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Epidemiology

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Lymphoma represents the third most common pediatric cancer in children between the ages of 0 to 19 years of age.1Sixty percent of all childhood and adolescent lymphomas are classified as non-Hodgkin lymphoma (NHL). NHL represents approximately 8% to 10% of all malignancies that occur in children between ages 5 to 19 years. Approximately 750 to 800 cases of NHL are diagnosed annually in children and adolescents younger than age 20 years in the United States. Non-Hodgkin lymphoma occurs throughout childhood and adolescence starting as early as infancy and increasing in frequency during adolescence with a preponderance of a 3:1 male to female ratio. The incidence of NHL in Caucasians is approximately twice that found in African American children with approximately 9.2 versus 4.6 cases per million.2-4 Risk factors for the development of NHL in children and adolescents are largely unknown, except that children with either inherited and/or acquired immunodeficiencies have a significantly increased risk for developing NHL.

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Pathology

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As opposed to non-Hodgkin lymphoma (NHL) seen in adults, over 95% of childhood and adolescent NHL has been classified to be either an intermediate or aggressive pathological subtype by the World Health Organization (WHO) classification.5The WHO classification, which is the most recent classification for NHL, incorporates morphological, immunophenotypic, and molecular characteristics to subclassify each type of lymphoma. There are four major pathological subtypes of childhood NHL and the distribution of these four histological subtypes include Burkitt lymphoma (BL) (40%), diffuse large B-cell lymphoma (DLBCL) (20%), lymphoblastic lymphoma (LL) (30%) and anaplastic large-cell lymphoma (ALCL) (10%) (Fig. 451-1).2,3,6The remaining (5%) of childhood NHL occurs in very rare subtypes including some indolent B-cell lymphomas, such as follicular lymphoma and rare T-cell subtypes including peripheral T-cell lymphoma or subtypes of natural killer cell lymphomas.7

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Figure 451-1.
Graphic Jump Location

Hematoxylin and eosin staining of morphology of the 4 most common subgroups of childhood and adolescent non-Hodgkin lymphoma (NHL): (A) Burkitt lymphoma (BL)—high power, (B) diffuse large B-cell lymphoma (DLBCL)—high power, (C) precursor T-lymphoblastic lymphoma—high power, (D) anaplastic large cell lymphoma (ALCL)—high power.

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Clinical Presentation, Diagnostic Evaluation, and Staging

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The clinical manifestations of childhood and adolescent non-Hodgkin lymphoma (NHL) depends primarily on the pathological subtype and the primary site of involvement.2,3,7-9 Less than 10% of childhood and adolescent NHL presents in peripheral nodal tissue; the majority presents either in extranodal lymphoid tissue, including the thymus or Peyer patches within the gastrointestinal tract, or manifests in nonlymphatic tissue such as bone marrow, spinal fluid, skin, bone, gonads, or within the central nervous system (CNS). The most common sites of presentation include head and neck (30%), abdomen (30%), and mediastinal and/or hilar lymph nodes (30%). Each pathological subtype has distinct primary ...

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