields.
Query for this cancer: (TITLE:"Central nervous system germ cell tumours" OR ABSTRACT:"Central nervous system germ cell tumours" OR TITLE:"germinoma and non-germinomatous" OR ABSTRACT:"germinoma and non-germinomatous" OR TITLE:"Intracranial germ cell tumour" OR ABSTRACT:"Intracranial germ cell tumour" OR TITLE:"CNS germinoma" OR ABSTRACT:"CNS germinoma" OR TITLE:"Pineal germinoma" OR ABSTRACT:"Pineal germinoma" OR TITLE:"Suprasellar germinoma" OR ABSTRACT:"Suprasellar germinoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Central nervous system germ cell tumours (germinoma and non-germinomatous), not a curated reading list.
Basis for risk-adapted therapy in Japan and later international trials.
Calaminus and colleagues (Neuro-Oncology): equivalent survival, more ventricular relapses with focal fields; whole-ventricular irradiation adopted.
Goldman and colleagues (JCO).
The targets of this cancer's medicines and the ones linked to it directly.
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.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Dose by Calvert formula using GFR (see the calculators).
Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
Reduce to 75% for CrCl 15-50.
The leukaemia risk after chemotherapy was described in the era of mustards and etoposide, and it did not stay there. Platinum drugs carry it, PARP inhibitors raise it about two and a half times against placebo, and lenalidomide with oral melphalan raises it nearly fivefold against melphalan alone. The absolute numbers are small, but the choice of partner drug is sometimes a real decision.
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).
See all on the product pages:CarboplatinCisplatinEtoposideIfosfamideThiotepa·Printable cards in the navigator
Newly diagnosed? Read the first 60 days with Central nervous system germ cell tumours, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
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.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.