Question

Discuss the pathophysiology and prognosis of neuroblastoma in children

Answer 1

Answer : Neuroblastoma is a common malignant tumour of neural crest cells, mainly affecting children below 5 years.

Site is mainly abdomen ie, adrenal medulla and paravertebral autonomic ganglia and rarely cerebral hemisphere. Cases are mostly sporadic, but some shows ie, about 1 to 2 % cases shows germline mutation of anaplastic lymphoma kinase gene and are familial.

Catecholamines and it's various metabolites like vanillyl mandelic acid and homovanillic acid are produced by Neuroblastoma and they can be observed in the 24 hour urine.

Local symptoms are abdominal distension, fever, weight loss and malaise.

Clinical manifestation include rapid local growth, metastatic spread or development of hormonal syndrome.

X ray of abdomen reveals foci of calcification.

Prognosis depends upon favourable prognostic features like children ageing less than 2 years, location of tumour in extra-abdominal sites, schwannian or ganglionic differentiation in tumour histology, tumour present in organ of origin or extending beyond origin but not crossing the midline and telomerase expression being absent, lack of amplification of N-MYC oncogene and favourable genetic features of hyperdiploidy.