# Central nervous system germ cell tumours (germinoma and non-germinomatous)

Source: https://onco.cc/cancers/cns-germ-cell-tumours/  
OnCo record `cns-germ-cell-tumours` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Summary

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: Intracranial germ cell tumour; CNS germinoma; Pineal germinoma; Suprasellar germinoma; Non-germinomatous germ cell tumour (NGGCT); Intracranial teratoma
- Tags: subtype-page; cns; paediatric
- Group: central nervous system
- Burden: 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.
- Subtypes: Germinoma of the pineal or suprasellar region (bifocal when both); Non-germinomatous germ cell tumour: embryonal carcinoma, yolk sac tumour, choriocarcinoma, mixed; Mature and immature teratoma (surgical disease; growing teratoma after chemotherapy); Teratoma with somatic-type malignancy; Metastatic or disseminated CNS germ cell tumour (cerebrospinal fluid spread)
- Biomarkers: Alpha-fetoprotein in serum and cerebrospinal fluid; Beta-hCG in serum and cerebrospinal fluid; Placental alkaline phosphatase and c-KIT on germinoma cells; Cerebrospinal fluid cytology and spinal MRI for staging; Pituitary hormone panel for suprasellar tumours

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/cns-germ-cell-tumours/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/cns-germ-cell-tumours/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/cns-germ-cell-tumours/#what-it-is [5 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/cns-germ-cell-tumours/#finding-it [5 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/cns-germ-cell-tumours/#treating-it [5 settings, 4 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/cns-germ-cell-tumours/#evidence [2 trials, 4 key papers, 5 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/cns-germ-cell-tumours/#science [1 target]
- Countries and centres (own page): 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. https://onco.cc/cancers/cns-germ-cell-tumours/where-you-are/ [1 centre]
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/cns-germ-cell-tumours/#living-with-it [19 questions, 6 red cards]
- Pipeline and open problems (own page): 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. https://onco.cc/cancers/cns-germ-cell-tumours/coming/ [5 medicines, 2 trials, 4 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/cns-germ-cell-tumours/data/ [30 connected records]

## Standard of care

- Localised germinoma: 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. ([Carboplatin](https://onco.cc/drugs/carboplatin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Proton therapy](https://onco.cc/technologies/proton-therapy/), [ACNS1123](https://onco.cc/trials/acns1123/))
- Disseminated germinoma: Craniospinal irradiation with boosts, with or without chemotherapy. ([IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Proton therapy](https://onco.cc/technologies/proton-therapy/))
- Non-germinomatous germ cell tumour: 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). ([Cisplatin](https://onco.cc/drugs/cisplatin/), [Carboplatin](https://onco.cc/drugs/carboplatin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Proton therapy](https://onco.cc/technologies/proton-therapy/), [ACNS0122](https://onco.cc/trials/acns0122/), [ACNS1123](https://onco.cc/trials/acns1123/))
- Teratoma: Complete surgical resection; growing teratoma after chemotherapy is also managed surgically. ([MRI](https://onco.cc/technologies/mri/))
- Relapse: High-dose chemotherapy (thiotepa-based) with autologous stem cell rescue, with re-irradiation where possible. ([Thiotepa](https://onco.cc/drugs/thiotepa/), [Autologous stem cell transplant (high-dose therapy)](https://onco.cc/technologies/autologous-stem-cell-transplant/), [Re-irradiation](https://onco.cc/terms/re-irradiation/))

## State of the art

- Germinoma is cured in the great majority with chemotherapy and reduced-field radiotherapy, and each trial generation has lowered the radiation dose and volume.
- Tumour markers in blood and spinal fluid allow diagnosis and risk assignment with less surgery than any other brain tumour.
- Proton therapy is reducing the endocrine and cognitive cost of ventricular and craniospinal irradiation in children.

