Neuro Neoplasms Flashcards
Diffuse glioma appearance in an adult patient:
● Astrocytoma, Isocitrate dehydrogenase (IDH)-mutant (previous grades 2-4)
● Oligodendroglioma, IDH-mutant and 1p/19q co-deleted
● Glioblastoma, IDH-wild type (meaning, no mutation)
Astrocytoma
● 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.
Oligodendroglioma
● 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
Co-deletion of 1p/19q and IDH1/2- mutation are required for diagnosis of _____
Oligodendroglioma
Treatment of Oligodendroglioma
● Surgical treatment is usually quite successful. Chemotherapy sometimes.
Historically known as a Grade-4 Astrocytoma
Glioblastoma
Characterized by no IDH1/2-mutation (“IDH-wild type”)
Glioblastoma
Glioblastoma course and treatment
● 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%.
Ependymoma
● 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
Because of their position,
______ often lead to
early signs of increased
intracranial pressure
(obstructive hydrocephalus)
ependymomas
Treatment of ependymomas
The tumor is best treated
surgically if possible.
Brainstem Glioma
● 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.
Treatment for Brainstem Glioma
● Because of the location, these are
inoperable and treatment involves
shunting CSF and radiation.
The most common type of primary CNS
malignancy seen in children
Medulloblastoma
Medulloblastoma
● 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
Medulloblastoma course and treatment
● 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.
Cerebellar Hemangioblastoma
● 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
Cerebellar Hemangioblastoma treatment
Treatment is surgical (risk of lots of
blood loss) and sometimes radiation
Acoustic Neuroma
More appropriately called a Vestibular Schwannoma, these tumors are
benign neoplasms derived from the myelin of CN VIII.
Ipsilateral hearing loss is the most
common initial symptom for ______
Acoustic Neuroma
Presentation and management of an acoutstic neuroma
● 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.
Cancer Most commonly originating from the dura mater
Meningioma
Course and treatment of a meningioma
● 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.
Craniopharyngioma
● 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)
Craniopharyngioma treatment
● Treatment is generally surgical but
total removal may not be possible and
radiation may be used as well.
Primary Cerebral Lymphoma
● Primary CNS lymphoma is derived from WBCs in the brain and most
commonly occurs in immunocompromised hosts (such as AIDS, etc.).
Presentation of Primary Cerebral Lymphoma
● 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.
Primary Cerebral Lymphoma treatment
● Surgical treatment is ineffective and
corticosteroids, radiation, and chemotherapy are
the treatments of choice.
● Prognosis depends on CD4 count at diagnosis
Intracranial Mass Lesions S/S
○ 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
Frontal Lobe Lesions
○ 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
Temporal Lobe Lesions
○ 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.
Parietal Lobe Lesions
○ 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
Occipital Lobe Lesions
○ 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
Brainstem and Cerebellar Lesions
○ 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
Herniation Syndromes
○ 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
Most common Herniation Syndrome
○ 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
Diagnostic Approach of Intracranial Mass Lesions
○ 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
When is a lumbar puncture indicated for an intracranial mass lesion?
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
_____ can help reduce cerebral edema
that surrounds the tumor, and often alleviates some symptoms
Corticosteroids such as Decadron (IV or PO)
The most common sources of brain mets:
■ Carcinoma of the lung (by far, the most common)
■ Breast cancer
■ Renal Cell Carcinoma
■ Melanoma
■ Colorectal
Leptomeningeal Metastases
○ Also known as Carcinomatous Meningitis, some cancers can spread to the meninges, which is not as common but is difficult situation to treat
Leptomeningeal Metastases treatment
○ 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
Spinal Mass Lesions
● 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.
Spinal Mass Lesions S/S
○ 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
Spinal Mass Lesions diagnostic approach
○ 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.
Spinal Mass Lesions treatment
○ 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
Neurofibromatosis
● Considered a Neurocutaneous Disease, Neurofibromatosis may occur either
sporadically or on a familial basis with autosomal dominant inheritance
Three distinct forms of Neurofibromatosis are recognized:
○ 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)
Pathophysiology of Neurofibromatosis
○ 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
Signs and Symptoms of Neurofibromatosis Type 1
■ 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
Signs and Symptoms of Neurofibromatosis Type 2
■ 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)
Signs and Symptoms of Schwannomatosis
■ 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
Neurofibromatosis 1 diagnosis
○ 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
Diagnostic Consideration for Neurofibromatosis
○ 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
