POM II - CNS Neoplasms - Exam 2

Primary Tumor Lesions - Statistics

-about 25,400 malignant tumors of brain or spinal cord were dx in 2023

-about 18,760 people estimated to die from brain and spinal cord tumors

-lifetime risk of developing malignant tumor of brain or spinal cord <1%

Primary Intracranial Tumors

-due to uncontrolled division of cells in the brain (astrocytes, oligodendrocytes, ependymal cells, Schwann Cells)

-1/3 are meningiomas

-1/4 are gliomas

-Remainder: pituitary adenomas, neurofibromas, and other tumors

Microglia

-resident macrophages of the brain and spinal cord

-immunologically active

Oligodendrocytes

-source of myelin in the CNS

-support of neurons

Astrocytes

-physical support to neurons

-phagocytosis

-play a role in nourishment of the neuron

posterior fossa/infratentorial

what is the most common location of primary NS tumors in children?

anterior 2/3 of cerebral hemisphere/supratentorial

what is the most common location of primary NS tumors in adults?

M/C Childhood Primary Brain Tumors

-Medulloblastoma (malignant, 20%) Mini Medulloblastoma

-pilocytic Astrocytoma (grade I non-malignant)

-ependymoma (5% childhood tumors)

MC Adult Primary Brain Tumors

-glioblastoma (malignant)

-meningioma (most non-malignant, approx 35%)

Primary Brain Tumor - S/S

-headache, n/v, seizures, weakness, etc

-sx of increased IOP

-cranial nerve palsies

Frontal Lobe Lesions - S/S

-progressive intellectual decline

-slowing of mental activity

-personality changes

Temporal lobe Lesions - S/S

-seizures

-motor phenomena

-hallucinations

-depersonalization

Parietal Lobe Lesions - S/S

-contralateral disturbances of sensation

-seizures

-sensory loss or inattention

Occipital Lobe Lesions - S/S

-contralateral homonymous hemianopia OR a partial field defect

-loss of color perception

-prosopagnosia (inability to identify a familiar face)

Brainstem lesions - s/s

-cranial nerve palsies

-ataxia

-incoordination

-nystagmus

-pyramidal and sensory deficits

Cerebellar Lesions - s/s

-ataxia

-incoordination

-hypotonia of limbs

Primary Brain Lesions - Dx

-disturbance of cerebral function

-neuroradiologic evidence of space-occupying lesion - MRI

-biopsy

Primary Brain Lesion - Tx

-corticosteroids

-whole brain radiation therapy for tx of more than one tumor in the brain

-surgery - for solitary brain metastasis

-stereotactic radiosurgery - cyber knife

-chemo

-anti-seizure meds are given only if patient develops seizures

Primary Intracranial Tumor Types

-meningiomas

-gliomas

-pituitary adenomas

-neurofibromas

-other

Meningiomas

-common primary tumor in the CNS

-originates from dura mater or arachnoid (the meninges)

-usually, benign

-S/S: sx vary with tumor site

Meningiomas - Dx

-CT scan

-MRI: strong, homogenous enhancement with "dural tail"

Meningiomas - Tx

-Surgical: tumor may recur if removal is incomplete

-radiotherapy

Gliomas

-Benign or malignant

-about 35-50% of all gliomas in adults are glioblastomas (malignant)

-derived into: astrocytomas, oligodendrogliomas, ependymomas

Astrocytoma

-infiltrative tumor

-developed from astrocytes

-4 prognostic grades: Grade I, II, III, IV

-prognosis variable

Grade I Pilocytic Astrocytoma

-most common benign tumor in children

-rarely undergo malignant transformation

-well-demarcated

-Tx: surgery

Grade II Low Grade Astrocytoma

-infiltrative tumor

-usual presentation: seizures in young adults

-malignant transformation

-Tx: surgery, chemo and/or radiation depending upon recurrence or if unresectable

Grade III Anaplastic Astrocytoma

-median age 40-50 yrs

-Tx: surgery followed by combo radiation and chemo, or radiation then chemo

Grade IV Glioblastoma

-AKA glioblastoma multiforme

-most common malignant primary brain tumor in adults

-highly infiltrative

-median age 60-70 yrs

-tx: surgery, combo chemo + radiation

Glioblastoma Multiforme - Tx

-surgery

-radiation therapy and temozolomide

-poor prognosis

Oligodendroglioma

-arise from oligodendrocytes

-slow growing

-median age 40-50s

-Grade II (oligodendrogliomas), Grade III (anaplastic oligodendrogliomas)

-Grade II represents similar to Grade II Astrocytomas

Oligodendroglioma - Dx

-Co-deletion of 1p/19q and IDH mutation

-pathologic features: fried egg appearance

Oligodendroglioma - tx

-Surgical

-radiation and chemo (temozolomide or procarbazine)

Ependymoma

-arises from ependymal cells

-any age, most often children

-s/s: signs of increased ICP

-Tx: surgery, radiation

Neuronal and Mixed Neuronal Glial Tumors

-slow growing

-some benign, some malignant potential

-S/S: seizures, HA, dizziness and balance problems

-Tx: surgery

Brainstem Glioma

-less common

-s/s: CN palsies then long tract signs in limbs, increase ICP (late)

-Tx: inoperable, radiation and shunt for increased ICP

Medulloblastoma

-most common malignant brain tumor of childhood

-most likely dx in posterior fossa

-S/s: HA, N/V, papilledema

Medulloblastoma - Dx

-MRI of brain and whole spine

-CSF cytology

Medulloblastoma - Tx

surgery combined with radiation + chemo

Pineal Tumor

-s/s: increased ICP, sometimes associated with impaired upward gaze, hydrocephalus, lethargy

