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]

Question 2 (10 marks)

Discuss the risk-adapted definitive management and the survivorship of classical Hodgkin lymphoma, and contrast it with the approach to the rapidly growing abdominal mass of Burkitt lymphoma and its tumour lysis risk. [3]

A full-mark answer reproduces the risk-adapted chemotherapy of Hodgkin lymphoma, the Burkitt chemotherapy, and the tumour lysis prophylaxis, and the survivorship of the two diseases. [10]

Hodgkin lymphoma definitive management (3 marks). The treatment is the risk-adapted combination chemotherapy, with the modern protocols such as the EuroNet OEPA-COPDAC or the COG ABVE-PC, reserving the involved-site radiotherapy for the slow responders and the bulky disease. The interim PET-CT guides the response-adapted de-escalation, and the complete metabolic response allows the radiotherapy to be omitted. The contemporary survival of childhood Hodgkin lymphoma is above ninety-five percent in the early stage and above ninety percent overall, making it one of the most curable cancers in medicine. [1][2]

Burkitt lymphoma management (3 marks). The contrast is the rapidly growing abdominal mass of the Burkitt lymphoma, driven by the c-MYC translocation t(8;14) and doubling every twenty-four to forty-eight hours. The Burkitt is treated with the short intensive fractionated chemotherapy, such as the LMB or the BFM group, that exploits the rapid cycling, with the central nervous system-directed therapy and the resection of the abdominal mass where feasible, and the survival is above ninety percent in the standard-risk. [3]

Tumour lysis prophylaxis (3 marks). The Burkitt carries the high tumour lysis risk, and the prophylaxis begins before the first chemotherapy dose. The three legs are the hyperhydration with an isotonic fluid without potassium to maintain a high urine output, the urate lowering with the rasburicase for the high-risk child after the glucose-6-phosphate dehydrogenase screen is clear, and the four-to-six-hourly biochemistry of the potassium, the phosphate, the calcium, the creatinine and the urate. The rasburicase is contraindicated in the glucose-6-phosphate dehydrogenase deficiency because it causes the haemolysis and the methaemoglobinaemia. The established syndrome is treated in the intensive care with the cardiac monitoring and the renal replacement therapy for the refractory hyperkalaemia and the acute kidney injury. [10]

Survivorship (1 mark). The late effects are the burden of the cure, and the Hodgkin survivor is watched for the radiotherapy-related second malignancy, the breast cancer in the irradiated girl, the thyroid and the cardiac disease, the anthracycline cardiotoxicity and the infertility, and the de-escalation aims to spare the child these. The fellow who can hold the cure and the late effects together, and the mediastinal mass and the tumour lysis together, demonstrates the breadth the boards reward. [2][10]