Neuro shelf - CNS tumors

associated with increased risk of meningiomas, astrocytomas, and sarcomas

chromosome 17

glioma (optic nerve) and ependymoma

chromosome 22q12 meningioma and glioma

chromosome 3p25

hemangioblastoma

chromosome 17p13.1

glioma and medulloblastoma

decreased appetite and weight loss, reduced school performance, dizziness, ataxia, neck pain, bulbar weakness, gaze palsy, opisthotonos

decreased peak of N-acetyl aspartate (NAA) associated with neuronal loss

gliomas and meningiomas

• astrocytoma
• oligodendroglioma
• ependymoma
• choroid plexus papilloma

arises from astrocytes. See necrosis and neovascular proliferation

tx: chemo - temozolamide and BCNU (gliadel wafers) implanted in cavity to slow delivery of the nitrosourea drug

prognosis: poor

arises from astrocytes or ependymoma cells

in kids, more common in cerebellar hemispheres, but more common in 4th decate of life

seizure typical presentation of slow growing tumors

prognosis: medial survival 7 years

arises from oligodendrocytes

peak incidence ages 35-45. common in frontal lobes, though can appear in basal ganglia and thalamus

calcifications common. microscopically "fried-egg" appearance (perinuclear halos with swollen cytoplasm)

prognosis: tends to recur locally and progress into a malignant form

arises from ependymal lining of ventricles

in kids, 90% are intracranial (often 4th ventricle) with subarachnoid spread. in adults 75% arise in spinal canal as intramedullary tumors of spine

path: perivasular pseudorosettes

arises from meningothelial (mesodermal) cells of dura mater

almost always benign, more common in women ages 40-60. NF2 may cause.

path: sheets of plump, uniform meningothelial cells with tendency to form whorls. progesterone receptors found frequently. calcifications seen on CT

hyperostosis on CT may indicate tumor invasion of the bone

arises at the medullary velum of the 4th ventricle. a primitive neuroectodermal tumors (PNET)

path: small round cells with high mitotic index

presents as fast growing tumor that infiltrates surrounding tissue and extends towards 4th ventricle, producing hydrocephalus with headache, unsteadiness, and vomiting. May spread via CSF

arises from schwann cells

more common in middle-aged women, common in vestibular cranial nerve (acoustic neuroma) at the cerebellopontine agnle. Bilateral schwannomas in NF2

path: sheets of uniform spinde cells, forming palisades called verocay bodies

seen in kids and young adults, include a mixture of neurons and glial cells. usually located in the cerebral hemisphere and have slow growth. T2 MRI shows characteristic swollen gyri. can be surgically removed

cerebellar cystic lesions, often associated with von hippel lindau disease (hereditary retinal angiomas, pancreatic cysts, and kidney tumors)

lung, skin (melanoma), kidney (renal cell carcinoma), breast, colon

located at junction of gray and white matter, tend to be solitary

intradural extramedullary spinal mets that arise from intracranial lesions. most common source is ependymomas and medulloblastomas

bilateral vestibular schwannomas, meningiomas, and intramedullary ependymomas

melanoma, renal carcinoma, and choriocarcinoma