High-Stage Childhood Anaplastic Large Cell Lymphoma
Note: Some citations in the text of this section are followed by a level of evidence. The PDQ Editorial Boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)
Children and adolescents with high-stage (stage III or IV) anaplastic large cell lymphoma (ALCL) have a disease-free survival of approximately 60% to 75%.[1,2,3,4,5] It is unclear which strategy is best for the treatment of high-stage ALCL. The German Berlin-Frankfurt-Munster (BFM) group used six cycles of intensive pulsed therapy, similar to their B-cell non-Hodgkin lymphoma (NHL) therapy (GER-GPOH-NHL-BFM-90 [NHL-BFM-90]).; [Level of evidence: 1iiA] Building on these results, the FRE-IGR-ALCL99-vinblastine study randomly assigned patients to limited vinblastine versus prolonged (1 year) vinblastine exposure. It was reported that while the addition of vinblastine to a 4-month chemotherapy regimen consisting of dexamethasone, methotrexate, ifosfamide, cytarabine, etoposide, and doxorubicin delayed the time of relapse, it did not reduce the overall risk of recurrence in patients with high-risk ALCL. Also, patients receiving the vinblastine plus chemotherapy regimen had a better event-free survival (EFS) in the first year after therapy (91%) than those not receiving vinblastine (74%); however, after 2 years of follow-up, the EFS was 73% for those who received vinblastine and 70% for those who did not receive vinblastine.[Level of evidence: 1iiDi] Of note, the European Intergroup for Childhood NHL (EICNHL) group showed in the FRE-IGR-ALCL99 study (based on the GER-GPOH-NHL-BFM-90 regimen) that methotrexate 1 g/m2 infused over 24 hours has a similar outcome compared with methotrexate 3 g/m2 infused over 3 hours without intrathecal methotrexate. Additionally, they showed that there was less toxicity with 3 g/m2 of methotrexate over 3 hours compared with 1 g/m2 over 24 hours. The Pediatric Oncology Group (POG) trial (POG-9317) demonstrated no benefit of adding methotrexate and high-dose cytarabine to 52 weeks of the APO (doxorubicin, prednisone, and vincristine) regimen. The Italian Association of Pediatric Hematology/Oncology group used a leukemia-like regimen for 24 months in LNH-92, with similar results as other regimens. The CCG-5941 study tested an approach similar to LNH-92, with more intensive induction and consolidation with maintenance for 1 year total duration of therapy, with similar outcome, but significant hematologic toxicity was observed.[Level of evidence: 2A]
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A retrospective study suggested that the amount of tumor involvement, as measured by polymerase chain reaction in the marrow, is predictive for relapse. An immune response against the ALK protein (i.e., anti-ALK antibody titer) appears to correlate with lower clinical stage and absence of clinical risk features (mediastinal and visceral organ involvement) and predict relapse risk but not overall survival. Another retrospective study of ALCL by the EICNHL grouped patients as standard risk and high risk, which is defined as involvement of mediastinum, skin, or viscera. In the EICNHL analysis, patients at high risk were reported to have an inferior 5-year progression-free survival (PFS). However, in the CCG-5941 study, only bone marrow involvement predicted inferior PFS.[Level of evidence: 2A]