# Germ cell tumours of childhood and adolescence (extracranial and CNS)

Source: https://onco.cc/cancers/paediatric-germ-cell-tumours/  
OnCo record `paediatric-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 arise from the cells meant to become eggs or sperm and can appear in the gonads, lower back, chest or brain. They are among the most curable childhood cancers because they respond to cisplatin chemotherapy and release blood markers that make monitoring easy. The work now is to cure with less: surgery alone for low-risk tumours, gentler platinum drugs, and protecting hearing.

## Summary

Paediatric and adolescent germ cell tumours (GCTs) are a heterogeneous family (mature and immature teratoma, yolk sac tumour, germinoma or seminoma or dysgerminoma, embryonal carcinoma, choriocarcinoma and mixed tumours) arising in gonadal and extragonadal midline sites: sacrococcygeal region, retroperitoneum, mediastinum and the pineal and suprasellar regions of the brain. Infant tumours are mostly yolk sac tumours and teratomas with a distinct genome; adolescent tumours resemble adult testicular cancer, with 12p gain. Serum AFP and beta-hCG are diagnostic, prognostic and used for surveillance.

Malignant extracranial GCTs are cured in most children with surgery and cisplatin-based chemotherapy (PEb: cisplatin, etoposide, bleomycin in North America; JEb with carboplatin in the United Kingdom). The Malignant Germ Cell International Consortium (MaGIC) pooled COG and CCLG data to build a shared risk classification, which underpins the current joint trial AGCT1531: low-risk tumours are managed with surgery and active surveillance, with chemotherapy only on relapse, and standard-risk patients are randomised between cisplatin and carboplatin to test whether hearing and kidney toxicity can be reduced without losing cure. Cisplatin ototoxicity, the main long-term harm, can be reduced with sodium thiosulfate given after each dose (ACCL0431 and SIOPEL 6, approved for children with localised solid tumours). Salvage for relapse uses high-dose chemotherapy with stem-cell rescue as in adults.

CNS germ cell tumours are treated differently: germinomas are exquisitely radiosensitive and are cured with chemotherapy followed by reduced-dose whole-ventricular radiotherapy (SIOP CNS GCT II and ACNS1123 approaches), while non-germinomatous tumours need chemotherapy, craniospinal or focal radiotherapy and second-look surgery. Ovarian GCTs in adolescents are managed with fertility-sparing surgery and, for malignant tumours beyond stage I, the same platinum regimens. Because the adult, adolescent and paediatric worlds treat the same disease with different protocols, harmonising them is itself a research programme.

## Fields

- Kind: Cancer
- Last checked: 2026-09-10
- Also known as: Childhood extracranial germ cell tumour; CNS germ cell tumour; Germinoma; Ovarian germ cell tumour; Sacrococcygeal teratoma; Malignant germ cell tumour (children and adolescents)
- Tags: nci-coverage; paediatric; germ-cell
- Group: paediatric
- Burden: A few percent of childhood cancers overall, with peaks in infancy (sacrococcygeal and testicular teratomas and yolk sac tumours) and adolescence (ovarian, testicular and mediastinal tumours) (NCI PDQ).
- Subtypes: Teratoma (mature, immature; sacrococcygeal in infants); Yolk sac tumour (infants; AFP-secreting); Germinoma / seminoma / dysgerminoma (adolescents; CNS germinoma); Embryonal carcinoma and choriocarcinoma (hCG-secreting); Mixed malignant germ cell tumour; CNS non-germinomatous germ cell tumour; Ovarian germ cell tumour (adolescent)
- Biomarkers: Serum and cerebrospinal-fluid AFP; Serum and cerebrospinal-fluid beta-hCG; Lactate dehydrogenase; Isochromosome 12p (adolescent tumours); MaGIC risk group (site, stage, age, marker level); Audiometry before and after cisplatin; Fertility assessment and preservation

