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

Abstract

Objective: Rare disease 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.

Double-Expressor Appendiceal Burkitt’s Lymphoma: A Case Report and Literature Review

“…Although the most common finding on flow cytometry is an expression of IgM immunoglobulin on the surface of biopsied tissue, mature B-cell markers such as CD19, CD20, CD22, CD79a, and CD10 can be found. However, CD5, CD23, CD34, and tdTare usually negative [9]. Patients with Burkitt's lymphoma who have an abdominal mass may present with nausea, vomiting, loss of appetite, gastrointestinal bleeding, signs and symptoms of acute abdomen, intestinal perforation, or renal failure [9].…”

“…However, CD5, CD23, CD34, and tdTare usually negative [9]. Patients with Burkitt's lymphoma who have an abdominal mass may present with nausea, vomiting, loss of appetite, gastrointestinal bleeding, signs and symptoms of acute abdomen, intestinal perforation, or renal failure [9].…”

Acute promyelocytic leukemia presenting with acute appendicitis and coagulopathy in a pediatric patient: A previously unreported site of extramedullary disease

“…Acute appendicitis has been described as the initial presentation of pediatric patients with hematologic malignancies, including acute lymphoblastic leukemia, 8 AML, 9 and Burkitt lymphoma. 10,11 There are, however, no reported cases of appendicitis at initial presentation of APL in pediatric patients, though some have been reported in adults. 12,13 More often, extramedullary disease is associated with relapsed APL, occurring in 3%-5% of patients, with the central nervous system being the most common site.…”

Burkitt Lymphoma Presenting as Perforated Appendicitis

“…1,3 In the absence of data to suggest a survival benefit with hemicolectomy, appendectomy is the preferred index operation. 2 Patients should receive adjuvant combination chemotherapy with cyclophosphamide, vincristine, doxorubicin, and methotrexate. There is emerging evidence in support of CD20-directed monoclonal antibody therapy with rituximab.…”

“…3 Instead, they may present with right iliac fossa pain, nausea, vomiting, and leukocytosis, mimicking acute appendicitis. [1][2][3][4] Imaging findings that should heighten suspicion for primary appendiceal lymphoma are an appendiceal diameter greater than 3 cm and abdominal lymphadenopathy. 1,3 In the absence of data to suggest a survival benefit with hemicolectomy, appendectomy is the preferred index operation.…”