Neoplastic disorders of the nervous system

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Author:
jknell
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195346
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Neoplastic disorders of the nervous system
Updated:
2013-01-25 21:51:35
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MBB II
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Neoplastic
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  1. Neoplasm - general principles
    • focal or multifocal
    • subacute or chronic
    • 4 out of 5 (80%) are metastases

    • Benign vs. Malignant
    • Adults: most benign tumors are extraparenchymal; most malignant tumors are intraparenchymal

    70%
     of childhood primary brain tumors are located in the posterior fossa

    70% of adult primary brain tumors are located in the supratentorial space
  2. Neoplasms
    presentation
    most common types
    Common presentations: personality change, headache, seizures

    Not uncommon: worsening focal deficit, asymptomatic

    Uncommon: Herniation syndrome, hydrocephalus

    Rare: stroke-like syndrome

    • 98% of brain tumors belong to one of 5 types
    • 1. Metastases - 80% (malignant)
    • 2. Gliomas - 10% (malignant)
    • 3. Meningiomas - 5%
    • 4. Pituitary adenomas - 4%
    • 5. "acoustic neuromas" - 1%
  3. Neoplasms
    Treatment
    classification
    • Treatment:
    • -Glucocorticoids - reduce vasogenic edema
    • -surgical resection
    • -radiation therapy - limitations to the amount of radiation: radiation necrosis
    • -chemotherapy - limited use due to the blood brain barrier

    • Classification: based on WHO
    • grade I to IV (higher is worse); but each grade has different name
  4. Spinal tumors
    • Incidence is 1/10th that of brain tumors
    • Presentation: spinal pain, radicular pain or deficit, myelopathy, vascular compromise of the spinal cord. Or asymptomatic

    • Location:
    • - Extradural (about 1/3 of spinal tumors, typically starting in the spine bones) - metastasis > lymphoma, myeloma
    • - Intradural but extraparenchymal (about 1/2 spinal tumors) - half are meningioma, the other half are nerve sheath tumor
    • - Intraparenchymal (about 1/6 spinal tumors) - ependymoma and low grade astrocytoma

    Tx: same as brain tumors
  5. Metastases - parenchymal
    • Median survival: 6 to 9 months (determined mostly by the primary neoplasm)

    • Primary cancer: 
    • 1. Lung cancer
    • 2. Breast cancer
    • 3. Melanoma - 75% of melanomas that metastasize go to the brain
    • 4, 5. kidney, GI (colon, rectum)

    Pathophysiology: Hematogenous >> local expansion from bone

    • Pathology:
    • -multiple or solitary; often at the gray-white junction, or at small deep penetrating vessels
    • -sharply demarcated from the surrounding brain; can be surrounded by vasogenic edema
    • -central regions of necrosis surrounded by gliosis (brain response)
    • -sometimes hemorrhagic

    • Evaluation/Tx: FIND THE PRIMARY TUMOR
    • -if it is primary, most likely malignant (most often a glioblastoma multiforme)
  6. Metastases - meningeal
    • Meningeal lesion, but NOT diffuse (like meningitis); multifocal
    • Much less common than metastasis to the parenchyma

    Presentation: multifocal meningeal lesion, most often with subacute stepwise development of one or more cranial and spinal neuropathy. Headache, back pain and delirium

    -Occurs in ~5% of pts with adenocarcinoma of the breaest, lung, and GI, melanoma, childhood leukemia, systemic lymphoma

    Pathology: Nodules of tumors in the brain, spinal cord, nerve root located within the dura

    • Evaluation/Tx:
    • -CSF: find tumor cells in the CSF (large volume
    • -Tx includes radiation therapy, and possibly intrathecal chemotherapy

    Median survival after diagnosis is about 6 months
  7. Gliomas
    glial cell tumor (can be astrocytes, oligodendrocytes, and ependymal cells)

    • Presentation: 
    • -subacute
    • -almost all are mortal (except childhood pilocytic astrocytoma, choroid plexus papilloma, and many spinal cord ependymomas)

    • Epidemiology: infiltrating astrocytomas account for about 80% of primary brain tumors in adults
    • - most often within the cerebral hemisphere
    • - ages 30-50s
    • -grade III and IV is slightly higher in men than women (1.6:1)
  8. Gliomas
    types
    • Diffuse astrocytoma (grade II) - appears as poorly defined, gray, infiltrative tumor that expands and distorts the brain

    Anaplastic astrocytoma (grade III) - regions that are more densely cellular and have greater nuclear pleomorphisms. Mitotic figures are observed

    • Glioblastoma (grade IV) - most common and  the most malignant of gliomas
    • -heterogeneous; macroscopically (can be firm and white, or soft and yellow, or degenerative, or hemorrhagic...
    • -microscopically: appears like anaplasatic astrocytoma + areas of necrosis
    • - "pseudopalisading"

