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ASCO Educational Book; 2009
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Clinical Features and Treatment Approaches for the Uncommon Non-Hodgkin Lymphoma Subtypes in Children

John T. Sandlund, MD

From Oncology and Leukemia/Lymphoma, St. Jude Children's Research Hospital, Memphis, TN

Author's disclosure of potential conflicts of interest are found at the end of this article.

Supported in part by grants from the National Cancer Institute (CA 21765) and the American Lebanese Syrian Associated Charities (ALSAC).

Address reprint requests to John T. Sandlund, MD, Oncology and Leukemia/Lymphoma, St. Jude Children's Research Hospital, MS 260, Room C-6063, 262 Danny Thomas Pl, Memphis, TN 38105-3678; e-mail: John.Sandlund{at}stjude.org

Overview: There is relatively little controversy regarding the global treatment strategies for the commonly encountered non-Hodgkin's lymphomas (NHLs) of childhood. However, for children who present with an uncommon NHL histologic subtype, optimal treatment approaches have yet to be fully agreed on, thus posing a significant challenge for the pediatric oncologist. Among those children who present with either follicular or marginal zone B-cell lymphoma (both mature B-cell lymphomas), conservative approaches are generally effective, except in certain high-risk cases. Among those who present with a mature T-cell subtype (i.e., hepatosplenic T-cell lymphoma, subcutaneous panniculitis-like T-cell lymphoma, extranodal natural killer/T-cell lymphoma [nasal type], and mycosis fungoides), the prognosis is generally poorer despite more aggressive treatment approaches. Additional research that focuses on both the clinical and biologic features of these uncommon NHL subtypes is clearly needed and may provide insights into novel targeted approaches to therapy.