Acute lymphoblastic leukemia (ALL) is the most common pediatric cancer. Within ALL, precursor B-cell disease predominates and now has survival >90%. Mature B-cell, or Burkitt leukemia/lymphoma, is distinct from ALL and requires short intensive chemotherapy and with the addition of Rituximab, survival rates of >95% are achieved. Its defining characteristic is MYC translocation at 8q24. Patients who have features of both ALL and Burkitt leukemia/lymphoma represent a rare subpopulation of ALL and present a diagnostic and treatment conundrum. We have performed a systematic review on the occurrence of and treatment of MYC positive precursor B-ALL, reported between 1980 and 2016. The review highlighted a lack of data to guide any consensus about how to treat this important group of children and focused research in this area is needed.
Keywords: MYC; Pediatric; acute lymphoblastic leukemia; precursor B.