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Neuro- CNS - Metabolic Disease > Neuro - CNS - Neoplastic Diseases > Flashcards

Neuro - CNS - Neoplastic Diseases Flashcards

1
Q

Neoplastic Diseases - General Presentation

A
  • Patient usually presents with either:
  • subacute progression of a focal neurological deficit
  • Non-focal neurological disorder (HA, dementia, gait dysfunction, etc.)
  • **Malaise, weight loss, anorexia and fever are usually suggestive of metastasis
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2
Q

Classification of Astrocytomas - Grade 1

A
  • Prognosis - variants of astrocytoma with excellent prognosis
  • Clinical Findings - varied
  • Radioogy - varied
  • Histopathology - varied
  • Examples - juvenile pilocytic astrocytoma; subependymal giant cell astrocytoma; pleomorphic xantho-astrocytoma
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3
Q

Classification of Astrocytomas - Grade II

A
  • Prognosis - can remain static or convernt into a more aggressive form of tumor
  • Clinical - presents with seizures, headaches or localizing signs; age range in 20-30 years deterioration in 93 months post diagnosis
  • Radiology - Vague, hemispheric white matter hypo-densities
  • Histopathology - Macroanatomy: grossly poorly defined area of expanded, discolored white matter, often obscuring the grey-white junctions; Microanatomy - infiltrating astrocytic “naked nuclei”
  • Examples - Fibrillary Astrocytoma
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4
Q

Classification of Astrocytomas - Grade III

A
  • Prognosis - more aggressive form of tumor
  • Clinical - presents with edema and rapid clinical deterioration, 12 months post diagnosis
  • Histopathology - a more cellular tumor with nuclear pleomorphism and mitotic figures
  • Examples - Anaplastic astrocytoma
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5
Q

Classification of Astrocytomas - Grade IV

A
  • Prognosis - most aggressive form of glial tumor, highly malignant, very poor prognosis
  • Clinical - very rapid deterioration, 5 months post prognosis
  • Radiology - Large mass with necrotic core; ring enhancing lesion
  • Histopathology - Macro: variegated gross appearance, with areas of solid tissue, necrosis and hemorrhage.
    Micro: mitotic figures, necrosis, and vascular endothelial proliferation (“glomeruloid”)
  • Examples - glioblastoma multiforme
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6
Q

Pilocytic Astrocytoma

A
  • Two major forms of astrocytes are recognized: those with narrow distributions, such as the pilocytic astrocytoma and those with diffuse distributions termed astrocytomas.
  • Grade I - is reserved for pilocytic astrocytoma.
  • It is a realtively benign, slow growing astrocytoma of children, typically seen in the cerebellum
    • AKA - spongioblastomas, juvenile astrocytoma
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7
Q

Pilocytic Astrocytoma - History

A
  • Accounts for 6% of intracranial tumors; incidence is >1 per 100,000 cases per year
  • Most frequent brain tumor of infants
  • Peak incidence is 8 to 13 years of age
  • Location:
  • cerebral hemispheres and spinal cord in older children
  • optic nerve, optic chiasm, brainstem and cerebellum in younger children
  • hypothalamus, pineal gland
  • Frequency of Distribution: (descending order)
  • cerebellum
  • optic nerve and chiasm (common in patients with NF-1)
  • cerebral hemisphere - medial aspect of temporal lobe
  • brainstem
  • Pathology
  • Bipolar processes (hair-like)
  • Rosenthal fibers: extracellular protein globules
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8
Q

Pilocytic Astrocytoma - Presentation

A
  • Slow growing, thus clinical history may precede diagnosis by months to years
  • Focal neuro signs and symptoms depend on location:
  • cerebellum > ataxia and increased ICP/hydrocephalus, seizures
  • optic system > decreasing visual acuity
  • Hypothalamic involvement > endocrine abnormalities
  • brainstem involvement > cranial nerve defects
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9
Q

Pilocytic Astrocytoma - diagnostic testing

A
  • CT imaging - enhanced
  • MR imaging
  • Stereotactic biopsy and cytology
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10
Q

Fibrillary Astrocytoma-

A
  • Two major forms of astrocytes are recognized: those with narrow distributions such as the pilocytic astrocytoma and those with diffuse distributions termed astrocytomas.
  • Astrocytomas are the most common CNS neoplasms in adults (80%)
  • They are malignant, relentlessly progressing, and arise almost exclusively in cerebral hemispheres.
    • Diffuse Astrocytomas have 3 grades:
  • Grade II Fibrillary
  • Grade III Anaplastic
  • Grade IV Glioblastoma
  • Of these grades, the Fibrillary astrocytoma is the most differentiated and slowest growing neoplasm. They also feature diffuse infiltration of surrounding structures.

