Neuro Neoplasms

Question 1

Diffuse glioma appearance in an adult patient:

Answer 1

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

Question 2

Astrocytoma

Answer 2

● 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.

Question 3

Oligodendroglioma

Answer 3

● 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

Question 4

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

Answer 4

Oligodendroglioma

Question 6

Treatment of Oligodendroglioma

Answer 6

● Surgical treatment is usually quite successful. Chemotherapy sometimes.

Question 7

Historically known as a Grade-4 Astrocytoma

Answer 7

Glioblastoma

Question 8

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

Answer 8

Glioblastoma

Question 9

Glioblastoma course and treatment

Answer 9

● 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%.

Question 10

Ependymoma

Answer 10

● 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

Question 11

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

Answer 11

ependymomas

Question 12

Treatment of ependymomas

Answer 12

The tumor is best treated
surgically if possible.

Question 13

Brainstem Glioma

Answer 13

● 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.

Question 14

Treatment for Brainstem Glioma

Answer 14

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

Question 15

The most common type of primary CNS
malignancy seen in children

Answer 15

Medulloblastoma

Question 15

Medulloblastoma

Answer 15

● 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

Question 15

Medulloblastoma course and treatment

Answer 15

● 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.

Question 16

Cerebellar Hemangioblastoma

Answer 16

● 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

Question 17

Cerebellar Hemangioblastoma treatment

Answer 17

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

Question 17

Acoustic Neuroma

Answer 17

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

Question 18

Ipsilateral hearing loss is the most
common initial symptom for ______

Answer 18

Acoustic Neuroma

Question 19

Presentation and management of an acoutstic neuroma

Answer 19

● 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.

Question 20

Cancer Most commonly originating from the dura mater

Answer 20

Meningioma

Question 21

Course and treatment of a meningioma

Answer 21

● 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.

Question 22

Craniopharyngioma

Answer 22

● 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)

Question 23

Craniopharyngioma treatment

Answer 23

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

Question 24

Primary Cerebral Lymphoma

Answer 24

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

Question 25

Presentation of Primary Cerebral Lymphoma

Answer 25

● 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.

Question 26

Primary Cerebral Lymphoma treatment

Answer 26

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

Question 27

Intracranial Mass Lesions S/S

Answer 27

○ 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

Question 28

Frontal Lobe Lesions

Answer 28

○ 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

Question 29

Temporal Lobe Lesions

Answer 29

○ 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.

Question 30

Parietal Lobe Lesions

Answer 30

○ 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

Question 31

Occipital Lobe Lesions

Answer 31

○ 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

Question 32

Brainstem and Cerebellar Lesions

Answer 32

○ 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

Question 33

Herniation Syndromes

Answer 33

○ 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

Question 34

Most common Herniation Syndrome

Answer 34

○ 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

Question 35

Diagnostic Approach of Intracranial Mass Lesions

Answer 35

○ 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

Question 36

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

Answer 36

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

Question 37

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

Answer 37

Corticosteroids such as Decadron (IV or PO)

Question 38

The most common sources of brain mets:

Answer 38

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

Question 39

Leptomeningeal Metastases

Answer 39

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

Question 40

Leptomeningeal Metastases treatment

Answer 40

○ 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

Question 41

Spinal Mass Lesions

Answer 41

● 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.

Question 42

Spinal Mass Lesions S/S

Answer 42

○ 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

Question 43

Spinal Mass Lesions diagnostic approach

Answer 43

○ 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.

Question 44

Spinal Mass Lesions treatment

Answer 44

○ 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

Question 45

Neurofibromatosis

Answer 45

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

Question 46

Three distinct forms of Neurofibromatosis are recognized:

Answer 46

○ 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)

Question 47

Pathophysiology of Neurofibromatosis

Answer 47

○ 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

Question 48

Signs and Symptoms of Neurofibromatosis Type 1

Answer 48

■ 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

Question 49

Signs and Symptoms of Neurofibromatosis Type 2

Answer 49

■ 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)

Question 50

Signs and Symptoms of Schwannomatosis

Answer 50

■ 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

Question 51

Neurofibromatosis 1 diagnosis

Answer 51

○ 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

Question 52

Diagnostic Consideration for Neurofibromatosis

Answer 52

○ 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