Germ-cell tumours

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Target exams

Red flags

• A newborn with a large sacrococcygeal teratoma and hydrops or a high-output cardiac state is at risk of cardiovascular collapse and needs stabilisation before any surgery
• A markedly raised serum alpha-fetoprotein in a child over one year of age with a solid mass indicates a yolk sac tumour or a malignant germ cell tumour until histology proves otherwise
• A child with diabetes insipidus, growth failure or precocious puberty and a suprasellar or pineal mass has an intracranial germ cell tumour until the serum markers and the magnetic resonance imaging settle it
• A mediastinal germ cell tumour can cause superior vena cava obstruction and a dangerous airway under anaesthesia, so the biopsy is planned with the anaesthetist and the surgeon together
• Sacrococcygeal teratoma recurs and may turn malignant if the coccyx is not removed at the primary resection, so the coccygectomy is mandatory and not optional

Life stages

Care settings

Clinical exam formats

Board mappings

• Recognises that germ cell tumours arise from primordial germ cells, follow a midline distribution from the sacrococcygeal region to the intracranial pineal and suprasellar region, and show a bimodal age peak in the infant and the adolescent
• Identifies the tumour markers alpha-fetoprotein and beta-human chorionic gonadotropin and the germ cell tumours that secrete them, including the physiological elevation of alpha-fetoprotein in the neonate
• Names the sacrococcygeal teratoma as the commonest tumour of the newborn and the imperative of the coccygectomy at the primary resection to prevent recurrence
• Explains the histological classification into the germinoma or dysgerminoma, which secretes little or no alpha-fetoprotein, and the nongerminomatous tumours such as the yolk sac tumour and the choriocarcinoma that secrete alpha-fetoprotein and beta-human chorionic gonadotropin
• Distinguishes the highly curable intracranial germinoma, treated with chemotherapy and reduced-dose radiotherapy, from the more guarded nongerminomatous intracranial germ cell tumour that needs craniospinal irradiation
• Coordinates the risk-adapted pathway from the serum markers and the imaging through the surgery and the platinum-based PEB chemotherapy to the surveillance of the late effects
• A markedly raised alpha-fetoprotein in a child over one year with a solid mass is a yolk sac tumour or a malignant germ cell tumour until histology proves otherwise
• Sacrococcygeal teratoma recurs and may turn malignant if the coccyx is not removed, so the coccygectomy is mandatory at the primary resection
• The physiological alpha-fetoprotein is very high at birth and falls to the adult range by about eight to twelve months, so a raised value in the young infant must be interpreted against the age
• Counsels the family of a child with a malignant germ cell tumour on the excellent contemporary survival of over ninety percent for localised disease and the role of the platinum-based chemotherapy
• Builds the multidisciplinary plan that runs paediatric oncology, paediatric surgery, urology, gynaecology, radiation oncology and the family together
• Synthesises the long-case child with a sacrococcygeal teratoma into a surgical and surveillance plan that addresses the recurrence risk, the malignant transformation, the bladder and bowel function and the cosmetic outcome
• Level 1: Recognition of the mass lesion and the serum markers of a paediatric germ cell tumour and the urgent referral
• Level 2: Application of the diagnostic pathway with the serum markers and the cross-sectional imaging and the risk-adapted chemotherapy
• Level 3: Coordination of the definitive therapy and the long-term surveillance of the late effects and the fertility
• The primordial germ cell origin and the midline distribution of the germ cell tumours
• The tumour markers alpha-fetoprotein and beta-human chorionic gonadotropin and the histological subtypes that secrete them
• The Altman classification of the sacrococcygeal teratoma and the principle of the coccygectomy
• Examination of the child with a sacral or gonadal mass and the assessment of the newborn with a sacrococcygeal teratoma
• Communication of a new diagnosis of a germ cell tumour and the discussion of the chemotherapy and the fertility
• Recognition of the emergencies of the high-output cardiac failure of the large sacrococcygeal teratoma and the superior vena cava obstruction of the mediastinal mass
• Germ cell tumours as a common and highly curable group of paediatric malignancies with a bimodal age distribution
• The clinical presentation by location: sacrococcygeal, gonadal, mediastinal and intracranial
• The principles of the surgery and the platinum-based chemotherapy and the avoidance of unnecessary treatment in the completely resected early-stage disease
• Recognition of the clinical features of a paediatric germ cell tumour that mandate the serum markers, the imaging and the specialist referral
• Application of the initial stabilisation of the newborn with a large sacrococcygeal teratoma and the child with a mediastinal mass
• Coordination of the diagnostic and the multidisciplinary pathway for a child with a germ cell tumour
• Canadian approach to the urgent evaluation and the referral of a child with a suspected germ cell tumour
• The risk-adapted chemotherapy and the role of the surgery and the radiotherapy across the extracranial and the intracranial disease
• The late effects of the platinum-based chemotherapy and the surveillance of the paediatric germ cell tumour survivor