AKA - well-differentiated astrocytoma, fibrillary diffuse astrocytoma, low grade astrocytoma

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11
Q

Fibrillary Astrocytoma- History

A
  • Preferential location is in the cerebral hemispheres
  • Grade II fibrillary astrocytomas account for 25% of all gliomas in the cerebral hemispheres
  • young, 20-30 years of age
  • associated with mutation of chromosome 17 > inactivation of p53 suppressor and overexpression of PDGF-A
  • survival of 5-6 years after presentation
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12
Q

Fibrillary Astrocytoma- Presentation

A
  • Seizures
  • headaches
  • Focal neurologic signs related to location of tumor
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13
Q

Fibrillary Astrocytoma- Diagnostic Testing

A
  • CT shows vague hemispheric white matter hypodensity
  • MRI
  • Angiography can be useful
  • Lumbar puncture and CSF cytology
  • Stereotactic biopsy and cytology
  • infiltrating astrocytes with “naked nuclei”
  • mitosies present, cellular
  • usually do not resect because tumor is so infiltrated
  • chemotherapy and radiation not used because side effects worse than tumor.
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14
Q

Anaplastic Astrocytoma

A
  • Diffuse astrocytomas have three grades:
    Grade II - fbrillary
    Grade III - Anaplastic
    Grade IV - Glioblastoma
  • Anplastic astrocytomas represent a form of diffuse astrocytoma characterized focal of dispersed anaplasia (reversion of cells to an immature or less differentiated form).
  • The anaplastic nature indicates a higher grade of malignancy.

AKA - AA, Malignant astrocytoma, high grade astrocytoma

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15
Q

Anaplastic Astrocytoma - History

A
  • Primary brain tumorshave an incidence of 14/100,000
  • Gliomas represent 40% of primary brain tumors
  • Anaplastic astrocytomas represent 8.3% of all CNS gliomas
  • In order of decreasing frequency, AAs appear in:
  • Frontal > parietal > temporal > Occipital lobes
  • Mutations of chromosomes 9 & 19
  • Track along white matter pathways to infiltrate adjacent hemisphere -
  • Can metastasize down spinal cord but are rare outside of CNS
  • Survival of <3 yrs
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16
Q

Anaplastic Astrocytoma - Presentation

A
  • Rapidly progressing tumor
  • Seizures
  • Headache
  • Focal neurologic signs related to the location of the tumor
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17
Q

Anaplastic Astrocytoma - Diagnostic Testing

A
  • CT imaging
  • MR imaging
  • Angiography can be useful
  • Lumbar puncture and CSF cytology
  • Stereotactic biopsy and cytology
  • uses computer imaging in at least two planes to guide tumor removal
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18
Q

Glioblastoma Multiforme

A
  • Grade IV Glioblastoma multiforme
  • Grade IV is reserved for glioblastoma multiforme.
  • It is the most aggressive of the glial tumors.
  • It may be the result of progression of a fibrillary astrocytoma or it may present de novo in the elderly.

AKA - GBM

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19
Q

Glioblastoma Multiforme - History

A
  • Median survival rate is < 1 year
  • longer survival correlates with younger age, performance status, extent of surgical resection
  • Etiology - loss of p53
  • Pathology -
  • variegated (exhibiting different colors, irregular patches) gross appearance
  • necrosis and hemorrhage
  • mitotic figures (cells undergoing mitosis (elongated dark blob)
  • glomeruloid: vascular endothelial proliferation
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20
Q

Glioblastoma Multiforme - Presentation

A
  • Focal neurological signs and symptoms depend on location
  • Cerebellum involvement > ataxia, increased ICP, seizures
  • optic system > decreasing visual acuity
  • hypothalamic involvement > endocrine abnormalities
  • brainstem involvement > cranial nerve defects
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21
Q

