Challenging differential diagnosis, Management of emergency care, Rare disease
Alexander H. Mimery, Joe Jabbour, Blake Sykes, Ewan MacDermid, Mohamed Al-Askari, Stefaan De Clercq
Department of Surgery, Gladstone Hospital, Gladstone, Queensland, Australia
Am J Case Rep 2020; 21:e921568
Available online: 2020-01-30
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.
Keywords: Appendiceal Neoplasms, appendicitis, Burkitt Lymphoma