Treatment Option Overview for Childhood NHL

Many of the advancements in childhood cancer survival have been made by using combinations of known and/or new agents to improve the best available, accepted therapy. Clinical trials in pediatrics are designed to compare potentially better therapy with currently accepted standard therapy. This comparison may be done in a randomized study of two treatment arms or by evaluating a single new treatment and comparing the results with those previously obtained with standard therapy.

All children with non-Hodgkin lymphoma (NHL) should consider enrolling in a clinical trial. Treatment planning by a multidisciplinary team of cancer specialists with experience treating tumors of childhood is strongly recommended to determine, coordinate, and implement treatment to achieve optimal survival. Children with NHL should be referred for treatment by a multidisciplinary team of pediatric oncologists at an institution with experience in treating pediatric cancers. Information about ongoing National Cancer Institute (NCI)–supported clinical trials is available from the NCI website.

NHL in children is generally considered to be widely disseminated at diagnosis, even when the tumor is apparently localized. As a result, combination chemotherapy is recommended for most patients.[1] Exceptions to this treatment strategy include the following:

In contrast to the treatment of adults with NHL, the use of radiation therapy is limited in children with NHL. Study results include the following:

Radiation therapy may have a role in treating patients who have not had a complete response to chemotherapy. Data to support limiting the use of radiation therapy in the treatment of pediatric NHL come from the Childhood Cancer Survivor Study.[7] This analysis demonstrated that radiation exposure was a significant risk factor for subsequent neoplasms and death in long-term survivors.

The treatment of NHL in childhood and adolescence has historically been based on the histological subtype of the disease. A study by the Children’s Cancer Group demonstrated that the outcomes for patients with lymphoblastic lymphoma were superior with longer acute lymphoblastic leukemia–like therapy, while patients with nonlymphoblastic NHL (Burkitt lymphoma) had superior outcomes with short, intensive, pulsed therapy. The outcomes for patients with large cell lymphoma were similar with either approach.[8]

Outcomes for children and adolescents with recurrent NHL remain very poor, with the exception of patients with anaplastic large cell lymphoma.[9-14] Patients or families who desire additional disease-directed therapy should consider entering trials of novel therapeutic approaches. Regardless of whether a decision is made to pursue disease-directed therapy at the time of progression, palliative care remains a central focus of management. This ensures that quality of life is maximized while attempting to reduce symptoms and stress related to terminal illness.

Table 3 describes the treatment options for newly diagnosed and recurrent childhood NHL.