BRAIN TUMORS

So.... what is a brain tumor?

the growth of abnormal cells in the tissues of the brain, which can be benign or malignant

What is a primary brain tumor?

starts in the brain & arises from brain cells

What are some names of primary brain tumors?

gliomas, meningiomas, vestibular schwannomas, pituitary adenomas, neurofibromas, CNS lymphomas

What is a secondary brain tumor?

metastasis from other area of body, more common in adults

What are glial cells?

cells in the nervous system that support, nourish, and protect neurons

Malignant brain tumors are typically....

gliomas (glial cell tumors)

Describe benign brain tumors.

do not contain cancer cells, can be removed & usu won't grow back, clear border/edge, won't invade other tissue or spread

Describe malignant brain tumors.

contain cancer cells, grow rapidly and crowd/invade surrounding tissue, can spread to other parts of body

What is the most common presentation of a brain tumor?

progressive neurological deficit, usually motor weakness

What are some other symptoms of brain tumors?

headache, seizures, N/V, speech/vision/ hearing/gait/ personality changes

= all depends on location of tumor!

This description of a headache is only seen in minority of brain tumor patients.

predominates in AM when patient is supine & improves during the day

S/S of brain tumor in the cerebellum

lack of coordination and balance

S/S of brain tumor in the frontal lobe

changes in mood, personality, or ability to concentrate

S/S of brain tumor in the occipital lobe

vision changes

S/S of brain tumor in the parietal lobe

C/L sensory or motor deficit, memory issues, muscle jerking/twitching, seizures/convulsions, numbness & tingling

What is the most commonly diagnosed primary brain tumor?

meningioma

What are meningiomas?

extra-axial mass attached to the dura which are typically benign; F>M & not common in kids

How is a meningioma diagnosed?

symptoms and CT scan of the brain (dense & uniform enhancement)

How is a meningioma graded?

Grade I benign (majority)

Grade II atypical

Grade III malignant

How is a meningioma treated?

total resection of brain mass *curative*

grades II & III also get radiation; chemo is unproven

What if the meningioma is small & patient is asymptomatic?

can just follow radiologically as outpatient

What is the most common primary intracranial neoplasm?

astrocytoma

What is an astrocytoma?

tumor which develops from astrocytes (type of glial cell)

Graded I-IV 'glioblastoma'

What are some risk factors for an astrocytoma?

ionizing radiation, hereditary symptoms like tuberous sclerosis & neurofibromatosis

Grade 1 astrocytoma

these usually occur in children, can be cured with surgery, kids often present with hydrocephalus

ex- pilocytic astrocytoma

Grade 2 astrocytoma

low grade OR diffuse astroctyoma, usually occurs in adults

Grade 3 astrocytoma

anaplastic astrocytoma, also called malignant astroctyoma

Grade 4 astrocytoma

glioblastoma multiforme contains

dead tissue within it, malignant

How is a grade 2 astrocytoma treated?

resection, may do chemo; higher risk of recurrence & can transform to malignant (Grade 3)

How is a grade 3/4 astrocytoma treated?

difficult to treat, can do surgery for dx/control mass effect, radiation therapy prolongs survival, systemic chemo with temozolomide (temodar)

What is glioblastoma multiforme (GBM)?

ring enhancing mass with central necrosis & edema- most malignant type of astrocytoma, most are supratentorial & not often in the cerebellum

How is a GBM treated?

surgical resection → radiation therapy →

chemo therapy with temozolomide (Temodar) then

extended adjuvant temozolomide

What are some ongoing clinical trials for treading glioblastoma?

immunotherapy such as Nivolumab & CAR-T cell therapy, targeted therapies like Niraparib & PARP1/2, other technologies

What is an oligodendroglioma?

glial tumor with fried egg appearance, reticular pattern of blood vessel growth

How is an oligodendroglioma treated?

surgical resection → RT → chemo with temozolomide (temodar)

What is an ependymoma?

tumor derived from ependymal cells

that line the ventricular surface, makes up 5% of childhood tumors

Ependymomas typically arise from where?

4th ventricle wall in posterior fossa

What do ependymomas typically present with?

hydrocephalus, since tumor is obstructing the ventricle

Where do ependymomas usually occur in adults?

filum terminale of spine

What is a pituitary adenoma?

benign tumor of the pituitary gland, M=F, age 20-50

What are some symptoms of a pituitary adenoma?

hyperprolactinemia (excess prolactin), Cushing's (excess ACTH), acromegaly (excess GH), secondary hyperthyroidism (TSH)

Patients with a pituitary adenoma commonly present with _________ b/c of compression of the optic chiasm

bitemporal hemianopsia

Besides lab tests, visual fields, endocrine eval.... what is the test of choice for diagnosing a pituitary adenoma?

MRI

How is a pituitary adenoma treated?

Bromocriptine, transphenoidal adenomectomy IF patient has visual field defects

What is the most common pediatric brain malignancy?

medulloblastoma

What is a medulloblastoma?

small-cell embryonal tumor of the

cerebellum found predominantly in

children, usually arises in the cerebellar vermis in roof of the 4th ventricle

What are some s/s of a medulloblastoma?

HA, LOC, N/V (hydrocephalus symptoms), CN palsies such as swallowing difficulty, ataxia

What is the treatment of choice for a medulloblastoma?

surgical debulking; usu cannot do complete excision

What is a vestibular schwannoma (aka acoustic neuroma)?

benign tumor of the Schwann cell of CN

VIII, seen freq in pts w neurofibromatosis, M>F, 40-70 y/o

What is the clinical triad of a vestibular schwannoma?

ipsilateral (sensorineural) hearing loss, tinnitus, dysequilibrium (balance issues not vertigo!)

Work-up for all patients with vestibular schwannoma includes....

MRI with/without gadolineum, audiometrics

What is the treatment for a vestibular schwannoma?

follow symptoms via serial MRIs, radiation therapy, surgery

What is idiopathic intracranial HTN (pseudotumor cerebri)?

heterogeneous group of conditions characterized by

increased ICP with no evidence of mass, hydrocephalus,

infection or hypertensive encephalopathy

What are some risk factors of IIH?

female, obese, childbearing age

What are the s/s of IIH?

headache, papilledema, visual field deficit, nausea, diplopia, increased CSF pressure, normal-small ventricles with no mass!!

What is the only localizing sign of IIH?

CN VI palsy (abducens)

How is IIH diagnosed?

CT or MRI, LP (opening pressure >25 cm), labs, optho eval, BP

What is the treatment for IIH?

stop drugs that might be causative agent, restrict fluids & salt, diuretics (acetazolamide), steroids, weight loss, surgery only when nothing else has worked

What do the surgical options for IIH look like?

serial lumbar punctures, lumbar shunts, suboccipital/subtemporal decompression, venous sinus stenting