Extragonadal germ cell tumour
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.
Overview
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.
State of the art
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.
- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
BEP for three cycles or EP for four; radiotherapy only for small retroperitoneal disease; PET to assess residual masses.
BEP for three or four cycles by IGCCCG risk group, then resection of residual masses over one centimetre.
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.
Subtypes & biomarkers
top- Mediastinal seminoma
- Primary mediastinal non-seminomatous germ cell tumour
- Retroperitoneal germ cell tumour
- Intracranial germ cell tumours (pineal, suprasellar)
- Sacrococcygeal teratoma (infants)
- Alpha-fetoprotein (AFP)
- Human chorionic gonadotropin (hCG)
- Lactate dehydrogenase (LDH)
- IGCCCG risk group (mediastinal non-seminoma is poor risk)
- Isochromosome 12p
How often this target appears
- 1977Cisplatin combinations cure disseminated germ cell tumours
Einhorn's PVB regimen (cisplatin, vinblastine, bleomycin) turned a usually fatal cancer into a curable one.
- 1987BEP replaces PVB
Etoposide in place of vinblastine gave equal cure with less neurotoxicity in the Indiana and SECSG trials.
- 1997IGCCCG classification
The International Germ Cell Cancer Collaborative Group defined good, intermediate and poor risk; a mediastinal non-seminomatous primary alone places a patient in the poor-risk group.
Open problems and what is being done
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).
Trials
topTrials recruiting now
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Expert centres
topExpert centres
- Histiocyte SocietyPitman, USvia Vinblastine
- Indiana University Melvin and Bren Simon Comprehensive Cancer CenterIndianapolis, IN, USNCI comprehensivevia Cisplatin
Questions to ask
topQuestions to ask your oncologist about Extragonadal germ cell tumour
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Alpha-fetoprotein, Human chorionic gonadotropin, Lactate dehydrogenase, IGCCCG risk group, Isochromosome 12p), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Mediastinal seminoma, Primary mediastinal non-seminomatous germ cell tumour, Retroperitoneal germ cell tumour.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Seminoma (mediastinal or retroperitoneal)
- For my situation (seminoma (mediastinal or retroperitoneal)), which of the standard options do you recommend and why?Why: Guideline options include: BEP for three cycles or EP for four; radiotherapy only for small retroperitoneal disease; PET to assess residual masses.
- Am I a candidate for Bleomycin, Etoposide, Cisplatin, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Non-seminoma, retroperitoneal
- For my situation (non-seminoma, retroperitoneal), which of the standard options do you recommend and why?Why: Guideline options include: BEP for three or four cycles by IGCCCG risk group, then resection of residual masses over one centimetre.
- Am I a candidate for Bleomycin, Etoposide, Cisplatin, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Non-seminoma, mediastinal (poor risk)
- For my situation (non-seminoma, mediastinal (poor risk)), which of the standard options do you recommend and why?Why: Guideline options include: 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.
- Am I a candidate for Etoposide, Ifosfamide, Cisplatin, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “Cure rates for mediastinal non-seminoma remain well below those of testicular primaries”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “The role of high-dose chemotherapy in first-line poor-risk disease is still being tested (TIGER trial)”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
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Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
5targets
1drugs
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3Latest papers
topQuery for this cancer: (TITLE:"Extragonadal germ cell tumour" OR ABSTRACT:"Extragonadal germ cell tumour" OR TITLE:"Extragonadal Germ Cell Tumor" OR ABSTRACT:"Extragonadal Germ Cell Tumor" OR TITLE:"Extragonadal germ cell tumor" OR ABSTRACT:"Extragonadal germ cell tumor" OR TITLE:"Mediastinal germ cell tumour" OR ABSTRACT:"Mediastinal germ cell tumour" OR TITLE:"Retroperitoneal germ cell tumour" OR ABSTRACT:"Retroperitoneal germ cell tumour" OR TITLE:"Primary mediastinal nonseminomatous germ cell tumour" OR ABSTRACT:"Primary mediastinal nonseminomatous germ cell tumour") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Extragonadal germ cell tumour, not a curated reading list.
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