## Open problems

- Which non-germinomatous patients can safely avoid craniospinal irradiation.
- Late endocrine, cognitive and vascular effects of radiotherapy in survivors treated as teenagers.
- Relapsed non-germinomatous tumours have poor salvage rates.
- Why incidence is several times higher in East Asia.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Germinoma
- Wikipedia: https://en.wikipedia.org/wiki/Germinoma
- NCI PDQ: childhood CNS germ cell tumours: https://www.cancer.gov/types/brain/hp/child-cns-germ-cell-treatment-pdq
- SIOP CNS GCT 96 germinoma results (Neuro-Oncology 2013): https://doi.org/10.1093/neuonc/not019

## Connected records

- cancers: [Atypical teratoid/rhabdoid tumour (ATRT)](https://onco.cc/cancers/atrt/), [Brain and spinal cord tumours (all types)](https://onco.cc/cancers/brain-tumours/), [Craniopharyngioma](https://onco.cc/cancers/craniopharyngioma/), [Extragonadal germ cell tumour](https://onco.cc/cancers/extragonadal-germ-cell-tumour/), [Germ cell tumours of childhood and adolescence (extracranial and CNS)](https://onco.cc/cancers/paediatric-germ-cell-tumours/), [Mediastinal germ cell tumour](https://onco.cc/cancers/mediastinal-germ-cell-tumour/), [Medulloblastoma](https://onco.cc/cancers/medulloblastoma/), [Pineocytoma and pineal parenchymal tumour of intermediate differentiation](https://onco.cc/cancers/pineal-parenchymal-tumours/), [Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma](https://onco.cc/cancers/pituitary-tumours/), [Testicular germ cell tumours](https://onco.cc/cancers/testicular/)
- technologies: [Autologous stem cell transplant (high-dose therapy)](https://onco.cc/technologies/autologous-stem-cell-transplant/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [MRI](https://onco.cc/technologies/mri/), [Proton therapy](https://onco.cc/technologies/proton-therapy/)
- drugs: [Carboplatin](https://onco.cc/drugs/carboplatin/), [Cisplatin](https://onco.cc/drugs/cisplatin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [Thiotepa](https://onco.cc/drugs/thiotepa/)
- companies: [Children's Oncology Group (COG)](https://onco.cc/companies/childrens-oncology-group/)
- institutions: [SIOP Europe (European Society for Paediatric Oncology)](https://onco.cc/institutions/siop-europe/)
- terms: [Alpha-fetoprotein (AFP)](https://onco.cc/terms/afp/), [Late effects and survivorship toxicity](https://onco.cc/terms/late-effects/), [Re-irradiation](https://onco.cc/terms/re-irradiation/)
- bottlenecks: [Rare and paediatric cancers without markets](https://onco.cc/bottlenecks/b-rare-cancers/), [Survivorship and late effects are neglected](https://onco.cc/bottlenecks/b-survivorship/)
- key papers: [ACNS1123: response-based reduced radiotherapy for localised CNS non-germinomatous germ cell tumours](https://onco.cc/key-papers/paper-acns1123-nggct-reduced-radiotherapy-fangusaro-jco-2019/), [ACNS1123: response-based reduced-dose whole-ventricular radiotherapy for localised germinoma](https://onco.cc/key-papers/paper-acns1123-germinoma-neuro-oncology-2022/), [SIOP CNS GCT 96: outcomes for children and adults with intracranial germinoma treated with radiotherapy alone or chemotherapy plus reduced radiotherapy](https://onco.cc/key-papers/paper-siop-cns-gct-96-calaminus-neuro-oncology-2013/), [The 2021 WHO classification of tumours of the central nervous system: a summary](https://onco.cc/key-papers/paper-who-2021-cns-louis-neuro-oncology-2021/)
- trials: [ACNS0122](https://onco.cc/trials/acns0122/), [ACNS1123](https://onco.cc/trials/acns1123/)

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JSON: https://onco.cc/api/v1/entities/cns-germ-cell-tumours.json