Burkitt Leukemia Presenting as Acute Appendicitis: A Case Report and Literature Review

BACKGROUND Appendicitis is the most common cause of an acute abdomen. Approximately 1% of appendicectomies will have an incidental finding of an appendiceal neoplasm. A primary appendiceal lymphoma is extremely rare, and is found in 0.015% of all appendiceal specimens. Burkitt lymphoma is an aggressive B cell lymphoma characterized by translocation and dysregulation of the c-Myc gene. Burkitt leukemia is considered to be an alternative manifestation of the same pathology, and is defined by the presence of >25% Burkitt blasts within the bone marrow. The treatment approaches for Burkitt leukemia/lymphoma are similar. CASE REPORT A 6-year old girl presented with a history, examination, and radiological imaging consistent with acute appendicitis. An inflamed, edematous appendix was identified intraoperatively, and a cecectomy was performed. Histopathological investigations demonstrated Burkitt leukemia with isolated extra-nodal involvement of the appendix. The patient was subsequently started on a multi-agent steroid and chemotherapy regimen. A literature review was performed, identifying cases of Burkitt leukemia/lymphoma presenting as appendicitis. CONCLUSIONS This case highlights the importance of clinical vigilance and routine specimen histopathology review, and explores key management considerations associated with the incidental diagnosis of Burkitt leukemia/lymphoma.