کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
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4172798 | 1275777 | 2010 | 8 صفحه PDF | دانلود رایگان |
Germ cell tumours (GCTs) are a heterogeneous group of predominantly midline tumours uniquely occurring from birth to late adulthood. All are believed to arise from the totipotent primordial germ cell. In childhood, ∼50% are gonadal and ∼50% extragonadal (∼20% intracranial and ∼30% extracranial) and clinical presentation depends on tumour site. Teratomas account for ∼50% of all paediatric GCTs and are considered benign in childhood. Malignant GCTs often secrete the tumour markers alpha-fetoprotein (AFP) and human chorionic gonadotrophin (ß-HCG), which may help in diagnosis and follow-up. Management involves complete surgical resection for teratomas and non-metastatic gonadal tumours. In the UK, chemotherapy is reserved for stage 2–4 extracranial malignant GCTs, resulting in >90% five-year overall survival (OS). Radiotherapy is rarely indicated for extracranial disease. Intracranial tumours typically occur in the pineal or suprasellar region. Intracranial germinomas are cured in >90% cases with radiotherapy or combined chemo-radiotherapy. About two-thirds of non-germinomatous intracranial tumours are cured with combined chemo-radiotherapy. Current issues relating to the management of teenagers and young adults (TYAs) with GCTs and implications of tumour biology are highlighted in this review.
Journal: Paediatrics and Child Health - Volume 20, Issue 3, March 2010, Pages 109–116