CNS Neuroblastoma

Neuroblastoma

Embryonal malignant tumour Neural crest origin Neoplastic neuroblasts Site: adrenal medulla &sympathetic ganglia 7-10% of solid paediatric malignancies. Sporadic occurance. Rarely familial (bilateral,multifocal)

Pathology of neuroblastoma

Site :Paravertebral, Posterior mediastinum, abdomen ,Adrenal 1/3 Gross appearance: Nodular, of varying size May be encapsulated or infiltrative Cut section: grey-tan, soft and friable Varigated,necrosis,hemorrhage, calcificaton,cystic change

Clinical features

Abdominal mass, fever Blueberry muffin Wide metastasis Secrete catecholamines Vanillylmandelic acid (VMA)/Homovanillic acid (HVA) screening.

Prognosis

Stage spread to regional lymph nodes,liver,lungs,bones etcAge :< 1 yr.Morphology –gangliocytic differentiation better MYCN (N myc) gene amplification-worse