Lymphoma in children: SAQ

This teenager has a painless supraclavicular node, B symptoms and an anterior mediastinal mass, which together make a classical Hodgkin lymphoma the working diagnosis, and the immediate task is to protect the airway while securing the tissue diagnosis, then to stage and to treat. The contrast with the rapidly growing abdominal mass of Burkitt lymphoma tests the candidate on the tumour lysis syndrome, the non-Hodgkin biology and the histology-specific chemotherapy. ^[1][8]

Question 1 (10 marks)

Outline your immediate assessment and the airway-protective diagnostic pathway for this fifteen-year-old boy with the supraclavicular node and the anterior mediastinal mass. ^[8]

A full-mark answer covers the recognition of the mediastinal mass as an anaesthetic emergency, the airway-protective approach to the biopsy, the staging pathway, and the multidisciplinary coordination. ^[1]

Recognition and the first decision (1 mark). The combination of a painless supraclavicular node, the B symptoms of drenching night sweats and weight loss, and an anterior mediastinal mass on the chest radiograph is classical Hodgkin lymphoma until the excision biopsy proves otherwise. The first priority is the airway, because the mediastinal mass with the tracheal deviation places the boy at risk of the airway compromise under sedation and the supine position. ^[8]

Airway protection and the safe biopsy (3 marks). The boy is assessed for the stridor, the orthopnoea and the superior vena cava syndrome of the facial plethora and the distended veins, and he is kept upright. No sedation, no general anaesthesia and no supine positioning is permitted before the airway is secured in a controlled setting, because the loss of thoracic tone on induction can collapse the trachea against the mass and cause the fatal cannot-intubate cannot-ventilate situation. The supraclavicular node is biopsied under the local anaesthesia where possible, and the general anaesthetic for a mediastinal node is 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 steroids are reserved for the life-threatening obstruction, with the knowledge that they confound the biopsy. ^[8][9]

Staging pathway (3 marks). The diagnosis is made by the excision biopsy of the node with the morphology, the immunohistochemistry for the CD15 and CD30 of the classical Hodgkin, and the flow cytometry to exclude the non-Hodgkin. The staging is the Ann Arbor system with the Cotswold modifications, supported by the contrast CT of the chest, abdomen and pelvis and the whole-body fluorodeoxyglucose PET-CT for the metabolic assessment and the response. The bone marrow biopsy is performed for the advanced stage, and the laboratory panel includes the full blood count, the lactate dehydrogenase, the erythrocyte sedimentation rate, the renal and hepatic function, and the baseline echocardiogram and pulmonary function tests. ^[1][2]

Multidisciplinary coordination (3 marks). The boy is managed in the specialist paediatric oncology centre, and the multidisciplinary team is assembled: the paediatric oncologist, the anaesthetist, the surgeon, the pathologist, the radiation oncologist, the clinical nurse specialist, the social worker and the psychologist. The fertility preservation is discussed before the chemotherapy, the family is counselled on the diagnosis, the treatment phases and the expected survival, and the fever and the bleeding emergencies are taught. The boy is enrolled on the national protocol, and the survivorship surveillance is begun from the diagnosis. ^[1]