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.
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.
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.
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.
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Query 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.
Bokemeyer and colleagues pool 635 patients from eleven centres: seminomas do as well as testicular disease, mediastinal non-seminomas do worst, and prognostic factors are defined.
The Alliance and European groups randomise first-relapse germ cell tumours between conventional-dose TIP and high-dose TI-CE with stem cell rescue, the first randomised test of high-dose salvage since the 1990s.
Einhorn's PVB regimen (cisplatin, vinblastine, bleomycin) turned a usually fatal cancer into a curable one.
Etoposide in place of vinblastine gave equal cure with less neurotoxicity in the Indiana and SECSG trials.
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.
The targets of this cancer's medicines and the ones linked to it directly.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Pulmonary toxicity rises with G-CSF, high inspired oxygen, renal impairment and age over 40.
Reduce for CrCl below 50.
Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
Reduce to 75% for CrCl 15-50.
Interstitial lung disease (ILD) is lung inflammation, a serious side effect of some ADCs (especially Enhertu) and immunotherapy.
See all on the product pages:BleomycinCisplatinEtoposideIfosfamide·Printable cards in the navigator
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