## 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/paediatric-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/paediatric-germ-cell-tumours/#overview [4 state-of-the-art points]
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/paediatric-germ-cell-tumours/#what-it-is [7 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/paediatric-germ-cell-tumours/#finding-it [7 biomarkers]
- Treating it (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/paediatric-germ-cell-tumours/#treating-it [4 settings, 1 regimen, 3 decisions with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/paediatric-germ-cell-tumours/#evidence [3 trials, 3 key papers, 6 milestones]
- The science (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/paediatric-germ-cell-tumours/#science [1 target]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/paediatric-germ-cell-tumours/where-you-are/ [1 centre]
- Living with it (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/paediatric-germ-cell-tumours/#living-with-it [18 questions, 6 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/paediatric-germ-cell-tumours/coming/ [6 medicines, 3 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/paediatric-germ-cell-tumours/data/ [36 connected records]

## Standard of care

- Low-risk malignant extracranial GCT (e.g. stage I testicular, stage I ovarian): Complete resection followed by active surveillance with serial tumour markers and imaging; chemotherapy only for relapse (AGCT1531 stratum). ([Alpha-fetoprotein (AFP)](https://onco.cc/terms/afp/), [Active surveillance](https://onco.cc/technologies/active-surveillance/), [AGCT1531](https://onco.cc/trials/agct1531/))
- Standard- and high-risk malignant extracranial GCT: Cisplatin, etoposide and bleomycin (PEb) or carboplatin-based JEb, with surgery of residual masses; sodium thiosulfate for otoprotection; high-dose chemotherapy with stem-cell rescue at relapse. ([Cisplatin](https://onco.cc/drugs/cisplatin/), [Carboplatin](https://onco.cc/drugs/carboplatin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Bleomycin](https://onco.cc/drugs/bleomycin/), [Sodium thiosulfate (otoprotectant)](https://onco.cc/drugs/sodium-thiosulfate/), [Autologous stem cell transplant (high-dose therapy)](https://onco.cc/technologies/autologous-stem-cell-transplant/))
- CNS germinoma: Platinum-based chemotherapy followed by reduced-dose whole-ventricular radiotherapy with tumour boost (SIOP CNS GCT II, ACNS1123). ([Carboplatin](https://onco.cc/drugs/carboplatin/), [Etoposide](https://onco.cc/drugs/etoposide/), [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/))
- CNS non-germinomatous GCT: Intensive platinum-based chemotherapy, second-look surgery for residual disease, then craniospinal or whole-ventricular radiotherapy depending on response and stage. ([Cisplatin](https://onco.cc/drugs/cisplatin/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [Etoposide](https://onco.cc/drugs/etoposide/), [Proton therapy](https://onco.cc/technologies/proton-therapy/))

## State of the art

- Most children and adolescents with malignant germ cell tumours are cured; the agenda is reducing toxicity, tested directly in the joint COG/CCLG AGCT1531 trial.
- Active surveillance replaces chemotherapy for low-risk resected tumours, sparing many children platinum exposure altogether.
- Sodium thiosulfate after cisplatin protects hearing without compromising cure in localised disease and is now approved for children.
- CNS germinoma is cured with chemotherapy plus reduced-dose ventricular radiotherapy, sparing cognition compared with historical craniospinal irradiation.

## Open problems

- Whether carboplatin can fully replace cisplatin without losing cures in standard-risk disease; AGCT1531 is the answer trial.
- Relapsed and platinum-refractory disease, especially mediastinal and CNS non-germinomatous tumours, where high-dose chemotherapy is the only option.
- Long-term hearing, kidney, fertility and cardiovascular effects; otoprotection, fertility preservation and survivorship clinics address them.
- Harmonising paediatric, adolescent and adult protocols so that a 17-year-old and a 19-year-old receive the same evidence-based care.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Germ_cell_tumor
- NCI PDQ: childhood extracranial germ cell tumours: https://www.cancer.gov/types/extracranial-germ-cell/hp/germ-cell-treatment-pdq
- NCI PDQ: childhood CNS germ cell tumours: https://www.cancer.gov/types/brain/hp/child-cns-germ-cell-treatment-pdq
- AGCT1531 (NCT03067181): https://clinicaltrials.gov/study/NCT03067181
- MaGIC risk classification (JCO 2015): https://doi.org/10.1200/JCO.2014.58.3369
- SIOPEL 6: sodium thiosulfate otoprotection (NEJM 2018): https://doi.org/10.1056/NEJMoa1801109

