Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ Key Features +++ Essentials of Diagnosis ++ Virtually all childhood NHLs are rapidly proliferating, high-grade, diffuse malignancies Tumors exhibit aggressive behavior but are usually very responsive to treatment Can arise in any site of lymphoid tissue, including the lymph nodes, thymus, liver, and spleen Common extralymphatic sites include bone, bone marrow, CNS, skin, and testes +++ General Considerations ++ Diverse group of cancers accounting for 5–10% of malignancies in children younger than 15 years About 500 new cases arise per year in the United States Incidence increases with age Male predominance of approximately 3:1 Nearly all pediatric NHLs are histologically classified into four main groups Lymphoblastic lymphoma Small noncleaved cell lymphoma (Burkitt lymphoma and Burkitt-like lymphoma) Large B-cell lymphoma Anaplastic large cell lymphoma +++ Clinical Findings ++ Lymphoblastic lymphoma Airway compression (cough, dyspnea, orthopnea) Superior vena cava obstruction (facial edema, chemosis, plethora, venous engorgement) These symptoms are a true emergency necessitating rapid diagnosis and treatment Pleural or pericardial effusions may further compromise the patient's respiratory and cardiovascular status CNS and bone marrow involvement are not common at diagnosis Burkitt lymphoma and Burkitt-like lymphoma Abdominal pain, distention, a right lower quadrant mass, or intussusception in a child older than 5 years Bone marrow involvement is common (~65% of patients) Burkitt lymphoma is the most rapidly proliferating tumor known and has a high rate of spontaneous cell death as it outgrows its blood supply Consequently, children with massive abdominal disease frequently have tumor lysis syndrome (hyperuricemia, hyperphosphatemia, and hyperkalemia) Large cell lymphomas Clinically similar to the small noncleaved cell lymphomas, although unusual sites of involvement are quite common Skin lesions, focal neurologic deficits, and pleural or peritoneal effusions without an obvious associated mass are frequently seen +++ Diagnosis +++ Laboratory Findings ++ CBC, liver function tests, and a biochemical profile (electrolytes, calcium, phosphorus, uric acid, renal function) should be obtained Elevated lactate dehydrogenase Reflects tumor burden Can serve as a marker of disease activity +++ Imaging ++ Chest radiograph and chest CT scan, an abdominal ultrasound or CT scan, and possibly a PET scan should be considered +++ Diagnostic Procedures ++ Bone marrow and CSF examinations are essential Diagnosis is made by biopsy of involved tissue with histology, immunophenotyping, and cytogenetic studies Occasionally necessary to initiate corticosteroids or low-dose emergency radiation therapy until mass is small enough for biopsy to be done safely Response to this approach usually prompt (12–24 hours) +++ Treatment ++ Nearly all patients require intensive intrathecal chemotherapy for CNS prophylaxis Surgical resection is not indicated unless the entire tumor can be resected safely, which is rare Partial resection or debulking surgery has no role Therapy for lymphoblastic lymphoma involves dose-intensive, multiagent chemotherapy; duration of therapy is 2 ... Your MyAccess profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth