ORIGINAL ARTICLE Clinical Presentation and Treatment Outcome of Children With Burkitt Lymphoma in Lebanon A Single Institution’s Experience Samar A. Muwakkit, MD,* Bassem I. Razzouk, MD,† Nina S. Shabb, MD,* Michael L. Hancock, MS,† Ibrahim Dabbous, MD,* Salim Firzli, MD,* and Miguel R. Abboud, MD* Abstract: The authors reviewed the medical records of 42 children younger than 13 years of age diagnosed with Burkitt lymphoma at the American University of Beirut Medical Center between 1983 and 1993. The male:female ratio was 3.9. The abdomen was the most common site of disease (86%). Jaw, central nervous system, and bone marrow involvement occurred in 16.6%, 16.6%, and 9.5%, respec- tively. The mean LDH level was 447 U/L. The mean age at diagnosis was 6.9 years. Thirty-nine patients received a variation of the COMP protocol. The total duration of treatment ranged from 6 to 18 months. At a median follow-up of 5 years the event-free survival was 100% for children with stages I and II disease, 77.4% (6 2 SE) for stage III, and 0% for stage IV. Failures in stage III patients were due to tumor lysis (3/24) and progressive disease (2/24). Aggressive therapy with high doses of methotrexate and anthracyclines may not be necessary for the treatment of children with extensive abdominal disease (stage III) in Lebanon. If confirmed in a larger series of patients, this study could have a major impact on the treatment of Burkitt lymphoma in Lebanon and other countries with limited resources. Key Words: Burkitt lymphoma, child, Lebanon, COMP regimen (J Pediatr Hematol Oncol 2004;26:749–753) I n Lebanon, as in more developed countries, lymphoma accounts for 11.3% of cases of childhood cancer, and it is the third most common cancer after leukemia (33.3%) and brain tumors (20.1%). 1,2 Burkitt lymphoma (BL) is the most com- mon type of non-Hodgkin lymphoma (approximately 50% of cases) in Lebanese children. 2 Although the clinical and molec- ular characteristics of BL in African and North American children have been well described, there is limited information about the presentation of this disease in other geographic loca- tions. 3,4 To characterize the clinical presentation and treatment outcome of BL in Lebanese children, we reviewed the records of all children at least 13 years of age with BL who were treated at Lebanon’s primary referral center for childhood cancer over an 11-year period. METHODS Patients We reviewed the medical records of 42 children younger than 13 years of age who had a diagnosis of BL consistent with the World Health Organization (WHO) criteria. 5 They in- cluded all consecutive patients at least 13 years of age seen at the American University of Beirut Medical Center between January 1983 and December 1993. Thirty-nine of them were treated at our institution. B-cell surface markers were immuno- phenotypically identified on all patients retrospectively and in some also upon diagnosis. A single pathologist (N.S.S.) retro- spectively reviewed all pathology slides initially interpreted as BL. Only cases confirmed as having small noncleaved undifferentiated malignant cells with positive B-cell surface markers were included in this review. Disease Staging Disease stage was established on the basis of physical findings and computed tomography (CT) imaging of the neck, chest, abdomen, and pelvis. If clinically indicated, CT imaging of the brain, orbits, and/or jaw was performed. All patients had bone marrow studies (bilateral aspirates or biopsies), bone scans, and cerebrospinal fluid (CSF) examination. The St. Jude staging system for non-Hodgkin lymphoma was used (Table 1). 6 Distinction was made between localized gastrointestinal tract lymphoma and more extensive intra-abdominal disease. Patients whose disease had spread to the para-aortic and retro- peritoneal areas (tumor implants and/or plaques in the mes- entery or peritoneum, or direct tumor extension to adjacent structures) were considered to have stage III disease. 6 Treatment All patients received the COMP chemotherapy regi- men. 7 Briefly, the treatment schedule consisted of cyclophos- phamide 1.2 g/m 2 IV (induction day 1); 1.0 g/m 2 IV (main- tenance day 1); vincristine 2 mg/m 2 IV (induction, days 3, 10, 17, and 24) and 1.5 mg/m 2 (maintenance, days 1 and 4); methotrexate 300 mg/m 2 IV (60% of doses as IVP, 40% by 4-hour IV infusion) on day 12 of induction and day 15 of Received for publication June 14, 2004; accepted August 9, 2004. From the *Children’s Cancer Center of Lebanon, American University of Beirut, Lebanon; and †St. Jude Children’s Research Hospital, Memphis, Tennessee. This work was supported by the American Lebanese Syrian Associated Charities (ALSAC). Reprints: S. Muwakkit, MD, Children’s Cancer Center, American University of Beirut Medical Center, P.O. Box 11-0236 Beirut, Lebanon (e-mail: sm03@aub.edu.lb). Copyright Ó 2004 by Lippincott Williams & Wilkins J Pediatr Hematol Oncol  Volume 26, Number 11, November 2004 749 JOBNAME: pho 26#11 2004 PAGE: 1 OUTPUT: Wed November 3 11:59:10 2004 lww/pho/88504/04-151R1