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  1. Most common malignant tumour of infancy (50% case occur in first 2 yrs).
  2. At birth patient may be born with metastases to placenta.
  3. Autosome dominant inheritance in familial incidence.
  4. Arie from neuroblast and sites are adrenal, sympathetic chain, retroperitoneal area, posterior mediastinum or cervical areas.
  5. Have a high frequency of spontaneous regression.
  6. Neuroblastoma is seen in patients with.
  7. Neurofibromatosis
  8. Nesidioblastosis
  9. Hirschsprung’s disease
  10. Pathologically has characteristic of Homer Wright pseudorosette.
  11. Most common presentation is asymptomatic, irregular, firm abdominal mass.
  12. May also present with Flu like syndrome: mediastinal mass or orbital metastasis with proptosis periorbital ecchymosis or bilateral black eyes (Panda eyes).
  13. Excess catecholamine or (Principally norepinephrine) causes flushing, seating nd hypocalcemia, watery diarrhea (due to VIP)
  14. Dancing eye syndrome (Opsomyoclonus and nystagmus) Paraplegia or myasthenia gravis may also present.
  15. CT, or MRI, Tumor markers, including homovanillic acid (HVA) and vanillylmandelic acid (VMA) in urine, are elevated in 95% of cases and help to confirm the diagnosis.
  16. Spinal involvement best detected by MRI.
  17. Patient may have widespread subcutaneous nodule blue in colour (Blueberry muffin baby).
  18. X-ray abdomen show stippled calcification.
  19. USG is helpful in diagnosis; CT helps in staging but standard mode of imaging is MRI.
  20. MIBG isotope scan is sensitive method to know primary and secondary.
  21. Site of metastasis : Infant – Liness
  22. In older children – Bone (M.C. Skull and diaphysis of dishal femur and humerus.)
  23. Most accurate method of biochemical diagnosis is urinary excretion of VMA and FIVA (Metabolites of catecholamine)
  24. Evan’s staging:-
    Stage I – tumor confined to single organ and can be completely resected.
    II – tumor extending beyond the organ but not crossing midline; regional lymph nodes on hemilateral side may be envolved.
    III – Tumor crossing beyond the mild line may have B/L lymph nodes.
    IV – Distant metastasis
    IV – Patients who otherwise be of stage I or II but have metastatis confined to Liver; skin or bone marrow cortical bone involvement (Prognosis is good).
  25. TOC for stage I, II and IVS is surgery.
  26. Neurone specific enolase and serum ferritin levels are important tumor and prognostic marker.
  27. In advanced disease – serum ferritin appears to be more critical prognostic factor.
  28. Thoracic and head neck tumors do better than abdominal tumor.
  29. Presence of opsoclous or nystagmus, VIP, mature history, good nutrition, normal serum ferritin and neuron specific enolase have good prognosis.
  30. In stage III and IV, chemotherapy is used with cryclophamide and Adriamycin.

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