1) malignant tumor of neural crest origin, from adrenal medulla or sympathetic ganglia 2) 33% of tumors occur in adrenals, 33% in abdomen, 20% in posterior mediastinum 3) metastasizes to regional lymph nodes, lungs, bone, liver, eye

Signs and Symptoms

1) common presenting sign is an enlarging abdominal mass with calcification 2) ascites from liver metastases 3) pain and irritability from bone metastases 4) respiratory distress from lung compression 5) gait and sphincter problems

Characteristic Test Findings

Laboratory – 1) increased urinary catecholamines – norepinephrine, VMA, HVA, dopamine 2) increased secretion of VIP

Histology/Gross Pathology

1) differentiating neuroblasts (small, round, blue cells) 2) mature ganglion cells (more mature tumors have higher proportion of ganglion cells) 3) rosettes and pseudorosettes (Homer-Wright rosettes)

Associated Conditions

1) deletions on chromosome 1 (1p35-36) 2) duplications on chromosome 2 3) affected gene is N-myc (which can be amplified, depending on differentiation

Inheritance/Epidemiology

1) mostly sporadic inheritance 2) 10% of childhood cancers 3) usually occurs before age 3 years 4) best prognosis – presents < age 2 years, extra-adrenal location, highly differentiated, VMA/HVA > 1, TRK gene expression

Treatment

1) if isolated mass, surgical resection 2) if metastatic, chemotherapy/radiation

Tips for USMLE

1) if question mentions a 2 month-old child with a large abdominal mass (with calcification on radiograph) that suddenly shrinks, think neuroblastoma 2) neuroblastomas “cross the midline,” whereas Wilms’s tumors do not

Related Posts

Retinoblastoma