CNS Tumours

Overview
Epedemiology
2nd most common tumour in children 6th most common tumour in adults Primary CNS neoplasms 8-12 per 100,000 Peaks in first and fifth/sixth decade

Classified according to cell type and degree of differentiation

Glial cells
Astrocytomas, oligodendroglioma, ependymoma, glioblastoma Medulloblastoma, neuroblastoma Meningioma Schwannoma, neurofibroma Lymphoma

Primitive neuroectodermal cells Arachnoidal cell Nerve sheath cells Lymphoreticular cells

Presentation
Local effects
Epilepsy/paralysis Space occupying lesion effects Mimic hydrocephalus Increased intracranial pressure

Mass effects

Metastasis is rare

Intrinsic Tumors
65% of adult primary CNS neoplasms ~100% of child primary CNS neoplasms Glial Cells
Astrocytomas
10% of adult primary CNS tumors
Cerebral hemispheres Cerebellum Age of patient Degree of differentiation Site and size

Primitive neuroectodermal
Meduloblastoma (commonest one in children) Fast growing Locally invasive Seeding via CSF 5 year survival 60% with Radiation therapy Uncommon Well defined cystic mass in cerebellum Composed of blood vessels/stromal cells Component of von Hippel-Lindau syndrome Uncommon Higher incidence amongst immunosuppressed patients
Aids Transplant patients

More frequent in children Prognosis

Hemangioblastoma

Glioblastoma
30% of adult tumours (rare in children)
Cerebral hemisphere white matter

Lymphoma

Most patients die within 1 year of diagnosis

3% of adult tumours (rare in children) Ill defined invading tumour arising in the white matter Arise from ependymal surface Usually fourth ventricle Projects into CSF pathway Myxopapillary ependymoma in Caudal equina

Oligodendroglioma

Ependymoma

Epstein-Barr virus implicated Poor prognosis 0% survival at 2-3 years Rare

Neuronal cells

Choroid Plexus Papilloma

Uncommon Leads to hydrocephalus May spread via CSF

Misc. Cysts

Extrinsic tumors
Coverings to brain and Spinal chord Surgical removal is often curative Meningiomas
18% of adult intracranial neoplasms 2:1 female:male Smooth, lobulated, adherent to Dura Occasionally malignant, may metastasise Invasion of brain is rare More common in adults and females Derived from Schwann cells of intracranial/intraspinal sensory nerve roots Vestibular branch of 8th Cranial nerve is the most common site Malignant change is uncommon

Schwannoma

Neurofibromas
Normally on Dorsal nerve roots Frequent among sufferers of neurofibromatosis Mixture of Schwann cells and fibroblasts

Reference
Underwood : general and systemic pathology