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Neuro Exam 1 > Neuro Neoplasms > Flashcards

Neuro Neoplasms Flashcards

1
Q

Diffuse glioma appearance in an adult patient:

A

● Astrocytoma, Isocitrate dehydrogenase (IDH)-mutant (previous grades 2-4)
● Oligodendroglioma, IDH-mutant and 1p/19q co-deleted
● Glioblastoma, IDH-wild type (meaning, no mutation)

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2
Q

Astrocytoma

A

● Astrocytomas are gliomas that are a primary CNS malignancies originating
from Astrocytes and can appear within the brain or spinal cord
● Defined by the presence of the IDH1/2-mutation on histological analysis, but no 1p/19q co-deletion.

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3
Q

Oligodendroglioma

A

● Tumor originating from the CNS
myelin cells, Oligodendrocytes.
● These usually arise in the cerebral
hemispheres of adults and are very
slow growing malignancies, usually
over several years.
● Co-deletion of 1p/19q and IDH1/2-
mutation are required for diagnosis

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4
Q

Co-deletion of 1p/19q and IDH1/2- mutation are required for diagnosis of _____

A

Oligodendroglioma

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5
Q
A
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6
Q

Treatment of Oligodendroglioma

A

● Surgical treatment is usually quite successful. Chemotherapy sometimes.

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7
Q

Historically known as a Grade-4 Astrocytoma

A

Glioblastoma

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8
Q

Characterized by no IDH1/2-mutation (“IDH-wild type”)

A

Glioblastoma

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9
Q

Glioblastoma course and treatment

A

● Glioblastomas are rapidly growing and commonly present with nonspecific
complaints of increased intracranial pressure.
● Course is rapidly progressive with a poor prognosis.
● Total surgical removal is usually not possible, although surgery is common to decompress the brain or to stop bleeding (which is common).
● Radiation and chemotherapy (Temozolomide) may or may not prolong survival.
● The 2-year survival rate is less than 20%.

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10
Q

Ependymoma

A

● A type of glioma that arises from the ependymal cells of a ventricle,
commonly the fourth ventricle
○ These are categorized as supratentorial, posterior fossa, or spinal

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11
Q

Because of their position,
______ often lead to
early signs of increased
intracranial pressure
(obstructive hydrocephalus)

A

ependymomas

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12
Q

Treatment of ependymomas

A

The tumor is best treated
surgically if possible.

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13
Q

Brainstem Glioma

A

● A rare form of cancer, brainstem gliomas
usually present during childhood and
grow deep in the brainstem
● They present with cranial nerve palsies
and long tract signs in the limbs.

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14
Q

Treatment for Brainstem Glioma

A

● Because of the location, these are
inoperable and treatment involves
shunting CSF and radiation.

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15
Q

The most common type of primary CNS
malignancy seen in children

A

Medulloblastoma

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15
Q

Medulloblastoma

A

● The most common type of primary CNS
malignancy seen in children.
● Rapidly growing mass that usually arises from the
floor of the fourth ventricle in the posterior fossa.
● Increased intracranial pressure due to CSF
blockage is a common presentation

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15
Q

Medulloblastoma course and treatment

A

● 5-year survival generally exceeds 70%.
● Unlike most CNS tumors, Medulloblastomas often spread to other
locations within the CNS by way of the subarachnoid space/CSF.
● Treatment involves surgery, radiation, and chemotherapy.

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16
Q

Cerebellar Hemangioblastoma

A

● These low-grade tumors are derived of vascular wall tissue most
commonly in the deep cerebellum.
● Patients present often in middle-age
with disequilibrium, ataxia, and
sometimes hydrocephalus

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17
Q

Cerebellar Hemangioblastoma treatment

A

Treatment is surgical (risk of lots of
blood loss) and sometimes radiation

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17
Q

Acoustic Neuroma

A

More appropriately called a Vestibular Schwannoma, these tumors are
benign neoplasms derived from the myelin of CN VIII.

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18
Q

Ipsilateral hearing loss is the most
common initial symptom for ______

A

Acoustic Neuroma

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19
Q

Presentation and management of an acoutstic neuroma

A

● Ipsilateral hearing loss is the most common initial symptom, and other symptoms include tinnitus, headache, vertigo, facial weakness or numbness, and long tract signs.
● Treatment is usually surgical and outcome is usually good, although hearing does not recover.

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20
Q

Cancer Most commonly originating from the dura mater

A

Meningioma

21
Q

Course and treatment of a meningioma

A

● Compresses rather than invades the adjacent neural tissue.
● Signs and symptoms completely depend on the location of the mass.
● Treatment is surgical, although it can recur if the removal is incomplete.
● Increasingly more common with advancing age.

