- Derived from the primordial neural crest cells migrated from the developing spinal cord to the sympathetic ganglia and adrenal medulla
- Two mutation theory: 1st in the germ cell, 2nd in the somatic cell
- Third most common childhood malignancy (after leukemia and brain tumors)(most common extracranial solid tumor in children)
- Younger age than rhabdomyosarcoma and Ewing's sarcoma (90% diagnosed by age 5)
- M:F = 1.25:1
- Original tumor found in the para-midline area from the base of the skull to pelvis (and adrenal and organ of Zuckerkandl) including dorsal root ganglia (63% retroperitoneal, 16% mediastinal, 3% sacral, 2% cervical)
- Rarely familial (AD with variable penetrance)
- Chronically ill, fever, weight loss, GI symptoms, anemia, joint pain
- 1/5 have hypertension, "blueberry muffin baby", "myoclonus-opsoclonus" syndrome
- 80-90% have elevated urinary catecholamines/metabolites (nor- and epinephrine/VMA, HVA, MHPG)
- "Opsoclonus-hypoclonus-ataxia syndrome )paraneoplastic syndrome)
- Metastases commonly occur in bones, esp. skull, femur, and humerus (may be symmetrical)
- White to yellowish soft, hemorrhagic lesions
- Shimado histopathology
- Chromosomal abnormalities in 80% (deletion/rearrangements of short arm of chromosome 1)
- E rosette inhibition due to serum ferritin synthesized by the tumor coating T lymphocytes
- Sheets of small lymphocyte-like cells.
- Rosettes can be found only rarely in metastases
- Taillike eosinophilic cell extension is virtually diagnostic
- EM: neurosecretory granules (except in undifferentiated tumors)
- If taken out of context can be mistaken for Ewing's sarcoma or lymphoma
- VMA/HVA ratio ?1.5 with ? prognosis
- ? prognosis with presence of serum neuron specific enolase
- ? prognosis with MYCN oncogene amplification
- 5 year survival is 15% (? prognosis if <1 year of age)
- XRT + chemotherapy
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