Lymphoma in children

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Target exams

Red flags

• A painless, rubbery, enlarging cervical or supraclavicular lymph node that does not settle after two weeks of antibiotics is lymphoma until excision biopsy proves otherwise
• An anterior mediastinal mass with stridor, orthopnoea, facial plethora or superior vena cava obstruction is an anaesthetic catastrophe; never sedate or intubate before the airway is secured in a controlled setting
• A rapidly growing abdominal mass with distension and pain in a young child is Burkitt lymphoma with a tumour doubling time of twenty-four hours until proven otherwise
• A bulky lymphoma with a high lactate dehydrogenase and a large tumour burden is at high risk of tumour lysis syndrome; begin hyperhydration and rasburicase before the first chemotherapy dose
• Drenching night sweats, unexplained fever and weight loss of more than ten percent of body weight are the B symptoms that upstage Hodgkin lymphoma and worsen its prognosis
• A mediastinal mass child who collapses when laid flat has airway and great-vessel compression; sit the child upright, give no sedation, and biopsy under local anaesthesia where possible

Life stages

Care settings

Clinical exam formats

Board mappings

• Separates Hodgkin from non-Hodgkin lymphoma by the presence of the Reed-Sternberg cell and by the contiguous, orderly spread of Hodgkin disease versus the high-grade, extranodal, rapidly progressive behaviour of the paediatric non-Hodgkin subtypes
• Names the four high-grade non-Hodgkin subtypes of childhood: Burkitt, lymphoblastic, diffuse large B-cell and anaplastic large cell lymphoma
• Recognises the red-flag pattern of a painless enlarging cervical or supraclavicular node, an anterior mediastinal mass with airway compromise, and a rapidly growing abdominal mass as probable lymphoma needing urgent referral
• Explains the pathogenesis of Burkitt lymphoma through the c-MYC translocation t(8;14) and its twenty-four-hour tumour doubling time, and the mediastinal mass of T-lymphoblastic lymphoma through thymic and nodal infiltration that compresses the airway and great vessels
• Coordinates the urgent diagnostic pathway from excision biopsy or core biopsy with flow cytometry, immunohistochemistry and cytogenetics to the named histology, while protecting the child with a mediastinal mass from dangerous sedation
• Applies the Ann Arbor staging to Hodgkin and the Murphy St Jude staging to non-Hodgkin lymphoma, and integrates the risk factors of bulky disease, lactate dehydrogenase and marrow involvement into the risk-adapted treatment plan
• An anterior mediastinal mass with stridor, orthopnoea or superior vena cava obstruction must never be sedated or anaesthetised before the airway is secured; biopsy under local anaesthesia is preferred and steroids are reserved for the life-threatening obstruction
• Tumour lysis syndrome prophylaxis for a high-risk lymphoma uses hyperhydration with an isotonic fluid without potassium, rasburicase for the high-risk child after glucose-6-phosphate dehydrogenase screening, and four-to-six-hourly biochemistry
• Hodgkin lymphoma is staged with contrast chest and whole-body imaging and treated with risk-adapted combination chemotherapy with or without involved-site radiotherapy, achieving survival above ninety-five percent in early-stage disease
• Counsels the family of a child newly diagnosed with lymphoma, explaining the diagnosis, the treatment phases, the expected survival and the late effects in honest and hopeful terms
• Builds the multidisciplinary plan that runs paediatric haematology-oncology, anaesthesia and intensive care for the mediastinal mass, surgery and pathology for the biopsy, and the family, school and primary care team together
• Synthesises the adolescent with Hodgkin lymphoma into a cure and survivorship plan that balances chemotherapy de-escalation against the late risks of radiotherapy, second malignancy, cardiotoxicity and infertility
• Level 1: Recognition of the persistent painless lymph node, the mediastinal mass with airway compromise and the rapidly growing abdominal mass as probable lymphoma needing urgent referral
• Level 2: Application of the excision biopsy with flow cytometry and cytogenetics and the Ann Arbor and Murphy St Jude staging to reach a named diagnosis and risk group
• Level 3: Coordination of risk-adapted multi-agent chemotherapy, mediastinal mass airway protection, tumour lysis prophylaxis and long-term survivorship surveillance
• The separation of Hodgkin from non-Hodgkin lymphoma and the four high-grade paediatric non-Hodgkin subtypes with their classic presentations and molecular drivers
• The Ann Arbor staging for Hodgkin and the Murphy St Jude staging for non-Hodgkin lymphoma, and the meaning of the B symptoms
• The oncologic emergencies of the mediastinal mass, the tumour lysis syndrome and the febrile neutropenia in the child with lymphoma
• Structured assessment of the child with lymphadenopathy for the size, site, consistency and distribution of the nodes, and for the hepatosplenomegaly, mediastinal and abdominal signs
• Communication of a new diagnosis of lymphoma to a child and family with appropriate honesty, hope and attention to the treatment duration and late effects
• Recognition of the anterior mediastinal mass as an anaesthetic emergency and the safe approach to diagnosis without sedation
• Lymphoma in a child as the third commonest malignancy, presenting as a persistent node, a mediastinal mass or an abdominal mass that demands rapid specialist referral
• The diagnostic roles of the excision biopsy with flow cytometry, immunohistochemistry and cytogenetics, and the staging imaging
• The mediastinal mass and tumour lysis syndrome as the two emergencies that govern the first hours of care
• Recognition of the red-flag features of childhood lymphoma that mandate urgent referral to paediatric haematology-oncology
• Application of the initial stabilisation of a child with a bulky lymphoma including airway protection for the mediastinal mass and tumour lysis prophylaxis
• Coordination of the diagnostic and multidisciplinary pathway for a child with newly diagnosed lymphoma
• Canadian approach to the urgent evaluation and referral of a child with a persistent node, mediastinal mass or abdominal mass suspected to be lymphoma
• Excision or core biopsy with flow cytometry and cytogenetics as the diagnostic standard, performed safely in the child with a mediastinal mass
• Risk-adapted multi-agent chemotherapy and the survivorship surveillance for late effects in the cured child