Glioblastoma Multiforme - Diagnostic testing

A
  • CT imaging
  • ring enhancing
  • loss of gray-white junction
  • MR imaging
  • Stereotactic biopsy and cytology
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22
Q

Oligodendroma

A
  • glial-cell tumors are highly infiltrative neoplasms believed to be derived from oligodendrocytes
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23
Q

Oligodendroma - History

A
  • comprise 5% of all intracranial neoplasms and 15-25% of gliomas in adults
  • *** More benign and better prognosis then astrocytomas
  • ***most frequently in cerebral hemispheres, frontotemporal region
  • Occur less frequently in cerebellum, brainstem, and spinal cord.
  • *** Tumors located near ventricular system can disseminate via CSF to the meninges
  • Tumors typically have sharply demarcated margins
  • become anaplastic when mitosis necrosis and nuclear atypia are more prominent
  • mixed gliomas have astrocytomas and oligodendrocytes
  • *** Inter-tumor calcification are common (40-80% of tumors), termed “brainstones”
    • Genetic alterations include centromeric translocation of chromosomes 1p and 19q
  • tumors with 1p deletion respond better to chemotherapy
  • deletion of both 1p and 10q predicts durable response to chemotherapy
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24
Q

Oligodendroma - classification and grading

A

Type: oligodendroglial tumors

(1) oligodendroma, grade: II, peak years: 30-55
(2) Anaplastic Oligodendroglioma, grade: III, age: 45-60

Type: Mixed Gliomas

(1) Oligoastrocytoma, grade: II, age: 35-45
(2) Anaplastic Oligoastrocytoma, grade: III, age: 40-50

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25
Q

Oligodendroma - Presentation

A
  • Temporal Profile
  • slowly progressing tumor
  • long interval between clinical presentation and diagnosis
  • Signs and Symptoms
  • seizures
  • headaches
  • increased ICP
  • can present initially with intracerebral hemorrhage
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26
Q

Oligodendroma - diagnostic testing

A
  • CT imaging
  • MR imaging
  • Angiography can be useful
  • Stereotactic biopsy: required for diagnosis
27
Q

Meduloblastoma

A
  • a malignant invasive embryonic tumor of the cerebellum most often seen in children and young adults
28
Q

Meduloblastoma- History

A
  • Meduloblastoma’s account for 20% of CNS tumors in children
    • Most frequent malignant brain tumors in children
  • five year survival rates are now greater than 50%
  • Etiology - Male> Female
  • Associated with chromosome 17p deletions > complete loss signals poor prognosis
29
Q

Meduloblastoma - Pathology

A
  • Exclusive occurence in the cerebellum
  • Midline presentation in children; can be more lateral in adults
  • Dissemination in CSF is common > non-communicating hydrocephalus
  • Sheets of anaplastic cells
30
Q

Meduloblastoma - Presentation

A
  • clinical signs are typically less than three months in duration
  • Common:
  • headaches (ICP)
  • persistent vomiting (ICP)
  • truncal ataxia and sometimes spasticity
  • papilledema (ICP)
  • nystagmus
  • Less Common
  • limb ataxia
  • Dysdiadokinesia
31
Q

Meduloblastoma - Diagnostic testing

A
  • CT imaging
  • MR imaging
  • Angiography canbe useful
  • Lumbar puncture and CSF cytology
  • Stereotactic biopsy and cytology
32
Q

Meningioma

A
  • Meningiomas are usually slow growing, benign tumors present on the meningeal layers surrounding the brain.
  • They arise from the meningeal coverings of the brain.
33
Q

Meningioma - Epidemiology

A
  • Account for 13-20% of all primary intracranial neoplasms
  • Most common in the 6th and 7th decade of life; rare in children
  • when in children, often assoc. with NF-2 neurofibromatosis
  • Risk factors -
  • radiation and radiation therapy
  • sex hormones, viral infections (SV-40) could play a role
34
Q

Meningioma - Etiology

A

Grading:

  • Grade 1 Meningioma (80%) - low risk of recurrence or aggressive growth
  • Grade II - atypical meningioma
  • Grade III - Anaplastic meningioma
  • Grade IV - Meningeal sarcoma
  • Progression of tumor grade type does occur and is associated with loss of chromosomal arms
35
Q

