The central nervous system
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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

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The Central Nervous System

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The central nervous system

The Central Nervous System


Tumors

Tumors

  • Gliomas

  • Neuronal tumors

  • Poorly differentiated neoplasms

  • Other parenchymal tumors

  • Meningiomas

  • Paraneoplastic syndromes

  • Peripheral nerve sheath tumors

  • Familial tumor syndromes


Tumors1

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 CNS

Symptoms – seizures, headaches, focal neurological deficits, hydrocephalus, ataxia


Gliomas

Gliomas

Astrocytomas

Infiltrating vs non-infiltrating (pilocytic)

Grade I – pilocytic – often cystic

Grade II – diffuse astrocytoma

Grade III – anaplastic astrocytoma

Grade IV – glioblastoma (pseudopalisading)

Oligodendrogliomas

White matter

Well-circumscribed, better prognosis

Ependymomas

NF2, ependyma-lined ventricular system

Perivascular pseudorosettes

Subepndymomas

Choroid plexus papillomas/ carcinomas

Colloid Cyst of third ventricle


Medulloblastoma

Medulloblastoma

Poorly differentiated

Well-circumscribed

Midline of the cerebellum

Desmoplastic variant

Drop metastases – cauda equina


Meningiomas

Meningiomas

Attached to the dura

Usually benign

En plaque

Psammoma bodies

Atypical

Anaplastic


Metastatic

Metastatic

¼ to ½ of intra-cranial tumors in hospitalized patients

Five most common

Lung

Breast

Melanoma

Kidney

GI


Peripheral nerve sheath tumors

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 syndromes

Familial Tumor Syndromes

Neurofibromatosis 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


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