Primary gonadal lymphomas are rare in childhood. This study aimed to evaluate seven patients with primary gonadal origin who were diagnosed with non- Hodgkin lymphoma (NHL) between 1980 and 2024 retrospectively. Among the 1360 NHL followed up in our department over a forty-four-year period, seven patients (0.5%) were diagnosed with primary gonadal lymphoma. In seven patients, whose ages ranged from 1 to 16 years, gonadal involvement was testicular in five and ovarian in two. The presenting complaint was scrotal swelling in patients with testicular tumors, abdominal distension, and systemic symptoms in patients with ovarian tumors. All male and one female patients underwent surgical excision, and the other female was diagnosed from ascitic fluid. Histopathology was consistent with Burkitt in three, diffuse large B cell in one, lymphoblastic lymphoma in one, and NHL subtype was undetermined in two. Three patients each had stage I and stage II disease, and one had stage III. All patients received chemotherapy. Median follow-up time was 52 months, and 5-year overall and event-free survival rates were 85.7%. Primary gonadal lymphomas are rare in childhood and have different characteristics than adults. Primary gonadal lymphomas should be kept in mind in pediatric patients presenting with an isolated gonadal mass.

Keywords: childhood, primary ovarian lymphoma, gonadal mass, ovary, primary testicular lymphoma