Orbital non-Hodgkin lymphoma (NHL) is rare in children. We report the case of a 13-year-old girl presenting with massive proptosis due to orbital NHL. Despite initial diagnosis by biopsy, the patient was lost to follow-up for one year, resulting in significant tumor growth and destruction of ocular structures. She was started on lymphoblastic induction chemotherapy, but further systemic evaluation revealed underlying acute myeloid leukemia (AML) with RUNX1-RUNX1T1 fusion and t(8;21) translocation, prompting a change in treatment regimen. AML-directed chemotherapy led to substantial tumor regression, cessation of bleeding, and cosmetic improvement. This case underscores the importance of early diagnosis, systemic evaluation, regular follow-up, and socioeconomic support in managing rare pediatric orbital tumors with underlying hematologic malignancy.
Patel et al. (2026) studied this question.