# 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

## 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/))
- 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/))
- 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: [Brain and spinal cord tumours (all types)](https://onco.cc/cancers/brain-tumours/), [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/), [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/)
- institutions: [Children's Oncology Group (COG)](https://onco.cc/institutions/childrens-oncology-group/), [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/)

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