Sex cord-stromal tumors may present in young girls with signs and symptoms similar to those of other ovarian tumors such as abdominal pain or distention, gastrointestinal symptoms or abdominal mass. Unlike children with germ cell or epithelial gonadal tumors, however, these patients often have clinical signs of hormone production and may present with signs of early puberty precocity including breast swelling and vaginal bleeding, primary or secondary amenorrhea, and /or signs of male hormone production.

Granulosa cell tumors are of two very distinct subtypes, juvenile and adult. Juvenile granulosa cell tumors are seen in young children and may present with precocious puberty. The vast majority of juvenile granulosa cell tumors are localized (i.e. Stage I) at diagnosis and associated with a favorable prognosis. Sertoli-Leydig cell tumors generally occur in children, adolescents and young women. Localized juvenile granulosa cell tumors and Sertoli Leydig cell tumors are associated with a favorable prognosis, however, patients with later stage disease or recurrence have a worse prognosis. Gynandroblastomas contain both juvenile granulosa cell and Sertoli-Leydig cell elements.

The majority of testicular stromal tumors present with a painless heterogeneous mass, easily palpable and visible on ultrasound. Children may present with breast enlargement or signs of early puberty. Children with Leydig cell tumors may have increased 17-ketosteroids. Testicular juvenile granulosa cell tumors often present in patients during the first six months of life and typically lack hormone-related symptoms. Testicular juvenile granulosa cell tumors are usually treated with surgery alone. The cause of testicular juvenile granulosa cell tumors is unknown.