Embryonal tumor of the sympathetic nervous system (neural crest origin), usually arising in the adrenal medulla or paraspinal ganglia; most common extracranial solid tumor of childhood.
Often lethal if high-risk (~15% of childhood cancer deaths) yet some low-risk tumors spontaneously regress. Unique features (catecholamine secretion, paraneoplastic syndromes) and classic comparisons (e.g., vs Wilms tumor) make it a high-yield pediatric malignancy.
Infant or toddler with a firm, irregular abdominal mass (often adrenal) that can cross the midline; may have weight loss, irritability.
Frequently metastatic at presentation: bone pain or limp (bone metastases), periorbital ecchymoses ("raccoon eyes" from orbital mets), or bluish subcutaneous nodules ("blueberry muffin" skin mets).
Paraneoplastic syndromes: opsoclonus-myoclonus ("dancing eyes, dancing feet" jerky eye movements and ataxia) or intractable diarrhea (from VIP secretion).
Thoracic or cervical tumor involvement: Horner syndrome (ptosis, miosis) if sympathetic chain is affected; paraspinal masses can extend into spinal canal and cause cord compression (weakness, paralysis).
Obtain urinary catecholamine metabolites (VMA, HVA) in any young child with an unexplained abdominal mass (elevated in >90% of neuroblastomas).
Differentiate neuroblastoma vs Wilms tumor: neuroblastoma usually in infants (<2 yr) with an adrenal mass that often crosses midline and causes systemic symptoms (↑catecholamines); Wilms typically in slightly older children (~3–4 yr) with a unilateral kidney mass, often asymptomatic (±hematuria or HTN).
Once diagnosed, complete staging with imaging (MRI/CT and mIBG scan) and bone marrow biopsies; risk stratification is based on age, stage, and tumor biology (N-MYC amplification, ploidy, histology).
catecholamine-producing adrenal medulla tumor (episodic hypertension, tachycardia) but extremely rare in young children
Low-risk: surgery alone (or observation for small infant tumors) often suffices (excellent prognosis).
Intermediate-risk: chemotherapy (few cycles) plus surgical resection (≥90% survival).
High-risk: intensive multimodal therapy – induction chemotherapy, then surgical resection, followed by high-dose chemo with autologous stem-cell transplant, plus immunotherapy (anti-GD2 monoclonal antibodies) and differentiation therapy with retinoic acid.
Think VMA/HVA for NeuroBlastoma – urine catecholamine metabolites are elevated in most cases.
Opsoclonus-myoclonus (dancing eyes and feet) in a young child is a classic clue to neuroblastoma (paraneoplastic).
Unique Stage 4S: in infants <1 yr, metastatic neuroblastoma (limited to skin, liver, marrow) can spontaneously regress with minimal therapy.
Back pain, lower extremity weakness, or bowel/bladder dysfunction in a child with neuroblastoma → suspect spinal cord compression (give corticosteroids immediately and urgent neurosurgical evaluation).
Refractory hypertension or tachyarrhythmia in a neuroblastoma patient (rare catecholamine surge) may signal a catecholamine crisis – manage with appropriate alpha/beta blockade (as in pheochromocytoma).
Suspected neuroblastoma (infant with abdominal mass) → initial workup: abdominal ultrasound and urine VMA/HVA levels.
If imaging and labs suggest neuroblastoma → obtain tumor biopsy for confirmation (small round blue cells on histology; send for N-MYC testing).
After diagnosis → stage with cross-sectional imaging (plus mIBG scan for mets) and bilateral bone marrow biopsy.
Risk-stratify (age, stage, biology) and treat: low-risk = observe or surgical resection; high-risk = multi-agent chemo → surgery → stem-cell transplant + immunotherapy.
Infant with a firm abdominal mass crossing midline, weight loss, and elevated urine VMA → Neuroblastoma.
15‑month‑old with rapid eye movements (opsoclonus), myoclonic jerks, and an abdominal tumor → Neuroblastoma (opsoclonus-myoclonus syndrome).
Child with periorbital ecchymoses (raccoon eyes), bone pain, and an adrenal mass → metastatic Neuroblastoma.
Neuroblastoma histology with Homer‑Wright rosettes (H&E stain).
