Paeds · haematology-oncology-and-transfusion

Lymphoma in children

Also known as Hodgkin lymphoma · Non-Hodgkin lymphoma · Burkitt lymphoma · Lymphoblastic lymphoma · Mediastinal mass syndrome · Superior vena cava syndrome

Fellowship guide to lymphoma in children. Covers the separation of Hodgkin from non-Hodgkin lymphoma, the four high-grade paediatric non-Hodgkin subtypes of Burkitt, lymphoblastic, diffuse large B-cell and anaplastic large cell, the Reed-Sternberg cell and Ann Arbor staging of Hodgkin lymphoma, the Murphy St Jude staging for the non-Hodgkin group, the pathophysiology of c-MYC and t(8;14) in Burkitt and the mediastinal mass of T-lymphoblastic disease, the urgent diagnostic pathway from excision biopsy with flow cytometry and cytogenetics, the anterior mediastinal mass as an anaesthetic catastrophe that forbids sedation before the airway is secured, the stabilisation with tumour lysis prophylaxis using hyperhydration and rasburicase, and the risk-adapted multi-agent chemotherapy that delivers survival above ninety percent.

high11 referencesUpdated 16 July 202628 min readVerification in progress

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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 otherwiseAn 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 settingA 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 otherwiseA 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 doseDrenching 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 prognosisA 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

infanttoddlerpreschoolschool-ageadolescentyoung-adult-transition

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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 subtypesNames the four high-grade non-Hodgkin subtypes of childhood: Burkitt, lymphoblastic, diffuse large B-cell and anaplastic large cell lymphomaRecognises 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 referralExplains 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 vesselsCoordinates 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 sedationApplies 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 planAn 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 obstructionTumour 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 biochemistryHodgkin 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 diseaseCounsels 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 termsBuilds 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 togetherSynthesises 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 infertilityLevel 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 referralLevel 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 groupLevel 3: Coordination of risk-adapted multi-agent chemotherapy, mediastinal mass airway protection, tumour lysis prophylaxis and long-term survivorship surveillanceThe separation of Hodgkin from non-Hodgkin lymphoma and the four high-grade paediatric non-Hodgkin subtypes with their classic presentations and molecular driversThe Ann Arbor staging for Hodgkin and the Murphy St Jude staging for non-Hodgkin lymphoma, and the meaning of the B symptomsThe oncologic emergencies of the mediastinal mass, the tumour lysis syndrome and the febrile neutropenia in the child with lymphomaStructured assessment of the child with lymphadenopathy for the size, site, consistency and distribution of the nodes, and for the hepatosplenomegaly, mediastinal and abdominal signsCommunication of a new diagnosis of lymphoma to a child and family with appropriate honesty, hope and attention to the treatment duration and late effectsRecognition of the anterior mediastinal mass as an anaesthetic emergency and the safe approach to diagnosis without sedationLymphoma in a child as the third commonest malignancy, presenting as a persistent node, a mediastinal mass or an abdominal mass that demands rapid specialist referralThe diagnostic roles of the excision biopsy with flow cytometry, immunohistochemistry and cytogenetics, and the staging imagingThe mediastinal mass and tumour lysis syndrome as the two emergencies that govern the first hours of careRecognition of the red-flag features of childhood lymphoma that mandate urgent referral to paediatric haematology-oncologyApplication of the initial stabilisation of a child with a bulky lymphoma including airway protection for the mediastinal mass and tumour lysis prophylaxisCoordination of the diagnostic and multidisciplinary pathway for a child with newly diagnosed lymphomaCanadian approach to the urgent evaluation and referral of a child with a persistent node, mediastinal mass or abdominal mass suspected to be lymphomaExcision or core biopsy with flow cytometry and cytogenetics as the diagnostic standard, performed safely in the child with a mediastinal massRisk-adapted multi-agent chemotherapy and the survivorship surveillance for late effects in the cured child

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Target exams

RACP DWERACP DCEMRCPCH TheoryMRCPCH ClinicalABP General Pediatrics

Red flags

Life stages

infanttoddlerpreschoolschool-ageadolescentyoung-adult-transition

Care settings

outpatientwarded-acutepicuretrieval

Clinical exam formats

written-only

Board mappings

Overview & Definition

A teenager arrives with a rubbery lump above the clavicle that has been growing for a month, and a younger child is rushed in breathless and blue in the face with a chest that is full of mass and empty of airway. Both have lymphoma, and both demand that the clinician move fast for very different reasons. Lymphoma is the malignant proliferation of lymphoid cells that begins in the lymph nodes or in extranodal lymphoid tissue, and it is the third commonest cancer of childhood after leukaemia and the brain tumours. The word itself carries less weight than the single distinction that splits it in two at the bedside, because Hodgkin and non-Hodgkin lymphoma behave so differently that the first question asked of any child with a suspicious node is which side of that line the disease falls on. [1][5]

Hodgkin lymphoma is defined by the presence of the Reed-Sternberg cell, a large binucleate B-cell derivative with the owl-eye nucleolus that sits within a background of reactive inflammatory cells. It spreads in an orderly, contiguous fashion from one lymph node group to the next, it tends to stay nodal, and it is one of the most curable cancers in all of medicine. Non-Hodgkin lymphoma, in the child, is a different creature entirely, because the paediatric form is almost always high-grade, extranodal, and fast. The childhood non-Hodgkin lymphomas double in size every twenty-four to forty-eight hours, they seed marrow and the central nervous system, and a delay of days can move a child from curable to catastrophic. [1][3]

