Snapshot A 4-year-old girl is brought to the emergency room after her parents felt an abdominal mass during her bath. She complains of headaches. On physical exam, her eyes jerk around erratically. Her feet also twitch occasionally. The abdominal mass is irregular, firm, and clearly crosses the midline. An MRI of her head shows potential metastasis to the skull. Introduction Malignant tumor from cells of sympathetic nervous system most commonly in adrenal medulla and paraspinal region most common adrenal tumor in kids (vs pheochromocytoma, most common in adults) Epidemiology < 4 years old Most common cancer in infancy Most common extracranial solid malignancy ETIOLOGY Pathogenesis neural crest cell origin anywhere along sympathetic chain Genetics associated with amplification of N-Myc oncogene Associated conditions Beckwith-Wiedemann syndrome sometimes (2-3%) seen with opsoclonus myoclonus ataxia syndrome with or without ataxia Presentation Symptoms abdominal distention abdominal pain hallmark of disease hypsarrythmia (dancing feet) opsoclonus (dancing eyes) Physical exam palpable, firm, and irregular mass crosses midline (vs Wilms' tumor, unilateral, smooth) may have diastolic hypertension Evaluation Biopsy histology Homer-Wright rosettes small round blue/purple cells Bombesin + stimulates neuroblastoma growth and expression of angiogenic markers electron microscopy neurosecretory granules Urine (breakdown products of dopamine) ↑ homovanillic acid ↑ vanillyl mandelic acid Imaging 131I-MIBG body scan to detect metastasis MRI Differential Diagnosis Wilms' tumor Rhabdomyosarcoma Germ cell tumors Hepatoblastoma Treatment Surgical resection With or without chemotherapy Complications Metastases to bone and skin Permanent neurological sequelae from opsoclonus myoclonus syndrome Prognosis Younger patients with better prognosis