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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.
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.
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).
Germ cell tumours drain along the spermatic cord to the para-aortic nodes high in the abdomen, not to the groin, which is why staging scans look at the retroperitoneum.
Same organ: Retroperitoneal germ cell tumour, Leydig cell tumour of the testis, Sertoli cell tumour of the testis, Spermatocytic tumour of the testis, Germ cell neoplasia in situ (GCNIS), Embryonal carcinoma of the testis, Yolk sac tumour of the testis, postpubertal type, Choriocarcinoma of the testis, Testicular germ cell tumours, Seminoma, Non-seminomatous germ cell tumour
Complete resection followed by active surveillance with serial tumour markers and imaging; chemotherapy only for relapse (AGCT1531 stratum).
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.
Platinum-based chemotherapy followed by reduced-dose whole-ventricular radiotherapy with tumour boost (SIOP CNS GCT II, ACNS1123).
Intensive platinum-based chemotherapy, second-look surgery for residual disease, then craniospinal or whole-ventricular radiotherapy depending on response and stage.
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Whole-ventricular irradiation with a boost is a reasonable option after a good response to chemotherapy, but the spinal failure pattern shaped the next trial and keeps craniospinal irradiation as an alternative.
Two cancer pages and one treatment page on OnCo cite this paper by its DOI; this record gives the citation a page of its own so a reader can follow it without leaving OnCo. Read the abstract above alongside the citing pages listed under Related; the record was created automatically from the Europe PMC entry and its figures have not been checked by hand.
One cancer page on OnCo cites this paper by its DOI; this record gives the citation a page of its own so a reader can follow it without leaving OnCo. Read the abstract above alongside the citing page listed under Related; the record was created automatically from the Europe PMC entry and its figures have not been checked by hand.
Query for this cancer: (TITLE:"Germ cell tumours of childhood and adolescence" OR ABSTRACT:"Germ cell tumours of childhood and adolescence" OR TITLE:"extracranial and CNS" OR ABSTRACT:"extracranial and CNS" OR TITLE:"Childhood extracranial germ cell tumour" OR ABSTRACT:"Childhood extracranial germ cell tumour" OR TITLE:"CNS germ cell tumour" OR ABSTRACT:"CNS germ cell tumour" OR TITLE:"Germinoma" OR ABSTRACT:"Germinoma" OR TITLE:"Ovarian germ cell tumour" OR ABSTRACT:"Ovarian germ cell tumour") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Germ cell tumours of childhood and adolescence (extracranial and CNS), not a curated reading list.
Einhorn's PVB regimen in testicular cancer, later adapted to children.
UKCCSG shows carboplatin can replace cisplatin in children with less hearing and kidney toxicity.
POG/CCG intergroup studies show surgery alone is safe for low-stage disease.
Frazier and colleagues (JCO) combine COG and CCLG data to define shared risk groups.
SIOPEL 6 (NEJM 2018) and ACCL0431 show reduced cisplatin ototoxicity; FDA approval 2022.
Joint COG/CCLG trial of active surveillance and carboplatin versus cisplatin.
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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 by Calvert formula using GFR (see the calculators).
Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
Reduce to 75% for CrCl 15-50.
See all on the product pages:BleomycinCarboplatinCisplatinEtoposideIfosfamide·Printable cards in the navigator
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