The clinical gravity of the disease is concentrated in two emergencies that frame this whole topic, and a candidate who masters them masters the examination question. The first is the anterior mediastinal mass, most often the T-lymphoblastic lymphoma, which compresses the airway and the great vessels and turns the act of going to sleep for a biopsy into a gamble with death. The second is the tumour lysis syndrome, the metabolic collapse that follows the rapid destruction of a bulky lymphoma, which is prevented before the first dose of chemotherapy or not at all. Around these two emergencies sits a curable disease, because the modern risk-adapted chemotherapy of childhood lymphoma delivers survival that exceeds ninety percent across the group. [8][10]

One-sentence answer for the exam

A child with lymphoma has a malignant proliferation of lymphoid tissue that splits into the Reed-Sternberg-cell, orderly, nodal and highly curable Hodgkin disease and the high-grade, extranodal, rapidly growing non-Hodgkin group of Burkitt, lymphoblastic, diffuse large B-cell and anaplastic large cell lymphoma; the diagnosis is made by excision biopsy with flow cytometry and cytogenetics, staged by the Ann Arbor system for Hodgkin and the Murphy St Jude system for non-Hodgkin, and the two emergencies that govern the first hours are the anterior mediastinal mass that forbids sedation until the airway is secured and the tumour lysis syndrome that is prevented with hyperhydration and rasburicase before the first chemotherapy dose, after which risk-adapted multi-agent chemotherapy delivers survival above ninety percent.

[1][3][8][10]

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References11Show ledger

[1]Mauz-Körholz C, Metzger ML, Kelly KM, et al. Pediatric Hodgkin Lymphoma J Clin Oncol, 2015.PMID 26304892
[2]Munir F, Hardit V, Sheikh IN Classical Hodgkin Lymphoma: From Past to Future-A Comprehensive Review of Pathophysiology and Therapeutic Advances Int J Mol Sci, 2023.PMID 37373245
[3]López C, Burkhardt B, Chan JKC, et al. Burkitt lymphoma Nat Rev Dis Primers, 2022.PMID 36522349
[4]Molyneux EM, Rochford R, Griffin B Burkitt's lymphoma Lancet, 2012.PMID 22333947
[5]Galardy PJ, Bedekovics T, Hermiston ML Targeting childhood, adolescent and young adult non-Hodgkin lymphoma: therapeutic horizons Br J Haematol, 2016.PMID 27019108
[6]Temple WC, Mueller S, Hermiston ML Diagnosis and management of lymphoblastic lymphoma in children, adolescents and young adults Best Pract Res Clin Haematol, 2023.PMID 36907639
[7]Lowe EJ, Woessmann W Anaplastic large cell lymphoma in children and adolescents Br J Haematol, 2025.PMID 40351161
[8]Pearson JK, Tan GM Pediatric Anterior Mediastinal Mass: A Review Article Semin Cardiothorac Vasc Anesth, 2015.PMID 25814524
[9]Garey CL, Laituri CA, Valusek PA Management of anterior mediastinal masses in children Eur J Pediatr Surg, 2011.PMID 21751123
[10]Perissinotti AJ, Bishop MR, Bubalo J Expert consensus guidelines for the prophylaxis and management of tumor lysis syndrome in the United States: Results of a modified Delphi panel Cancer Treat Rev, 2023.PMID 37579533
[11]Quintanilla-Martinez L, Swerdlow SH, Tousseyn T New concepts in EBV-associated B, T, and NK cell lymphoproliferative disorders Virchows Archiv, 2023.PMID 36216980

Lymphoma in children

Also known as Hodgkin lymphoma · Non-Hodgkin lymphoma · Burkitt lymphoma · Lymphoblastic lymphoma · Mediastinal mass syndrome · Superior vena cava syndrome

high11 referencesUpdated 16 July 202628 min readVerification in progress

Practise this topic

Overview & Definition

Paediatrics Fellowship Pro

You have read the opening of this topic. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References11Show ledger

[1]Mauz-Körholz C, Metzger ML, Kelly KM, et al. Pediatric Hodgkin Lymphoma J Clin Oncol, 2015.PMID 26304892
[2]Munir F, Hardit V, Sheikh IN Classical Hodgkin Lymphoma: From Past to Future-A Comprehensive Review of Pathophysiology and Therapeutic Advances Int J Mol Sci, 2023.PMID 37373245
[3]López C, Burkhardt B, Chan JKC, et al. Burkitt lymphoma Nat Rev Dis Primers, 2022.PMID 36522349
[4]Molyneux EM, Rochford R, Griffin B Burkitt's lymphoma Lancet, 2012.PMID 22333947
[5]Galardy PJ, Bedekovics T, Hermiston ML Targeting childhood, adolescent and young adult non-Hodgkin lymphoma: therapeutic horizons Br J Haematol, 2016.PMID 27019108
[6]Temple WC, Mueller S, Hermiston ML Diagnosis and management of lymphoblastic lymphoma in children, adolescents and young adults Best Pract Res Clin Haematol, 2023.PMID 36907639
[7]Lowe EJ, Woessmann W Anaplastic large cell lymphoma in children and adolescents Br J Haematol, 2025.PMID 40351161
[8]Pearson JK, Tan GM Pediatric Anterior Mediastinal Mass: A Review Article Semin Cardiothorac Vasc Anesth, 2015.PMID 25814524
[9]Garey CL, Laituri CA, Valusek PA Management of anterior mediastinal masses in children Eur J Pediatr Surg, 2011.PMID 21751123
[10]Perissinotti AJ, Bishop MR, Bubalo J Expert consensus guidelines for the prophylaxis and management of tumor lysis syndrome in the United States: Results of a modified Delphi panel Cancer Treat Rev, 2023.PMID 37579533
[11]Quintanilla-Martinez L, Swerdlow SH, Tousseyn T New concepts in EBV-associated B, T, and NK cell lymphoproliferative disorders Virchows Archiv, 2023.PMID 36216980