Paediatrics

Neuroblastoma

Neuroblastoma is an embryonal tumour of sympathetic neural-crest tissue, usually adrenal or paraspinal, with behaviour ranging from spontaneous regression in selected infants to aggressive metastatic disease; a child with an abdominal mass and systemic or neurological features needs urgent specialist assessment.

In a nutshell

Neuroblastoma is a sympathetic neural-crest tumour, often adrenal or paraspinal. A child may have an abdominal mass that crosses the midline, systemic upset, bone pain, limp, pallor, periorbital bruising, Horner syndrome or opsoclonus-myoclonus. Urinary VMA/HVA, specialist cross-sectional and functional imaging, marrow assessment and MYCN biology support staging. Spinal cord compression is an emergency. Treatment ranges from specialist observation or surgery for selected low-risk disease to intensive multimodal treatment for high-risk disease.

Classic presentation

A toddler is unwell with a firm abdominal mass, pallor, weight loss, bone pain and periorbital bruising. Refer very urgently; if gait, bladder or neurological function is changing, escalate immediately for possible cord compression.

Key points

Neuroblastoma arises from sympathetic tissue, commonly adrenal or paraspinal.
An abdominal mass may cross the midline or encase vessels; the child is often systemically unwell.
Bone or marrow spread causes pain, limp, pallor and periorbital bruising.
Urinary VMA/HVA support the diagnosis; MYCN amplification is an adverse biological feature.
Paraspinal extension can cause spinal cord compression and needs immediate specialist escalation.
Stage MS disease in infants can regress or mature, so treatment is risk- and symptom-directed.

First-line investigation

Urgent clinical assessment and abdominal ultrasound alongside very urgent specialist referral; specialist teams then use cross-sectional/functional imaging and tumour biology.

Management

Refer and look for emergencies

Refer a child with an abdominal mass or unexplained enlarged organ very urgently; escalate immediately for spinal, respiratory or haemodynamic compromise.^3,2

Define site and spread

Use specialist cross-sectional and functional imaging, urinary catecholamines, marrow assessment and tumour biology to assign risk.¹

Treat by risk group

Observation or surgery may suit selected low-risk disease; high-risk disease needs protocol-led multimodal treatment and immunotherapy.¹

Monitor relapse and late effects

Follow relapse, growth, endocrine, cardiac, hearing, renal, fertility, neurocognitive and psychosocial outcomes through the specialist service.¹

Exam traps

Neuroblastoma may cross the midline; Wilms tumour is the main renal-mass differential.
Normal catecholamine metabolites do not exclude neuroblastoma.
Periorbital bruising can be metastatic neuroblastoma, not simply trauma.
Horner syndrome or opsoclonus-myoclonus may be the presenting clue.
Do not observe a suspected tumour outside a specialist risk-stratified pathway.

Key sources

This page is exam revision material, not medical advice, and must not be used for patient care. Always check drug doses against the BNF and current guidance. Full disclaimer.