Sex cord-stromal tumours (SCSTs) in children are rare gonadal neoplasms exhibiting differentiation towards sex cord stromal elements. This case series explores paediatric SCSTs, presenting clinical, gross, histologic, and immunohistochemical findings, with a focus on limited information available about these tumours in African children. This retrospective study includes 9 cases of ovarian and testicular SCSTs (8 female and 1 male) in patients below 16 years of age. Female patients presented with various SCST subtypes, including juvenile granulosa cell tumours (JGCTs), fibroma, and mixed tumours. Precocious puberty was a common presentation. The male patient had a Leydig cell tumour. Tumour sizes varied, with the smallest in the testis and the largest in a 3-year-old girl. Histologically, most cases exhibited characteristic features of their respective subtypes. Paediatric SCSTs are rare but important considerations in children with gonadal masses. Their incidence in African children is largely unknown. JGCTs are the most prevalent ovarian subtype. Leydig cell tumours are rare in children and can present with virilisation. Mixed SCST-germ cell tumours, though extremely rare, warrant careful consideration and immunohistochemistry for accurate diagnosis.
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