-Tx: shunting, surgery, irradiation

-prognosis: depending on histopathologic findings and extent of tumor

Pituitary Adenoma

-common cause of pituitary malfunction in adults

-types: nonfunctioning and functioning

Nonfunctioning Pituitary Adenoma

-most common

-headache

-local mass effect

-bitemporal hemianopsia

-hypopituitarism

Functioning Pituitary Adenoma

-prolactinoma: 40-60%

-cortisol secreting: 15-25%

-GH secreting: 30%

Pituitary Adenoma - Dx

-MRI

-Prolactin level

-24 hr urine free cortisol

-OGTT

Pituitary Adenoma - Tx

-Bromocriptine or Cabergoline

-Surgical resection

-pituitary hormone replacement may be required

Vestibular Schwannoma

-rare, slow growing

-arise from Schwann cells on acoustic nerve

-S/S: ipsilateral/unilateral hearing loss, tinnitus, headache, vertigo, facial weakness, numbness

Vestibular Schwannoma - Dx

-MRI

-brainstem auditory evoked potential

surgery

what is the tx for vestibular schwannoma?

Primary Cerebral Lymphoma

-associated with AIDS/immunodeficient states

-S/S: focal deficits, disturbances of cognition/consciousness

-Tx: high dose MTX + corticosteroids, radiation

-prognosis: depends on CD4 count at dx

Cerebellar Hemangioblastoma

-slow growing

-abnormal vessel growth

-S/S: disequilibrium, ataxia of trunk or limbs, signs of increased ICP

-DX: MRI

-Tx: surgery, angiogenesis inhibitors

Neurofibroma

-seen in patients with neurofibromatosis type 1

-S/S: typically mild; soft, fleshy, sessile or pedunculated flesh-colored growths found along peripheral nerves under the skin or deeper inside body

Neurofibroma - Dx

-Medical hx

-PX

-imaging

Neurofibroma - Tx

-observation

-surgery

Spinal Tumors

-1/6th as common as brain tumors

-60% are benign

-classify by histology and by location

Spinal Tumor S/S

-develop gradually

-pain: characteristically aggravated by cough/straining, radicular, localized to back or diffuse in extremity

-motor deficits

-paresthesias or numbness, commonly legs

-bladder, bowel, and/or sexual dysfunction

Spinal Tumor - PX

-localized spinal tenderness

-LMN deficit

-Dermatomal sensory changes

-UMN deficit

-sensory disturbances

Spinal Tumor - Dx

-Imaging: MRI, CT myelography

-Other studies: CSF Cytology

Spinal Tumor - CSF

-xanthochromic

-increased protein

-normal cell count

-normal glucose

Spinal Tumor - Tx

-intramedullary --> decompression, dexamethasone, surgery

-surgical excision

-radiation

Intramedullary Spinal Cord Tumor

-ependymoma most common

-remainder are gliomas

Extramedullary Spinal Cord Tumor

-can be extradural or intradural in location

-neurofibromas

-meningiomas

Extradural Spinal Tumors (outside of the spinal cord)

-metastatic tumors, usually arise in vertebral body

-hallmarks: bony destruction

-symptoms: local back pain

-ex: schwannoma, meningioma

Intradural - extramedullary spinal tumor

-sx: radiculopathy, unilateral, segmentally distributed pain

-ex: schwannoma, meningioma, mets

Intradural - intramedullary spinal tumor

-hallmark: (spinal) cord expansion

-sx: diffuse pain, myelopathy, or radiculopathy

-ex: glioma-ependymoma, astrocytoma

Brain Mets - MC sites

-lung: 42.3%

-kidney: 9.8%

-skin - melanoma: 7.4%

-breast: 5%

-GI: 1.2%

Brain Mets - S/S

-headache

-n/v

-focal weakness, speech difficulty, etc

-confusion, agitation, lethargy

-focal signs: focal neuro deficit, optic papilledema

Brain Mets - Dx

-Imaging: CT, MRI

-Other studies: visual fields, endocrine workup, audiology, arteriogram

Brain Mets - Tx

-Medical tx: steroids, anticonvulsants only if seizures

-Surgery: biopsy, craniotomy

-Radiation: brain radiation, stereotactic radiosurgery

-Chemo: systemic, intrathecal

Paraneoplastic Syndromes

-rare

-triggered by altered immune system response to neoplasm

-60% of pts may present with sx before tumor known

Paraneoplastic Syndromes - Pathophys

-antibodies or T cells directed against the tumor may mistakenly attack normal nerve cells and destroy them

-physiologically active substances released by the tumor

-cancers produce proteins that are physiologically expressed in utero by embryonic and fetal cells but not expressed by normal adult cells

-paraneoplastic anti-neural antibody detection

Paraneoplastic Syndromes - Sx

-endocrine

-neuromuscular or MSK

-CV

-cutaneous

-hematologic

-GI

-renal

Paraneoplastic Disorders

-misc - fever, anorexia, cachexia

-rheum - polymyalgia, rheumatic polyarthritis, scleroderma, SLE

-renal - SCLC

-GI - watery diarrhea, electrolyte imbalances (thyroid CA)

-Heme - anemia, thrombocytosis

-Cutaneous - itching, herpes zoster

-endocrine - Cushing's (SCLC)

-neuro/neuromuscular