Lymphoma in children: Case

Clinical long case of a fifteen-year-old boy presenting with a painless enlarging supraclavicular node, B symptoms and a large anterior mediastinal mass, covering the airway-protective diagnostic pathway with the excision biopsy and the flow cytometry, the Ann Arbor staging with the contrast CT and the PET-CT, the risk-adapted combination chemotherapy with the response-adapted radiotherapy de-escalation, the survivorship surveillance for the late effects, and the contrast with the Burkitt lymphoma and its tumour lysis risk.

paediatric haematology long case5 min readVerification in progress

Prompt

A fifteen-year-old boy is brought to the emergency department with a three-week history of a painless, enlarging, rubbery mass in the right supraclavicular fossa, drenching night sweats that soak the bedclothes, and a six-kilogram weight loss. He has noticed a dry cough and a sense of breathlessness when he lies flat. On examination he is comfortable sitting upright, with a three-centimetre rubbery non-tender supraclavicular node, and the chest radiograph shows a large anterior mediastinal mass with tracheal deviation. The examiner asks how you frame the problem, how you protect the airway during the diagnosis, how you stage and treat the disease, and how your approach changes for the rapidly growing abdominal mass of a younger child.

Framing the case

This fifteen-year-old boy has the classic presentation of a classical Hodgkin lymphoma. The painless enlarging rubbery supraclavicular node, the B symptoms of the drenching night sweats and the six-kilogram weight loss, and the anterior mediastinal mass with the tracheal deviation together make the diagnosis, and the framework that organises the case is the separation of the Hodgkin from the non-Hodgkin lymphoma and the airway-protective approach to the mediastinal mass. The first priority is the airway, because the boy is orthopnoeic and the mediastinal mass deviates the trachea. ^[1]^[8]

The airway-protective diagnosis

The assessment begins with the airway, because the boy with the mediastinal mass is the one who can die during the procedure. He is comfortable sitting upright but breathless when laid flat, which marks the airway as not safe for the supine general anaesthetic. The principle is absolute: no sedation, no general anaesthesia and no supine positioning is permitted before the airway is secured in a controlled setting. The supraclavicular node is biopsied under the local anaesthesia, because it is accessible, it preserves the architecture for the morphology and the immunohistochemistry, and it avoids the general anaesthetic. ^[8]^[9]

If a mediastinal node were the only accessible target, the general anaesthetic would be reserved for the airway assessed as safe by the anaesthetic, the ENT and the cardiothoracic teams together, with the rigid bronchoscope and the ECMO on standby, the spontaneous ventilation maintained and the supine position avoided. The steroids would be reserved for the life-threatening obstruction, because they lyse the lymphoma and confound the histology, and the decision would be documented with the oncology and the anaesthesia teams. ^[8]

References6Show

[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
[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