    • -most glioblastomas are primary and de novo; some are secondary to increasing malignancy (low grade advanced to high)
    • -begin in the deep white matter - rapidly infiltrates
    • - can be multicentric (3 to 6%)
    • Survival: <20% survive 1 year; ~10% survive 2 years

    • Evaluation and treatment:
    • CANNOT BE REMOVED SURGICALLY - diffusely infiltrating
    • - radiosensitive, but the amount of radiation would be too high...
    • Rx: Temozolomide - chemotherapeutic drug that can treat anaplastic astrocytoma and glioblastoma (side effects: nausea, vomiting, headache, fatigue)
  9. Meningioma
    • BENIGN tumor from arachnoid cells that are typically attached to the dura
    • -chronic onset in older patients
    • -usually solitary, but can be multiple
    • - gradually indents the underlying CNS --> neurological dysfunction

    Tx: many can be removed completely

    • Complications:
    • 1. meningioma from arachnoid cells in the choroid plexus --> intraventricular meningioma
    • 2. sheet along the surface of the CNS. Meningioma "en plaque" can pick off cranial nerves

    • Epidemiology:
    • - most common benign brain tumor (and one of the two most common spinal cord tumors)
    • - Women > men (2:1 for intracranial meningiomas; 10:1 for intraspinal).
    • -50s and 60s have highest incidence
    • Risk factors:
    • -People with NF 2 have greatly increased risk for developing meningiomas
    • - radiation of scalp or cranium

    • Pathophysiology:
    • -acquired cytogenetic abnormality: loss of chrom 22, with a loss of the NF2 gene that codes for the protein merlin
    • -meningiomas often have progesterone receptors and sometimes estrogen receptors
    • meningiomas may grow more rapidly during pregnancy

    • Pathology:
    • -spherical masses with well-defined base on the dura; compresses the underlying CNS
    • -often encapsulated
    • -cells in the tumor are relatively uniform
    • -Psammoma bodies are often present (laminated calcific concentrations)

    • Evaluation/treatment:
    • neuroimaging
    • -grade I can be removed surgically
    • -high grade: surgery + radiation
  10. Pituitary adenoma
    tumor of the anterior, endocrinological part of the pituitary gland

    • Presentation:
    • - <1 cm = microadenoma
    • - <1 cm = macroadenoma
    • - secretory tumors tend to present earliest with smallest size
    • 1. Hypersecretion of pituitary trophic hormone
    • - prolactinoma >> GH, ACTH
    • -sx: amenorrhea, galactorrhea, loss of libido, infertility. Serum prolactin levels will correlate with the size of the tumor
    • - growth hormone secreting pituitary adenoma - gigantism if they are children; acromegaly is their epiphyses has fused
    • 2. Hypopituitarism - nonfunctional pituitary adenoma (25 to 30% of pituitary adenomas; compresses the adjacent normal pituitary
    • 3. Local mass effect - nonfunctional pituitary adenoma, compresses the optic chiasm
    • - less often, bony erosion of the walls of the sella (close to the cavernous sinuses)

    • Epidemiology 
    • -Adults age 35 to 60 years
    • -14% of the general population shows them on autopsy
    • -5% are familial

    • Pathology
    • - well circumscribed
    • - most often encapsulated
    • - macroadenomas are more likely to be invasive
    • - microscopically: relatively uniform, polygonal cells arranged in sheets or cords. 
    • - little supportive connective tissue

    • Evaluation/treatment:
    • -imaging
    • -Bromocriptine therapy - DA agonist (inhibits prolactin secretion)
    • -Somatostatin analogues - reduce the size of a growth hormone producing tumor
    • -Surgical excision; radiation therapy
  11. Nerve sheath tumors
    • Benign peripheral nerve tumors
    • -slow growing
    • -single sporadic tumors
    • -can occur as multiple tumors as part of either (unrelated) genetic disorders NF1 or NF2

    • 1. Schwannomas - schwann cells only
    • - compress the nerve from which it grows and adjacent structures
    • -often associated with abnormalities of the NF2 gene, always associated with loss of expression of merlin (protein product of NF2)
    • Pathology: well-circumscribed, encapsulated tumors that most commonly grow in relation to large nerve trunks (i.e. vestibular-cochlear and trigeminal nerves)
    • -two cell patterns: highly cellular group and more loosely arranged

    • 2. Neurofibromas - schwann cells together with perineural cells and/or fibroblasts
    • - symptoms by compression, but also grows within the nerve
    • -Sometimes associated with abnormalities of NF1 gene and its gene product neurofibromin

    Pathology: also in relation to large nerve trunks or in peripheral tissues sucha as skins

  12. Nerve sheath tumor
    acoustic neuroma
    • Acoustic neuroma
    • -schwannoma that arises from the vestibular portion of CNVIII

    presentation: unilateral hearing loss, vertigo, ataxia, weakness of ipsilateral muscles of facial expression, loss of somatosensation on the ipsilateral side of the face

    -Sporadic, begins in the internal acoustic meatus, grows in cerebellopontine angle

    bilateral cerebellar pontine angle mass => NF2

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