General Information

This cancer treatment information summary provides an overview of the prognosis, diagnosis, classification, staging, and treatment of childhood non-Hodgkin’s lymphoma (NHL).

The National Cancer Institute provides the PDQ pediatric cancer treatment information summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public. These summaries are updated regularly according to the latest published research findings by an Editorial Board of pediatric oncology specialists.

Cancer in children and adolescents is rare. Children and adolescents with cancer should be referred to medical centers that have a multidisciplinary team of cancer specialists with experience treating the cancers that occur during childhood and adolescence. This multidisciplinary team approach incorporates the skills of the primary care physician, pediatric surgical subspecialists, radiation oncologists, pediatric medical oncologists/hematologists, rehabilitation specialists, pediatric nurse specialists, social workers, and others in order to ensure that children receive treatment, supportive care, and rehabilitation that will achieve optimal survival and quality of life. Refer to the PDQ Supportive Care summaries for specific information about supportive care for children and adolescents with cancer.

Guidelines for pediatric cancer centers and their role in the treatment of children with cancer have been outlined by the American Academy of Pediatrics.[1] At these pediatric cancer centers, clinical trials are available for most of the types of cancer that occur in children and adolescents, and the opportunity to participate in these trials is offered to most patients/families. Clinical trials for children and adolescents with cancer are generally designed to compare potentially better therapy with therapy that is currently accepted as standard. Most of the progress made in identifying curative therapies for childhood cancers have been achieved through clinical trials. Information about ongoing clinical trials is available from the NCI Cancer.gov Web site.

In recent decades, dramatic improvements in survival have been achieved for children and adolescents with cancer. Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment. Refer to the PDQ Late Effects of Childhood Cancer Therapies summary for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.

Lymphoma (Hodgkin’s and non-Hodgkin’s) is the third most common childhood malignancy, and non-Hodgkin’s lymphoma (NHL) accounts for approximately 7% of cancers in children less than 20 years of age.[2,3] In the United States, there are about 800 new cases of NHL diagnosed each year. Incidence is approximately 10 per 1,000,000. Although there is no sharp age peak, NHL occurs most commonly in the second decade of life, and occurs less frequently in children less than 3 years of age. NHL is the most frequent malignancy in children with AIDS, and it often occurs before the age of 4 years in those who have vertical transmission of the virus.[4] Screening for HIV should be considered for all children with NHL.[5,6]

More than 70% of children and adolescents with NHL will survive at least 5 years with chemotherapy although outcome is variable depending on a number of factors.[7] The most important prognostic determinant, given optimal therapy, is the extent of disease at diagnosis as determined by pretreatment staging. Patients with a single extra-abdominal/extrathoracic tumor and those with totally resected intra-abdominal tumor have an excellent prognosis (a 5-year survival rate of approximately 90%), regardless of histology. Patients with NHL arising in bone also have an excellent prognosis regardless of histology.[8] Patients with extensive intrathoracic or intra-abdominal disease and patients with bone marrow or central nervous system involvement at diagnosis require intensified therapy.[2] These therapies have improved the outcome for patients with advanced stage disease.

Information about ongoing clinical trials is available from the NCI Cancer.gov Web site.

(Refer to the PDQ summary on Primary CNS Lymphoma Treatment for more information on treatment options for non-AIDS-related primary central nervous system (CNS) lymphoma.)

References

1. Guidelines for the pediatric cancer center and role of such centers in diagnosis and treatment. American Academy of Pediatrics Section Statement Section on Hematology/Oncology. Pediatrics 99 (1): 139-41, 1997.  [PUBMED Abstract]
   
2. Percy CL, Smith MA, Linet M, et al.: Lymphomas and reticuloendothelial neoplasms. In: Ries LA, Smith MA, Gurney JG, et al., eds.: Cancer incidence and survival among children and adolescents: United States SEER Program 1975-1995. Bethesda, Md: National Cancer Institute, SEER Program, 1999. NIH Pub.No. 99-4649. Also available online. Last accessed April 5, 2005., pp 35-50. Also available online. Last accessed March 29, 2004. 
   
3. Sandlund JT, Downing JR, Crist WM: Non-Hodgkin's lymphoma in childhood. N Engl J Med 334 (19): 1238-48, 1996.  [PUBMED Abstract]
   
4. Evans JA, Gibb DM, Holland FJ, et al.: Malignancies in UK children with HIV infection acquired from mother to child transmission. Arch Dis Child 76 (4): 330-3, 1997.  [PUBMED Abstract]
   
5. McClain KL, Joshi VV, Murphy SB: Cancers in children with HIV infection. Hematol Oncol Clin North Am 10 (5): 1189-201, 1996.  [PUBMED Abstract]
   
6. Serraino D, Franceschi S: Kaposi's sarcoma and non-Hodgkin's lymphomas in children and adolescents with AIDS. AIDS 10 (6): 643-7, 1996.  [PUBMED Abstract]
   
7. Pinkerton CR: The continuing challenge of treatment for non-Hodgkin's lymphoma in children. Br J Haematol 107 (2): 220-34, 1999.  [PUBMED Abstract]
   
8. Lones MA, Perkins SL, Sposto R, et al.: Non-Hodgkin's lymphoma arising in bone in children and adolescents is associated with an excellent outcome: a Children's Cancer Group report. J Clin Oncol 20 (9): 2293-301, 2002.  [PUBMED Abstract]

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