NEUROBLASTOMA

• Also called as sympathicoblastoma.
• Malignant tumour of neural crest cells occurring in children (5years) – MC extracranial tumours
• Mostly sporadic
• Familial causes are known to have germline mutation of ALK gene.

Clinical features-

• MC  site- adrenal medulla
• MC site of metastasis- Lymph node > bone > bone marrow
• Dancing eye syndrome & opsomyoclonus, Racoon’s eye sign.

• Diarrhoea, Proptosis, bone involvement

Histology-

• Small round blue cell tumours
• Cells arranged in irregular sheets separated by fibrovascular stroma
• Classical feature- Homer- Wright’s rosettes (pseudorosettes)
• Immunohistochemical markers- NSE, Neurofilament (NF), chromogranin

Favourable prognostic features-

• Age – >2yrs of child
• Tumour histology with schwannian or ganglion differentiation
• Genetic features of hyperploidy
• Lack of amplification of N- MYC oncogene
• Presence of Trk- A and absence of TrK- B

Investigations-

• Radiology- foci of calcification
• Urine test- produces vanillyl mandelic acid (VMA) and homovanillic acid (HVA)
• Bone marrow biopsy positive in 60% cases.

Treatment-

• Low risk- adrenalectomy
• Intermediate risk group- Adrenalectomy + multidrug chemotherapy
• High risk group- high dose multidrug chemotherapy + surgery

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