Radiation Oncology/NHL/Mantle cell lymphoma

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Front Page: Radiation Oncology | RTOG Trials | Randomized Trials

Non-Hodgkin lymphoma: Main Page | Randomized
Overview: Overview | Follicular | Diffuse large B-cell | MALT (extranodal marginal zone) | Nodal marginal zone | Mantle cell | CLL/SLL | Lymphoblastic | Burkitt | NK/T cell | Anaplastic large cell | Primary CNS Lymphoma
Treatment: Low grade and follicular | Diffuse and high grade | Marginal zone | Mantle cell | CLL/SLL | Lymphoblastic | Specific sites | Radioimmunotherapy


Contents

[edit] Overview

  • Rare subtype, comprises ~6% of NHL in US
  • Usually in older men
  • Only 5-15% present with Stage I-II
  • Most often Stage III or IV disease, extranodal involvement including spleen and liver and bone marrow common, frequent B symptoms (about 50%).
  • A variant can present as multiple polyps throughout the GI tract (lymphomatous polyposis)
  • Microscopically they have "angulated" nuclei, and resemble lymphocytes in mantle zone adjacent to the lymphoid follicles
  • Translocation t(11;14) rearranges the Bcl-1 gene (PRAD-1) which codes for Cyclin D1 (involved in cell cycle regulation) and puts it under the control of the immunoglobulin gene promotor.
  • Very poor survival. Aggressive and doesn't respond well to chemotherapy. Subset of elderly patients can present asymptomatic and have a very indolent course


[edit] Treatment

  • CHOP alone not very effective, early SWOG experience showed median OS 3 years
  • Addition of Rituximab (R-CHOP) did not improve PFS or OS
  • More intense chemotherapy regimens required
  • In asymptomatic elderly with limited disease, careful observation may be considered
  • RT has a potentially significant role in Stage I-II disease, as MCL is very radiosensitive


[edit] Limited Stage

  • British Columbia; 2003 PMID 14504058 -- "Limited-stage mantle-cell lymphoma." (Leitch HA, Ann Oncol. 2003 Oct;14(10):1555-61.)
    • Retrospective. 26 patients. Initial therapy IFRT +/- chemo (n=17), observation or chemo (n=9)
    • 5-year outcome: PFS 46%, OS 70%
    • Predictors: age (PFS <60 83% vs. >60 39%, SS), RT (PFS RT 68% vs. no RT 11%; OS 71% vs. 25%, NS)
    • Conclusion: Limited-stage MCL had improved outcome with RT


[edit] Advanced Stage

  • GLSG (Germany)
    • Randomized. 122 untreated patients, advanced-stage MCL. Treated with Arm 1) CHOP vs. Arm 2) R-CHOP. Patients <=65 with CR/PR second randomization to ASCT vs. IFN-alpha. Patients >65 received IFN-alpha maintenance
    • 2005 PMID 15668467 -- "Immunochemotherapy with rituximab and cyclophosphamide, doxorubicin, vincristine, and prednisone significantly improves response and time to treatment failure, but not long-term outcome in patients with previously untreated mantle cell lymphoma: results of a prospective randomized trial of the German Low Grade Lymphoma Study Group (GLSG)." (Lenz G, J Clin Oncol. 2005 Mar 20;23(9):1984-92.)
      • Outcome: R-CHOP better for CR (34% vs. 7%, SS), and TTF (21 months vs. 14 months, SS), but no difference in PFS or OS
      • Conclusion: Better response rate, but no diffference in PFS or OS
  • SWOG; 1995 -- PMID 7849295 -- "A clinical analysis of two indolent lymphoma entities: mantle cell lymphoma and marginal zone lymphoma (including the mucosa-associated lymphoid tissue and monocytoid B-cell subcategories): a Southwest Oncology Group study. (Fisher RI, Blood. 1995 Feb 15;85(4):1075-82.)
    • Retrospective. Treated with CHOP on SWOG-7204, SWOG-7426, SWOG-7713
    • Outcome: median FFS 1.7 years, median OS 3 years
    • Conclusion: MCL is not an indolent lymphoma, and requires innovative therapy

[edit] Reviews

  • Mayo; 2005 - PMID 16155027 — "Current Treatment Approaches for Mantle-Cell Lymphoma." Witzig TE et al. J Clin Oncol. Vol 23, No 26 (September 10), 2005: pp. 6409-6414.
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