Neuroblastoma

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Target exams

Red flags

• An abdominal mass crossing the midline in an infant or toddler, with a displaced but intact kidney, is neuroblastoma until proven otherwise, and the crossing of the midline distinguishes it from the Wilms tumour that stays unilateral
• A child with opsoclonus, myoclonus and ataxia has neuroblastoma until proven otherwise, because the opsoclonus-myoclonus-ataxia syndrome is the paraneoplastic signature of the disease
• A bulging, bruised or proptotic eye in a young child is an orbital metastasis of neuroblastoma, and the raccoon-eye periorbital ecchymosis is the classic sign
• A high-risk neuroblastoma with MYCN amplification carries a survival below fifty percent despite the most intensive treatment in paediatric oncology, and it is the diagnosis that tests the limits of cure
• An irritable infant with a firm abdominal mass, hypertension and a urine that turns dark on standing has a catecholamine-secreting neuroblastoma, and the urinary vanillylmandelic acid and homovanillic acid confirm it

Life stages

Care settings

Clinical exam formats

Board mappings

• Recognises neuroblastoma as the commonest extracranial solid tumour of childhood, arising from primordial neural crest sympathogonia that form the adrenal medulla and the sympathetic chain
• Identifies the red-flag clinical pattern of an abdominal mass crossing the midline in an infant, periorbital ecchymosis, and the opsoclonus-myoclonus-ataxia syndrome
• Names the urinary catecholamine metabolites vanillylmandelic acid and homovanillic acid as the biochemical markers that confirm over ninety percent of tumours
• Explains the International Neuroblastoma Risk Group staging system of L1, L2, M and MS, the image-defined risk factors that separate L1 from L2, and the age cut-off of eighteen months that defines the favourable MS stage
• Applies the MYCN amplification as the single most powerful adverse prognostic marker and integrates it with the stage, the age, the histology and the ploidy into the risk group
• Coordinates the risk-adapted treatment from the observation of low-risk localised disease to the induction chemotherapy, surgical resection, myeloablative consolidation with stem cell rescue, radiotherapy and anti-GD2 immunotherapy of high-risk disease
• A child with opsoclonus, myoclonus and ataxia has neuroblastoma until the urinary catecholamines and the imaging prove otherwise, because the opsoclonus-myoclonus-ataxia syndrome is the paraneoplastic signature of the disease
• The iodine-123 metaiodobenzylguanidine scan is the functional imaging that maps the disease extent in over ninety percent of neuroblastomas and that defines the metastatic stage
• The high-risk neuroblastoma with MYCN amplification is treated with the most intensive regimen in paediatric oncology, and the five-year survival remains below fifty percent despite the full sequence of induction, surgery, myeloablative consolidation, radiotherapy and anti-GD2 immunotherapy
• Examines the child with an abdominal mass systematically, distinguishing the cross-midline neuroblastoma from the unilateral Wilms tumour, and mapping the metastatic sites of the orbit, the liver, the bone and the marrow
• Counsels the family of a child newly diagnosed with neuroblastoma with honest, risk-group-specific information about the prognosis, the treatment duration and the late effects
• Synthesises the survivorship plan that watches for the late effects of the cisplatin hearing loss, the anthracycline cardiotoxicity, the growth and endocrine disturbances and the second malignancy
• Level 1: Recognition of the abdominal mass crossing the midline, the periorbital ecchymosis and the opsoclonus-myoclonus-ataxia syndrome as the red-flag presentations of neuroblastoma
• Level 2: Application of the urinary catecholamines, the iodine-123 metaiodobenzylguanidine scan, the biopsy with histology and the MYCN status to the risk stratification
• Level 3: Coordination of the risk-adapted treatment and the long-term survivorship surveillance across the low-risk, intermediate-risk and high-risk groups
• The neural crest origin of neuroblastoma and the spectrum from the immature neuroblastoma through the ganglioneuroblastoma to the mature ganglioneuroma
• The International Neuroblastoma Risk Group staging of L1, L2, M and MS, the image-defined risk factors, and the MYCN amplification as the key prognostic marker
• The opsoclonus-myoclonus-ataxia syndrome as the paraneoplastic neurological syndrome of neuroblastoma and its poor neurological outcome despite tumour cure
• Structured abdominal examination that distinguishes the cross-midline neuroblastoma from the flank Wilms tumour
• Recognition of the opsoclonus as the chaotic multidirectional eye movements that differ from the rhythmic nystagmus
• Communication of the risk-group-specific prognosis to the family, balancing the hope of the low-risk disease against the challenge of the high-risk
• Neuroblastoma as the commonest extracranial solid tumour of childhood and the commonest tumour of infancy, presenting as an abdominal mass, an orbital mass or with the opsoclonus-myoclonus-ataxia syndrome
• The diagnostic pathway from the urinary catecholamines and the iodine-123 metaiodobenzylguanidine scan to the biopsy with the histology and the MYCN status
• The risk-adapted treatment and the contrast between the spontaneous regression of stage MS and the intensive multimodal therapy of the high-risk disease
• Recognition of the red-flag features of neuroblastoma that mandate urgent referral to the paediatric oncology centre
• Application of the initial evaluation with the urinary catecholamines, the imaging and the biopsy
• Coordination of the multidisciplinary treatment pathway for the child with newly diagnosed neuroblastoma
• Canadian approach to the urgent evaluation and referral of the child with an abdominal mass, an orbital mass or the opsoclonus-myoclonus-ataxia syndrome suspected to be neuroblastoma
• The International Neuroblastoma Risk Group staging and the risk stratification with MYCN as the guide to the treatment intensity
• The risk-adapted treatment and the survivorship surveillance for the late effects in the cured child