The National Cancer Institute (NCI) 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.

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 to ensure that children receive treatment, supportive care, and rehabilitation that will achieve optimal survival and quality of life. Refer to the PDQ Supportive and Palliative 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 has been achieved through clinical trials. Information about ongoing clinical trials is available from the NCI 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 summary on the Late Effects of Treatment for Childhood Cancer for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.

Lymphoma (Hodgkin lymphoma and non-Hodgkin lymphoma [NHL]) is the third most common childhood malignancy, and NHL accounts for approximately 7% of cancers in children younger than 20 years.[2,3] In the United States, about 800 new cases of NHL are diagnosed each year. The incidence is approximately 10 cases per 1,000,000 people per year. Although there is no sharp age peak, NHL occurs most commonly in the second decade of life, and occurs less frequently in children younger than 3 years. NHL in infants is rare (1% in Berlin-Frankfurt-Munster trials from 1986 to 2002). Retrospective review demonstrated an inferior outcome for infants when compared with older patients with NHL.[4][Level of evidence: 3iiA] The incidence of NHL is increasing overall, and there is a slight increase in the incidence for those aged 15 to 19 years; however, the incidence of NHL in children younger than 15 years has remained constant over the past several decades.[2] The incidence of NHL is higher in Caucasians than in African Americans, and NHL is more common in males than in females.[2,5] Immunodeficiency, both congenital and acquired (human immunodeficiency virus infection or posttransplant immunodeficiency), increases the risk of NHL. Epstein-Barr virus is associated with most cases of NHL seen in the immunodeficient population.[2,3]

With current treatments, about 80% of children and adolescents with NHL will survive at least 5 years, though outcome is variable depending on a number of factors.[5] The most important prognostic determinant, given optimal therapy, is the extent of disease at diagnosis as determined by pretreatment staging. Patients with localized disease (i.e., single extra-abdominal/extrathoracic tumor or totally resected intra-abdominal tumor) have an excellent prognosis (a 5-year survival rate of approximately 90%), regardless of histology.[3,6,7] Patients with NHL arising in bone have an excellent prognosis regardless of histology,[8,9] and testicular disease does not affect prognosis.[10,11] Unlike adults, children and adolescents with nonlymphoblastic NHL involving the mediastinum have an inferior outcome, as compared with other sites of disease.[5,12,13] Patients with intrathoracic or extensive intra-abdominal disease and patients with bone marrow or central nervous system involvement at diagnosis require intensive therapy.[3,5,7,14] These intensive therapies have improved the outcome for patients with disseminated or advanced-stage disease.

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

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., pp 35-50. Also available online. Last accessed April 18, 2007. 
   
   
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. Mann G, Attarbaschi A, Burkhardt B, et al.: Clinical characteristics and treatment outcome of infants with non-Hodgkin lymphoma. Br J Haematol 139 (3): 443-9, 2007.  [PUBMED Abstract]
   
   
5. 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]
   
   
6. Link MP, Shuster JJ, Donaldson SS, et al.: Treatment of children and young adults with early-stage non-Hodgkin's lymphoma. N Engl J Med 337 (18): 1259-66, 1997.  [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]
   
   
9. Zhao XF, Young KH, Frank D, et al.: Pediatric primary bone lymphoma-diffuse large B-cell lymphoma: morphologic and immunohistochemical characteristics of 10 cases. Am J Clin Pathol 127 (1): 47-54, 2007.  [PUBMED Abstract]
   
   
10. Dalle JH, Mechinaud F, Michon J, et al.: Testicular disease in childhood B-cell non-Hodgkin's lymphoma: the French Society of Pediatric Oncology experience. J Clin Oncol 19 (9): 2397-403, 2001.  [PUBMED Abstract]
    
    
11. 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]
    
    
12. Woessmann W, Seidemann K, Mann G, et al.: The impact of the methotrexate administration schedule and dose in the treatment of children and adolescents with B-cell neoplasms: a report of the BFM Group Study NHL-BFM95. Blood 105 (3): 948-58, 2005.  [PUBMED Abstract]
    
    
13. Patte C, Auperin A, Gerrard M, et al.: Results of the randomized international FAB/LMB96 trial for intermediate risk B-cell non-Hodgkin lymphoma in children and adolescents: it is possible to reduce treatment for the early responding patients. Blood 109 (7): 2773-80, 2007.  [PUBMED Abstract]
    
    
14. Cairo MS, Gerrard M, Sposto R, et al.: Results of a randomized international study of high-risk central nervous system B non-Hodgkin lymphoma and B acute lymphoblastic leukemia in children and adolescents. Blood 109 (7): 2736-43, 2007.  [PUBMED Abstract]
    
    

Back to Top

< Previous Section  |  Next Section >