tumors gliomas neuronal tumors poorly differentiated neoplasms other parenchymal tumors ...

THE CENTRAL NERVOUS SYSTEM

Tumors

Gliomas Neuronal tumors Poorly differentiated neoplasms Other parenchymal tumors Meningiomas Paraneoplastic syndromes Peripheral nerve sheath tumors Familial tumor syndromes

Tumors

20% of all childhood cancers are CNS tumors

70% of childhood tumors arise in the posterior fossa

70% of adult tumors arise in cerebral hemispheres, supratentorial

Rarely metastasize outside of the CNSSymptoms – seizures, headaches, focal

neurological deficits, hydrocephalus, ataxia

GliomasAstrocytomasInfiltrating vs non-infiltrating (pilocytic)

Grade I – pilocytic – often cysticGrade II – diffuse astrocytomaGrade III – anaplastic astrocytomaGrade IV – glioblastoma (pseudopalisading)

OligodendrogliomasWhite matterWell-circumscribed, better prognosis

EpendymomasNF2, ependyma-lined ventricular systemPerivascular pseudorosettes

SubepndymomasChoroid plexus papillomas/ carcinomasColloid Cyst of third ventricle

Medulloblastoma

Poorly differentiated

Well-circumscribed

Midline of the cerebellum

Desmoplastic variant

Drop metastases – cauda equina

Meningiomas

Attached to the dura

Usually benign

En plaque

Psammoma bodies

Atypical

Anaplastic

Metastatic

¼ to ½ of intra-cranial tumors in hospitalized patients

Five most commonLungBreastMelanomaKidneyGI

Peripheral Nerve Sheath Tumors

Schwannoma

Neural crest-derived Schwann cell

Acoustic neuroma – actually vestibular

Neurofibroma

Cutaneous

Peripheral nerve – solitary or plexiform

Malignant peripheral sheath tumors

Familial Tumor SyndromesNeurofibromatosis Type I – AD, Lisch nodules, café au lait spots

Neurofibromatosis Type II – AD, 8th nerve schannomas and multiple meningiomas

Tuberous Sclerosis – AD, hamartomas ( cortical tubers) and benign neoplasms of the brain; seizures; liver, kidney, pancreas cysts; angifibromas, shagreen patches, ash-leaf spots, subungal fibromas

Von Hippel – Lindau Disease – AD, hemangioblastomas (cerebellum and retina); cysts of liver, pancreas, kidney; renal cell carcinoma, pheochromocytomas