Burkitt’s and Burkitt-like lymphoma in children and adolescents: a review of the Children’s Cancer Group Experience* Mitchell S. Cairo, 1 Richard Sposto, 2 Sherrie L. Perkins, 3 Anna T. Meadows, 4 Margo L. Hoover-Regan, 5 James R. Anderson, 6 Stuart E. Siegel, 7 Mark A. Lones, 8 Nicole Tedeschi- Blok, 2 Marshall E. Kadin, 9 Carl R. Kjeldsberg, 3 John F. Wilson, 3 Warren Sanger, 10 Erin Morris, 1 Mark D. Krailo 2 and Jonathan L. Finlay 11 1 Department of Pediatrics, Children’s Hospital New York, Columbia University, New York, NY, 2 Department of Biostatistics, Keck School of Medicine, University of Southern California, Los Angeles, CA, Children’s Cancer Group, Arcadia, CA, 3 Department of Pathology, University of Utah Medical Center, Salt Lake City, Utah, UT, 4 Department of Pediatrics, Children’s Hospital of Philadelphia, Philadelphia, PA, 5 Department of Pediatrics, Riley Children’s Hospital, Indiana University, Indianapolis, IN, 6 Department of Biostatistics, University of Nebraska, Omaha, NE, 7 Department of Pediatrics, Children’s Hospital Los Angeles, Los Angeles, 8 Department of Pathology, Children’s Hospital of Orange County/St. Joseph Hospital, Orange, CA, 9 Department of Pathology, Beth Israel Deaconess Medical Center, Boston, MA, 10 Department of Cytogenetics, University of Nebraska, Omaha, NE, and 11 Department of Pediatrics, New York University School of Medicine, New York, NY, USA Received 7 June 2002; accepted for publication 31 August 2002 Summary. Historically, the survival of children and ado- lescents with Burkitt’s and Burkitt-like lymphoma had been poor. Recently, short and intensive chemotherapy appears to have improved disease outcome. We therefore reviewed the results of four successive Children’s Cancer Group trials conducted on 470 children with disseminated Burkitt’s and Burkitt-like lymphoma. Of the patients studied, the median age was 8 years (0–21 years), the male:female ratio was 4:1, 58% had lactate dehydrogenase (LDH) ‡ 500 IU/l, 23% had M2 or M3 bone marrow (BM), and 12% demon- strated central nervous system involvement. In a multi- variate analysis, the 4-year event-free survival (EFS) in patients ‡ 15-years-old compared with < 15-year-old was 34 ± 7 versus 59 ± 2% (P <0Æ05), the 4-year EFS of M2/M3 compared with M1 BM was 38 ± 5 versus 63 ± 3% (P <0Æ001), and the 4-year EFS with LDH ‡ 500 IU/l compared with LDH < 500 IU/l was 49 ± 3 versus 71 ± 4% (P <0Æ001). Furthermore, patients treated on the most recent protocol, which was short and more intensive, had a significantly improved survival compared with those on previous trials (4-year EFS 80 ± 6 versus 54 ± 2%, P <0Æ001). In summary, the outcome for childhood Bur- kitt’s and Burkitt-like lymphoma has recently improved with the use of short and intensive B-cell non-Hodgkin’s lymphoma-directed therapy. Keywords: childhood, Burkitt’s, Burkitt-like, lymphoma, outcome. Small non-cleaved lymphoma (SNCL), also known as Burkitt’s and Burkitt-like lymphoma, represents approxi- mately 40% of all childhood non-Hodgkin’s lymphoma (NHL) and 3–4% of all childhood malignancies diagnosed each year in the USA. (Rappaport, 1966; Lukes & Collins, 1974; National Cancer Institute, 1982; Lennert & Feller, 1992; Harris et al, 1994; Perkins et al, 1995; Sandlund et al, 1996; Cairo & Perkins, 2000). The Burkitt’s variety of SNCL is characterized morphologically by a diffuse, homo- geneous proliferation of intermediate-size B cells (CD20), which have a scanty but intensely basophilic cytoplasm with numerous cytoplasmic lipid-filled vacuoles (Harris et al, 1994; Perkins et al, 1995). Cytogenetic and molecular investigations of Burkitt’s SNCL have demonstrated Correspondence: Mitchell S. Cairo, MD, Children’s Cancer Group, PO Box 60012, Arcadia, CA 91066–6012, USA. E-mail: mc1310@columbia.edu *Presented in part at the American Society of Clinical Oncology (ASCO) Annual Meeting, May 1998, and The American Society of Hematology, December 2000. British Journal of Haematology, 2003, 120, 660–670 660 Ó 2003 Blackwell Publishing Ltd