## Connected records

- cancers: [Adolescent and young adult cancers (ages 15 to 39)](https://onco.cc/cancers/aya-cancers/), [Central nervous system germ cell tumours (germinoma and non-germinomatous)](https://onco.cc/cancers/cns-germ-cell-tumours/), [Childhood cancers (all types)](https://onco.cc/cancers/childhood-cancers/), [Extragonadal germ cell tumour](https://onco.cc/cancers/extragonadal-germ-cell-tumour/), [Hepatoblastoma](https://onco.cc/cancers/hepatoblastoma/), [Neuroblastoma (paediatric)](https://onco.cc/cancers/neuroblastoma/), [Ovarian cancer](https://onco.cc/cancers/ovarian/), [Rare cancers of childhood (NCI PDQ umbrella)](https://onco.cc/cancers/rare-childhood-cancers/), [Retroperitoneal germ cell tumour](https://onco.cc/cancers/retroperitoneal-germ-cell-tumour/), [Testicular germ cell tumours](https://onco.cc/cancers/testicular/), [Yolk sac tumour of the testis, postpubertal type](https://onco.cc/cancers/yolk-sac-tumour-postpubertal/)
- technologies: [Active surveillance](https://onco.cc/technologies/active-surveillance/), [AFP, hCG and LDH in germ cell tumours (IGCCCG risk groups)](https://onco.cc/technologies/germ-cell-tumour-markers/), [Autologous stem cell transplant (high-dose therapy)](https://onco.cc/technologies/autologous-stem-cell-transplant/), [Cytotoxic chemotherapy](https://onco.cc/technologies/cytotoxic-chemotherapy/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Oncofertility and fertility preservation](https://onco.cc/technologies/fertility-preservation/), [Proton therapy](https://onco.cc/technologies/proton-therapy/), [Survivorship care and late-effects surveillance](https://onco.cc/technologies/survivorship-care-plan/)
- drugs: [Bleomycin](https://onco.cc/drugs/bleomycin/), [Carboplatin](https://onco.cc/drugs/carboplatin/), [Cisplatin](https://onco.cc/drugs/cisplatin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [Sodium thiosulfate (otoprotectant)](https://onco.cc/drugs/sodium-thiosulfate/)
- companies: [Children's Cancer and Leukaemia Group](https://onco.cc/companies/cclg/), [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: [Adolescent and young adult (AYA) oncology](https://onco.cc/terms/aya-oncology/), [Alpha-fetoprotein (AFP)](https://onco.cc/terms/afp/), [Late effects and survivorship toxicity](https://onco.cc/terms/late-effects/), [Retroperitoneum](https://onco.cc/terms/retroperitoneum/)
- pairings: [Caution: bleomycin lung toxicity, especially with brentuximab or G-CSF](https://onco.cc/pairings/bleomycin-omission-caution/)
- 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/), [Revised risk classification for pediatric extracranial germ cell tumors based on 25 years of clinical trial data from the United Kingdom and United States](https://onco.cc/key-papers/paper-frazier-j-clin-oncol/), [Sodium Thiosulfate for Protection from Cisplatin-Induced Hearing Loss](https://onco.cc/key-papers/paper-brock-n-engl-j-med/)
- trials: [ACNS1123](https://onco.cc/trials/acns1123/), [AGCT1531](https://onco.cc/trials/agct1531/), [Treatment of Newly Diagnosed Central Malignant Germ Cell Tumor People With Teniposide Injection Combined With Cisplatin](https://onco.cc/trials/nct07188441/)

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