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Key Features

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Essentials of Diagnosis
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  • 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

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General Considerations
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  • 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

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Clinical Findings

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  • 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

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Diagnosis

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Laboratory Findings
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  • 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

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Imaging
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  • Chest radiograph and chest CT scan, an abdominal ultrasound or CT scan, and possibly a PET scan should be considered

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Diagnostic Procedures
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  • 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)

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Treatment

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  • 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 years

  • Treatment of Burkitt lymphoma and Burkitt-like lymphoma using ...

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