Sporadic Burkitt’s lymphoma/acute B-cell leukaemia presenting with progressive proptosis and orbital mass in a child

Abstract

Burkitt's lymphoma (BL) is an aggressive B-cell non-Hodgkin lymphoma that is found predominantly in children, with the highest incidence occurring in Africa. The sporadic form occurs in non-endemic areas and typically involves the ileo-caecum and the bowel, whereas orbital and paranasal sinus involvement is rare. Here, we present an unusual case of sporadic BL in a Caucasian male child with rapidly progressive painful proptosis of the right eye. Magnetic resonance imaging showed an oval-shaped, extraconal mass in the supero-lateral part of the right orbit that deformed and dislocated the eyeball antero-inferiorly. The patient underwent anterior orbitotomy, and a biopsy of the excised tissue revealed a starry-sky appearance characteristic of BL. Postoperative aggressive chemotherapy was initiated with a good response after one week.

Outcomes of Burkitt lymphoma with bone marrow involvement or Burkitt leukemia in Chinese children

“…Although the biological and clinical behaviors of Burkitt lymphoma in pediatric patients are malignant and the disease develops rapidly, its long‐term outcomes are favorable. The most commonly involved regions include the abdomen, peripheral lymph nodes, neck, mediastinum, and kidney 8 . Sporadic bone marrow involvement is present in approximately 20% of patients with Burkitt lymphoma 1 .…”

Sporadic Burkitt Lymphoma with Orbital Extramedullary Infiltration

“…Most cases have centered on an aggressive, unilateral episode of proptosis, with focal infiltration [7, 9-11]. In 1 case series, the infiltration was so focal and pronounced, and progression so rapid, that the initial suspected diagnosis was rhabdomyoscarcoma [11].…”

“…Burkitt lymphoma is known for its aggressive nature and predominance in the pediatric population [7]. In endemic Burkitt, there is a higher association with Epstein-Barr virus and facial bones, as well as extramedullary involvement [8].…”