Lymphoma in children: Case

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]

The diagnosis and the staging

The excision biopsy of the supraclavicular node shows the classical Hodgkin lymphoma, nodular sclerosis subtype, with the Reed-Sternberg cells in the reactive inflammatory background and the immunohistochemistry positive for the CD15 and the CD30. The flow cytometry excludes the non-Hodgkin lymphoma. The staging is the Ann Arbor system with the Cotswold modifications: the boy is staged with the contrast CT of the chest, abdomen and pelvis and the whole-body fluorodeoxyglucose PET-CT, which shows the mediastinal mass and the supraclavicular and cervical nodes above the diaphragm, with no disease below, placing him at the Ann Arbor stage two with the B symptoms. The bulky mediastinal mass is noted as the adverse feature. [1][2]

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 the pulmonary function tests before the cardiotoxic and the pulmonary-toxic chemotherapy. The fertility preservation is discussed with the adolescent before the gonadotoxic chemotherapy, and the hepatitis and the HIV serology are sent. The bone marrow biopsy is performed for the advanced stage, and the boy is enrolled on the national EuroNet protocol. [1]

The definitive management

The definitive management is the risk-adapted combination chemotherapy, with the modern EuroNet OEPA-COPDAC regimen, reserving the involved-site radiotherapy for the slow responders and the bulky disease. The interim PET-CT after the induction guides the response-adapted de-escalation, and the complete metabolic response allows the radiotherapy to be omitted, which spares the boy the late risks of the second malignancy, the breast and the thyroid disease. The contemporary survival of the childhood Hodgkin lymphoma is above ninety-five percent in the early stage and above ninety percent overall, and the boy and the family are counselled in honest and hopeful terms. [1][2]

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 family is taught the fever and the bleeding emergencies, the neutropenic precautions, and the central line care. The survivorship surveillance is begun from the diagnosis, with the watch for the anthracycline cardiotoxicity, the endocrine late effects, the thyroid dysfunction, and the second malignancy. [1]

Contrasting the Burkitt lymphoma

The examiner asks the candidate to contrast this Hodgkin lymphoma with the rapidly growing abdominal mass of a younger child. A five-year-old with the Burkitt lymphoma would present with the ileocaecal abdominal mass, the distension and the pain over days, driven by the c-MYC translocation t(8;14) and doubling every twenty-four to forty-eight hours. The diagnosis would be the biopsy with the starry-sky histology and the flow cytometry, the staging would be the Murphy St Jude system, and the management would be the short intensive fractionated chemotherapy that exploits the rapid cycling. [3]

The decisive contrast is the tumour lysis risk. The Burkitt carries the highest tumour lysis risk of any tumour, and the prophylaxis with the hyperhydration without potassium, the rasburicase after the glucose-6-phosphate dehydrogenase screen, and the four-to-six-hourly biochemistry begins before the first chemotherapy dose. The rasburicase is contraindicated in the glucose-6-phosphate dehydrogenase deficiency because it causes the haemolysis and the methaemoglobinaemia, and the established syndrome is treated in the intensive care with the cardiac monitoring and the renal replacement therapy. The Hodgkin lymphoma, in contrast, carries a low tumour lysis risk. [10]

Communication and the family

The family is counselled honestly and hopefully. The candidate names the diagnosis, explains that it is one of the most curable cancers with a survival above ninety percent, and that the treatment runs through the induction, the consolidation and the response-adapted radiotherapy over months. The parents are introduced to the multidisciplinary team, given a written plan, taught the fever and the bleeding emergencies, and supported by the social work and the educational liaison. The adolescent is counselled on the fertility preservation and the late effects, and the siblings and the school are included. The fellow who holds the science and the humanity together in this conversation demonstrates the reasoning the boards reward. [1][2]