Meningioma - Pathology

A
  • Common locations
  • convexities of the cranium
  • along the falx
  • skull base - can invade skull tissue but not related to malignancy
  • usually solitary lobulated tumors attached to meninges
  • can grow en plaque: sheet-like fashion along dural surface
  • most likely develop from meningothelial (arachnoid) cells
  • typical expand and replace CNS tissue but do not invade
36
Q

Meningioma - presentation

A
  • can be asymptomatic and an incidental finding on imaging
  • **Focal neuro defects manifest due to compression of surrounding structures
  • **Seizures (most common presenting complaint)
  • ** Hemiparesis
  • Gait disturbance
37
Q

Meningioma - Diagnostic Testing

A
  • CT scan
  • MR imaging
  • Angiography
38
Q

Ependymomas

A
  • tumors arising from the ependymal cells lining the ventricular system of the brain.
39
Q

Ependymomas - Etiology

A
  • Accounts for 5-10% of the primary brain tumors in 0-20 year olds
  • ** The most common tumor of the 4th ventricle
  • Second most common site is supratentorial in the lateral ventricles
  • In adults, most common location is spinal cord
  • Frequent in setting of NF2
  • Supratentorial lesions: alterations in chromosome 9
  • Spinal cord lesions: alterations in chromosome 22
40
Q

Ependymomas - Pathology

A
  • Well differentiated tumors with relatively slow growth
  • Grade II tumors in the WHo classification
  • Recurrence rate is high at 44%
  • For fourth ventricle ependymomas, average survival post surgery - 4 years
  • Leptomeningeal spread is common
  • Secondary hydrocephalus is common due to progressive 4th ventricle obstruction
41
Q

Ependymomas - Presentation

A
  • Under the age of 3, clinical presentation involves:
  • vomiting
  • ataxia
  • headache
  • lethargy
  • increased head circumference
  • irritability
  • Older children can shows:
  • ataxia
  • nystagmus
  • gaze palsy
  • hemiparesis
  • neck pain
42
Q

Ependymomas - Diagnostic testing

A
  • CT Scan
  • MR imaging
  • Stereotactic biopsy
43
Q

Craniopharyngiomas

A
  • common childhood tumors that are derived from squamous epithelial cell rests that are remnants of Rathke’s pouch.
  • Typically these cystic tumors lie above the sella turcica (can disrupt pituitary function) and apply pressure to the optic chiasm and extends into the third ventricle.
44
Q

Craniopharyngiomas - History

A
  • Most typically seen in children, however can present at all ages
  • Account for 7-10% of all childhood tumors
  • WHO grade 1
45
Q

Craniopharyngiomas - Presentation

A
  • Headaches
  • Bitemporal hemianopsia
  • Diabetes insipidus
  • Growth retardation
  • Increased intracranial pressure
46
Q

Ganglion cell tumors (Gangliocytomas)

A
  • CNS tumor containing mature-looking neurons. When the presentation is mixed with glial-like cells, the tumor is termed a ganglioglioma
47
Q

Ganglion cell tumors (Gangliocytomas) - History

A
  • Very rare, occur usually within the first three decades of life
  • Slow growing
  • Commonly in:
  • 3rd ventricle
  • hypothalamus
  • temporal lobe
48
Q

Neurofibromatosis

A

Neurofibromatosis - a familial tumor syndrome, is in reality a complex spectrum of hereditary syndromes with neurocutaneous manifestations.

  • Two basic types have been identified, Type i (NF-1), and Type II (NF-2), each with a separate genetic origin.
    • Type 1 - characteristic of a peripheral nervous system disease
    • Type II - characteristic of a central nervous system disease
49
Q

Neurofibromatosis (NF-1 History)

A
  • Autosomal dominant
  • incidence est. 1/3000-4000
  • **NF-1 located on chromosome 17q11.2
  • **Encodes neurofibromin: appears to be a tumor suppressing, signal-transduction protein
  • Possibly part of RAS family of GTPases
  • Most NF-1 are benign, rare malignancies (<5-10%)
  • Increased risk of multiple tumors, including neurofibromas, Schwannomas, astrocytomas, and meningiomas
50
Q

