NEUROBLASTOMA

NEUROBLASTOMA


                                                                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

Exam Important

  • 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.
Histology-
  • 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
 
Don’t Forget to Solve all the previous Year Question asked on NEUROBLASTOMA
Click Here to Start Quiz

Module Below Start Quiz

Leave a Reply

%d bloggers like this:
Malcare WordPress Security