Standard Treatment Options
Treatment Options Under Clinical Evaluation
Current Clinical Trials

Patients with disseminated lymphoblastic lymphoma have long-term survival rates higher than 80%.[1,2] As opposed to other pediatric non-Hodgkin lymphoma (NHL), it has been shown that lymphoblastic lymphoma responds much better to leukemia therapy with 2 years of therapy than with shorter, intensive, pulsed chemotherapy regimens.[1] A Pediatric Oncology Group (POG) randomized trial (POG 8704) showed that patients receiving a weekly high-dose asparaginase regimen for 20 weeks had a superior outcome.[3] The best results to date come from the Berlin-Frankfurt-Munster (BFM) group. In the NHL-BFM-90 study, the 5-year disease-free survival was 90%, and there was no difference in outcome between stage III and stage IV patients.[2] Precursor B-cell lymphoblastic lymphoma appears to have similar results using the same therapy.[4] In the NHL-BFM-95 study, the prophylactic cranial radiation was omitted, and the intensity of induction therapy was decreased slightly. There were no significant increases in central nervous system (CNS) relapses, suggesting cranial radiation may be reserved for patients with CNS disease at diagnosis.[5] Of interest, the probability of 5-year event-free survival (EFS) rates was worse in BFM-95 than in BFM-90 (82% vs. 90% respectively). Although this difference was not statistically different, the omission of prophylactic cranial radiation and the reduction of asparaginase and/or doxorubicin in induction may have affected outcome. It was proposed that the major difference in EFS between BFM-90 and BFM-95 resulted from the increased number of secondary malignancies observed in BFM-95.[5] The reason for this is unclear, but it has been shown that patients treated for lymphoblastic lymphoma have a higher incidence of secondary malignancy than do patients treated for other pediatric NHL (10% vs. 2% at age 20 years).[6]

Involvement of the bone marrow may lead to confusion as to whether the patient has lymphoma or leukemia. Traditionally, patients with more than 25% marrow blasts are classified as having leukemia, and those with fewer than 25% marrow blasts are classified as having lymphoma. It is not yet clear whether these arbitrary definitions are biologically distinct or relevant for treatment design. All current therapies for advanced-stage lymphoblastic lymphoma have been derived from regimens designed for the treatment of acute lymphoblastic leukemia.

Mediastinal radiation is not necessary for patients with mediastinal masses, except in the emergency treatment of symptomatic superior vena caval obstruction or airway obstruction, where low-dose radiation is usually employed. (Refer to the Treatment Option Overview section of this summary for more information on such complications.) Because of the complexities of optimal therapeutic regimens and the possibility of toxic side effects, patients should be offered the opportunity to enter into a clinical trial. Information about ongoing clinical trials is available from the NCI Web site.

 [Note: Current data do not suggest superiority for the following standard treatment options.]

• NHL-BFM-90: prednisone, dexamethasone, vincristine, daunorubicin, doxorubicin, L-asparaginase, cyclophosphamide, cytarabine, methotrexate, 6-mercaptopurine, 6-thioguanine, CNS radiation therapy.[1]



• NHL-BFM-95: prednisone, dexamethasone, vincristine, daunorubicin, doxorubicin, L-asparaginase, cyclophosphamide, cytarabine, methotrexate, 6-mercaptopurine, 6-thioguanine, CNS radiation therapy for CNS-positive patients only.[2]

• COG A-5971 : A Children’s Oncology Group (COG) trial comparing the NHL-BFM-95 regimen (which utilizes high-dose methotrexate during interim maintenance for CNS prophylaxis) with the COG BFM-modified leukemia regimen (which uses oral methotrexate during interim maintenance and intensive intrathecal methotrexate for CNS prophylaxis). A second randomization compares standard BFM four-drug induction to the New York induction with high-dose daunorubicin and a single dose of cyclophosphamide during the first 3 days of therapy, in conjunction with vincristine, steroid, and L-asparaginase. The study has met accrual for the randomization. The study recently closed and is awaiting final analysis.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III childhood lymphoblastic lymphoma and stage IV childhood lymphoblastic lymphoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References

1. Anderson JR, Jenkin RD, Wilson JF, et al.: Long-term follow-up of patients treated with COMP or LSA2L2 therapy for childhood non-Hodgkin's lymphoma: a report of CCG-551 from the Childrens Cancer Group. J Clin Oncol 11 (6): 1024-32, 1993.  [PUBMED Abstract]
   
   
2. Reiter A, Schrappe M, Ludwig WD, et al.: Intensive ALL-type therapy without local radiotherapy provides a 90% event-free survival for children with T-cell lymphoblastic lymphoma: a BFM group report. Blood 95 (2): 416-21, 2000.  [PUBMED Abstract]
   
   
3. Amylon MD, Shuster J, Pullen J, et al.: Intensive high-dose asparaginase consolidation improves survival for pediatric patients with T cell acute lymphoblastic leukemia and advanced stage lymphoblastic lymphoma: a Pediatric Oncology Group study. Leukemia 13 (3): 335-42, 1999.  [PUBMED Abstract]
   
   
4. Burkhardt B, Zimmermann M, Oschlies I, et al.: The impact of age and gender on biology, clinical features and treatment outcome of non-Hodgkin lymphoma in childhood and adolescence. Br J Haematol 131 (1): 39-49, 2005.  [PUBMED Abstract]
   
   
5. Burkhardt B, Woessmann W, Zimmermann M, et al.: Impact of cranial radiotherapy on central nervous system prophylaxis in children and adolescents with central nervous system-negative stage III or IV lymphoblastic lymphoma. J Clin Oncol 24 (3): 491-9, 2006.  [PUBMED Abstract]
   
   
6. Leung W, Sandlund JT, Hudson MM, et al.: Second malignancy after treatment of childhood non-Hodgkin lymphoma. Cancer 92 (7): 1959-66, 2001.  [PUBMED Abstract]
   
   

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