22
Q

Craniopharyngioma

A

● Derived from pituitary embryonic
tissue, these tumors can arise at any
age from remnants of the Rathke
Pouch (a fold during embryogenesis)
● It grows above the sella and can cause
endocrine dysfunction and bitemporal
field defects (optic chiasm pressure)

23
Q

Craniopharyngioma treatment

A

● Treatment is generally surgical but
total removal may not be possible and
radiation may be used as well.

24
Q

Primary Cerebral Lymphoma

A

● Primary CNS lymphoma is derived from WBCs in the brain and most
commonly occurs in immunocompromised hosts (such as AIDS, etc.).

25
Q

Presentation of Primary Cerebral Lymphoma

A

● May appear just like cerebral toxoplasmosis.
● Presentation may be with focal neurologic deficits
or with altered consciousness.
● Often appears on MRI like a corpus callosum
glioma, so biopsy is important.

26
Q

Primary Cerebral Lymphoma treatment

A

● Surgical treatment is ineffective and
corticosteroids, radiation, and chemotherapy are
the treatments of choice.
● Prognosis depends on CD4 count at diagnosis

27
Q

Intracranial Mass Lesions S/S

A

○ The clinical presentation will completely depend on the location of the mass lesion and what part of the brain is involved.
■ A focal neurologic deficit will likely occur that corresponds with the function of the anatomically adjacent brain tissue.
○ May lead to new headaches or symptoms of increased intracranial pressure, such as headaches awaking the patient from sleep, or a
headache that worsens with valsalva, cough, or recumbency.
○ Could also cause personality changes, intellectual decline, emotional lability, seizures, nausea, vomiting, and malaise

28
Q

Frontal Lobe Lesions

A

○ Often leads to progressive intellectual decline, slowing of mental activity, and personality changes.
○ Expressive aphasia may occur if the posterior part of the left inferior frontal gyrus is involved (Broca)
○ Pressure on the olfactory nerves may lead to anosmia (loss of sense of smell)
○ Ipsilateral pupillary dilation may occur secondary to compression of the
3rd cranial nerve inferior to the frontal lobes.
○ Lesions in the posterior frontal lobes at the premotor and motor cortex
usually lead to focal motor seizures or contralateral pyramidal deficits

29
Q

Temporal Lobe Lesions

A

○ Lesions in the temporal lobe can be manifested by seizures with olfactory, gustatory, and/or auditory hallucinations
○ Left-sided lesions may lead to receptive aphasia secondary to Wernicke’s Area.
○ Interestingly, right-sided lesions sometimes distort perception of musical notes and melodies.

30
Q

Parietal Lobe Lesions

A

○ Characteristically, parietal lobe
tumors cause contralateral
disturbances of sensation and may
cause sensory seizures.
○ Astereognosis is common.
○ Involvement of the optic radiations
can cause contralateral
homonymous field defect

31
Q

Occipital Lobe Lesions

A

○ Characteristically produces contralateral
homonymous hemianopia or just partial
field defects
○ Other possible manifestations can include
blindness, nonspecific visual field defects,
sudden inability to see color, inability to
identify familiar faces, visual
hallucinations, etc.
○ Anton syndrome is the denial of blindness
or field defect, which can happen

32
Q

Brainstem and Cerebellar Lesions

A

○ Tumors in the region of the brainstem or cerebellum can lead to cranial nerve palsies, ataxia, incoordination, nystagmus, and pyramidal and sensory deficits in the extremities.
○ If the fourth ventricle or foramen that drain the ventricle become occluded, obstructive hydrocephalus can occur

33
Q

Herniation Syndromes

A

○ If the pressure is increased in one cranial compartment due to the
presence of an enlarging mass, brain tissue may herniate into a cranial
compartment with less pressure

34
Q

Most common Herniation Syndrome

A

○ Most common is temporal lobe Uncal Herniation, which results in compression of the 3rd CN, midbrain, and posterior cerebral artery.
■ Earliest sign is ipsilateral pupillary dilation, followed by stupor, coma, decerebrate posturing, and respiratory arrest

35
Q

Diagnostic Approach of Intracranial Mass Lesions

A

○ CT of the head is often the initial imaging performed, depending on the initial clinical presentation.
○ MRI with and without contrast is the diagnostic test of choice
○ Full excision is generally preferred if possible, but Stereotactic Needle Biopsy is often performed when the diagnosis is unclear or
excisional surgery is not feasible

36
Q

When is a lumbar puncture indicated for an intracranial mass lesion?