Neurofibromatosis (NF - 2 History)

A
  • Much less common then NF-1
  • frequency of 1/40,000 or 50,000
    • NF-2 gene is located on chromosome 22q12
    • Encodes Merlin (or schwannomin): similar to cytoskeletal structural protein that functions as growth regulator
51
Q

Neurofibromatosis (NF-1 Presentation)

A
  • Von Recklinghausen’s disease
  • –cafe au lait spots are the most common presentation
  • dermal lesions
  • Plexiform (rope-like) lesions on spinal nerves
  • palpable, rubbery, cutaneous tumors
  • – gliomas of the optic nerve
  • – pigmented nodules of the iris = Lisch nodules
52
Q

Neurofibromatosis (NF - 2 Presentation)

A
  • ** Bilateral eighth nerve schwannomas (>90%)
  • multiple meningiomas
  • Glioas (ependymomas) of the spinal cord
  • Nodular Schwann cell growths in the spinal cord
  • Meningoangiomatosis
  • Glial hamartia
53
Q

Lymphoma

A
  • In primary CNS lymphoma, the disease initiates intracranially and metastatic extracranial spread is a late occurence in the process.
  • Primary CNS lymphoma has to be differentiated from secondary CNS lymphoma that represents metastatic spread from a non-hodgkin lymphoma located elsewhere in the body.
54
Q

Lymphoma - History

A
  • Accounts for 1% of intracranial tumors
  • Immunosuppression is a strong risk factor
  • —primary brain lymphoma (PBL) is a relatively common neoplasm in immunocompromised patients
  • secondary BL casued by metastatic Non-hodgkin’s lymphoma from elsewhere in the body.
55
Q

Lymphoma - Pathology

A
  • Single mass or multiple lesions within the brain parenchyma
  • **Ring-enhancing on radiology > must be distinguished from abcess with abx treatment
  • Majority of PBL are B cell lymphoma, large cell type
  • ** Tumors in immunocompromised patients typically contain the Epstein-Barr virus genome
  • composed on malignant lymphoid cells
  • mitotic activity
  • necrosis
  • angiocentric pattern (surround vessels)
  • PBL responds initially to radiation and steroid therapy, but tends to recur.
  • Periventricular spread is common
56
Q

Metastasis

A
  • the spread of neoplastic growth to the CNS from a distant source
57
Q

Metastasis - Etiology

A
  • Intracranial masses appear in 25% of all patients with systemic cancer, of these:
  • 15% are brain metastases
  • 8 times more common than primary brain tumors
  • 5% are leptomeningeal metastases
  • 5% are metastatic lesion to the dura
58
Q

Metastasis - Pathology

A

Primary lesion locations:
COMMON
* lung (35-64%)
- non-small cell carcinoma of the lung is most common primary site
- melanoma and small-cell carcinoma (less frequent) have greatest propensity to metastasize to brain
* breast cancers (14-18%)
* Skin melanoma (4-21%)
* renal cancer
* GI cancer
* NOTE: lung, breast, kidney and skin account for <90% of all metastatic brain tumors

UNCOMMON

  • prostate
  • pancreas
  • tuerine
  • ovarian
  • hodgkin lymphoma
59
Q

Metastasis - routes

A
  • Mainly hematogenous spread

* rarely direct spread from surrounding bone, meninges, or pituitary

60
Q

Metastasis - Location

A
  • Can occur anywhere in CNS
  • cerebral hemispheres (80%)
  • cerebellum (10-15%)
  • ** Arterial watershed zones are most common sites, specifically MCA and PCA
  • Predilection for gray-white junction
61
Q

Metastasis - temporal profile

A
  • Systemic disease usually presents initially, however CNS metastatic lesions can be initial presentation
  • CNS metastatic lesion can manifest after primary systemic disease has been eradicated
62
Q

Metastasis - Distribution

A
  • Focal space-occupying lesions

* Penumbra of edema enhances ICP increase

63
Q

Metastasis - Specific Systems

A
  • Headache (25%)
  • Hemiparesis (25%)
  • Seizures (15%)
  • Cognitive or behavioral changes (15%)
64
Q

Metastisis - Diagnostic Testing

A
  • MRI with contrast

Neuro- CNS - Metabolic Disease (2 decks)

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