Prepubertal testicular and paratesticular tumours are a rare group of tumours, distinct from postpubertal paediatric and adult tumours of this region. Tumours within this group are testicular germ-cell tumours (such as benign teratoma, epidermoid cyst and malignant yolk-sac tumours) and stromal tumours (such as juvenile granulosa-cell, Leydig-cell, and Sertoli-cell tumours). Paratesticular tumours can be benign (lipoma, leiomyoma, haemangioma) or malignant (rhabdomyosarcoma, melanotic neuroectodermal tumour of infancy). Because of their rarity, centralised pathology and treatment, and national collaborative clinical trials have been important in establishing the optimum management of malignant tumours in this group. We provide an up-to-date and comprehensive review of the clinical presentation, imaging, pathology, and clinical management of prepubertal paratesticular and testicular tumours.
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