# Extragonadal germ cell tumour

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

## TL;DR

Extragonadal germ cell tumours are the same cancers as testicular germ cell tumours but arising in the midline of the body, most often the chest or the back of the abdomen. Seminomas are highly curable with chemotherapy; non-seminomas of the chest are the hardest germ cell tumours to cure and are treated with intensive chemotherapy followed by surgery.

## Summary

Germ cell tumours occasionally arise from germ cells left along the midline during development, in the mediastinum, retroperitoneum, or the pineal and suprasellar regions of the brain, without a testicular primary. They are diagnosed by biopsy or by the tumour markers alpha-fetoprotein, human chorionic gonadotropin and lactate dehydrogenase, and treated on the same principles as testicular cancer: cisplatin-based chemotherapy, usually BEP (bleomycin, etoposide, cisplatin) for three or four cycles, followed by surgery to remove residual masses. Retroperitoneal and mediastinal seminomas do as well as their testicular counterparts, but primary mediastinal non-seminomatous tumours are classed as poor risk by the IGCCCG system, carry associations with Klinefelter syndrome and with haematological cancers, and are the main setting where high-dose chemotherapy with stem cell rescue is considered at relapse.

## Fields

- Kind: Cancer
- Last checked: 2026-09-04
- Also known as: Extragonadal Germ Cell Tumor; Extragonadal germ cell tumor; Mediastinal germ cell tumour; Retroperitoneal germ cell tumour; Primary mediastinal nonseminomatous germ cell tumour
- Tags: rare
- Group: genitourinary
- Burden: Rare: only a few percent of germ cell tumours arise outside the testis or ovary, most in the chest (mediastinum) or the back of the abdomen (retroperitoneum) in young men; no separate GLOBOCAN count.
- Subtypes: Mediastinal seminoma; Primary mediastinal non-seminomatous germ cell tumour; Retroperitoneal germ cell tumour; Intracranial germ cell tumours (pineal, suprasellar); Sacrococcygeal teratoma (infants)
- Biomarkers: Alpha-fetoprotein (AFP); Human chorionic gonadotropin (hCG); Lactate dehydrogenase (LDH); IGCCCG risk group (mediastinal non-seminoma is poor risk); Isochromosome 12p

## 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/extragonadal-germ-cell-tumour/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/extragonadal-germ-cell-tumour/#overview [2 subtypes, 4 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/extragonadal-germ-cell-tumour/#what-it-is [7 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/extragonadal-germ-cell-tumour/#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/extragonadal-germ-cell-tumour/#treating-it [3 settings, 3 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/extragonadal-germ-cell-tumour/#evidence [3 trials, 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/extragonadal-germ-cell-tumour/#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/extragonadal-germ-cell-tumour/where-you-are/
- 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/extragonadal-germ-cell-tumour/#living-with-it [15 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/extragonadal-germ-cell-tumour/coming/ [5 medicines, 3 trials, 2 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/extragonadal-germ-cell-tumour/data/ [20 connected records]

## Standard of care

- Seminoma (mediastinal or retroperitoneal): BEP for three cycles or EP for four; radiotherapy only for small retroperitoneal disease; PET to assess residual masses. ([Bleomycin](https://onco.cc/drugs/bleomycin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Cisplatin](https://onco.cc/drugs/cisplatin/))
- Non-seminoma, retroperitoneal: BEP for three or four cycles by IGCCCG risk group, then resection of residual masses over one centimetre. ([Bleomycin](https://onco.cc/drugs/bleomycin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Cisplatin](https://onco.cc/drugs/cisplatin/))
- Non-seminoma, mediastinal (poor risk): Four cycles of BEP or VIP, then surgery of residual disease; consider high-dose chemotherapy with autologous stem cell rescue at relapse; treat in a specialist centre. ([Etoposide](https://onco.cc/drugs/etoposide/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [Cisplatin](https://onco.cc/drugs/cisplatin/))

## State of the art

- Extragonadal seminoma is cured as often as testicular seminoma: the 2002 international pooled analysis of 635 patients reported 88 percent overall survival whether the primary was mediastinal or retroperitoneal.
- Non-seminoma depends on where it starts: about 63 percent of patients with retroperitoneal primaries and 49 percent with mediastinal primaries were alive in the same analysis, which is why primary mediastinal non-seminoma sits in the IGCCCG poor-risk group by definition.
- Adverse factors for non-seminoma are liver, lung or brain metastases, a mediastinal primary and a raised beta-hCG; these guide the choice of four cycles and early referral to a high-volume centre.
- Salvage is the open question: the randomised TIGER trial comparing conventional-dose TIP with high-dose chemotherapy and stem cell rescue has finished recruiting and its result will set the standard for relapse.

## Open problems

- Cure rates for mediastinal non-seminoma remain well below those of testicular primaries.
- The role of high-dose chemotherapy in first-line poor-risk disease is still being tested (TIGER trial).

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Germ_cell_tumor
- NCI: Extragonadal germ cell tumors: https://www.cancer.gov/types/extragonadal-germ-cell

## Connected records

- cancers: [Cancer of unknown primary, favourable subsets](https://onco.cc/cancers/cup-favourable-subsets/), [Central nervous system germ cell tumours (germinoma and non-germinomatous)](https://onco.cc/cancers/cns-germ-cell-tumours/), [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/), [Retroperitoneal germ cell tumour](https://onco.cc/cancers/retroperitoneal-germ-cell-tumour/), [Testicular germ cell tumours](https://onco.cc/cancers/testicular/)
- drugs: [Bleomycin](https://onco.cc/drugs/bleomycin/), [Cisplatin](https://onco.cc/drugs/cisplatin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [Vinblastine](https://onco.cc/drugs/vinblastine/)
- terms: [Alpha-fetoprotein (AFP)](https://onco.cc/terms/afp/), [Autologous stem cell transplant (ASCT)](https://onco.cc/terms/autologous-transplant/), [Tumour markers (CEA, LDH, chromogranin, thyroglobulin)](https://onco.cc/terms/tumour-markers/)
- trials: [AGCT1531](https://onco.cc/trials/agct1531/), [Sodium Thiosulfate in Preventing Hearing Loss in Young Patients Receiving Cisplatin for Newly Diagnosed Germ Cell Tumor, Hepatoblastoma, Medulloblastoma, Neuroblastoma, Osteosarcoma, or Other Malignancy](https://onco.cc/trials/nct00716976/), [TIGER: standard-dose TIP versus high-dose TI-CE with stem cell transplant as first salvage for relapsed germ cell tumours](https://onco.cc/trials/tiger-trial/)
- technologies: [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/)

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JSON: https://onco.cc/api/v1/entities/extragonadal-germ-cell-tumour.json