A

The one exception is if an intracranial germ cell tumor (germinoma) is suspected, usually in young people, and the CSF would be evaluated for different germ cell markers

37
Q

_____ can help reduce cerebral edema
that surrounds the tumor, and often alleviates some symptoms

A

Corticosteroids such as Decadron (IV or PO)

38
Q

The most common sources of brain mets:

A

■ Carcinoma of the lung (by far, the most common)
■ Breast cancer
■ Renal Cell Carcinoma
■ Melanoma
■ Colorectal

39
Q

Leptomeningeal Metastases

A

○ Also known as Carcinomatous Meningitis, some cancers can spread to the meninges, which is not as common but is difficult situation to treat

40
Q

Leptomeningeal Metastases treatment

A

○ Surgical excision is not an option, so treatment is
generally radiation to the affected area, often
combined with intrathecal chemotherapy.
○ Long-term survival is poor- Only about 10% of patients
survive for 1 year, so palliative care is important

41
Q

Spinal Mass Lesions

A

● Primary nervous system neoplasms can occur in the spinal cord, but metastatic mass lesions are the most common, unfortunately.
● About 10% are intramedullary, with the most common form of intramedullary tumor being an Ependymoma (the rest are other types of gliomas).
● Primary extramedullary tumors include the benign neurofibromas and meningiomas, which may be
intradural or extradural.
● Prostate, breast, lung, and kidney are common primary sources for spinal metastases.

42
Q

Spinal Mass Lesions S/S

A

○ Tumors may lead to spinal cord
dysfunction by direct compression, by
ischemia secondary to arterial or venous
obstruction, or by invasive infiltration (as
with intramedullary neoplasms)
○ Symptoms generally develop slowly and
pain is common with extradural masses.
○ May have spinal tenderness.
○ Segmental lower motor neuron deficit is common at the level of the
lesion, with upper motor neuron deficits found below it

43
Q

Spinal Mass Lesions diagnostic approach

A

○ MRI of the spine with and without contrast is the diagnostic imaging modality of choice to localize and identify the lesion.
○ CT Myelogram is another option that can
highlight mass lesions in the spine
○ CSF may reveal xanthochromic color with
increased protein concentration.

44
Q

Spinal Mass Lesions treatment

A

○ Decompression of the spinal cord and excision of the mass is accomplished whenever feasible.
○ The prognosis generally depends on the state of the spinal cord and nerve roots before it was relieved.
○ Radiation is often utilized in addition to
chemotherapy, especially for metastatic lesions.
○ Dexamethasone is frequently used to treat Sxs

45
Q

Neurofibromatosis

A

● Considered a Neurocutaneous Disease, Neurofibromatosis may occur either
sporadically or on a familial basis with autosomal dominant inheritance

46
Q

Three distinct forms of Neurofibromatosis are recognized:

A

○ Neurofibromatosis Type 1 (“NF1”) - The most common
■ Characterized by hyperpigmented skin lesions and neurofibromas
○ NF2-related Schwannomatosis (“NF2”) - About 10% of cases
■ Characterized by formation of congenital vestibular schwannomas
○ Non-NF2 Schwannomatosis (“schwannomatosis”) - Very rare
■ Characterized by formation of painful schwannomas on the spinal
and peripheral nerves (but no vestibular schwannomas)

47
Q

Pathophysiology of Neurofibromatosis

A

○ The manifestation of NF1 results from mutation or deletion of the
NF1 gene, which normally serves as a tumor suppressor gene
○ The manifestations of NF2 results from a similar genetic alteration
of the NF2 (merlin) gene

48
Q

Signs and Symptoms of Neurofibromatosis Type 1

A

■ Usually appears in childhood, often by age 10 years.
■ Flat, light brown macules on the skin (cafe au lait spots)
■ Freckling in the armpits or groin area.
■ Tiny bumps on the iris of the eye.
■ Soft raised lesions on or unders the skin (neurofibromas)
■ Learning disabilities are common in patients with NF1
■ Larger than average head size
■ Short stature

49
Q

Signs and Symptoms of Neurofibromatosis Type 2

A

■ Much less common than NF1.
Neurofibromatosis NEURO-NEO-4
“Current Medical Diagnosis and Treatment,” Papadakis and McPhee.
■ Characterized by benign vestibular
schwannomas bilaterally
■ Sometimes can lead to the growth of
schwannomas on other nerves (rare)

50
Q

Signs and Symptoms of Schwannomatosis

A

■ This is a rare type of neurofibromatosis that
usually affects people after the age of 20.
■ Affects peripheral nerves but not CN VIII,
and no hearing loss occurs.
Neurofibromatosis NEURO-NEO-4
“Current Medical Diagnosis and Treatment,” Papadakis and McPhee.
■ This condition is characterized by the
extensive formation of several schwannomas
throughout the body, which are painful and
can result in paralysis and paresthesias

51
Q

Neurofibromatosis 1 diagnosis

A

○ NF1 is largely a clinical diagnosis as it is
based on skin patches and lesions
○ Genetic tests are available for all three
forms of the disease

52
Q

Diagnostic Consideration for Neurofibromatosis

A

○ If the patient has symptoms of
vestibular schwannomas, MRI of the
brain with and without contrast is
the imaging modality of choice, and
it will likely reveal bilateral tumors
in NF2.
○ Schwannomatosis can be identified
with MRI of soft tissue lesions in the
affected parts of the body

Neuro Exam 1 (6 decks)

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