Central nervous system tumor  

Anatomy

- Brain

- Spinal cord

 

Brain

Developing brain

 

Spnial cord

Histology

- neuron

- glial cells

Neuronal cell

definition?

- transmit nerve signals to and from the brain

- consist of cell body (or soma) with branching dendrites (signal receivers) and a projection of called an axon, which conduct the nerve signal

Types

- sensory neurons or bipolar neurons

- interneurons or pseudopolar (spelling) cells

- motoneurons or multipolar neurons

 

Glial cells

definition

- nerve cells that do not carry nerve impulses

- perform many functions, including digestion of parts of dead neurons, manufacturing myelin for neurons, providing physical and nutritional support for neurons, and more

Types

- astrocyte

- microglia

- Schwann's cells

- oligodendroglia

- satellite cells

Cerebral perfusion pressure

CPP = mABP - ICP

normal: 60-80 mmHg

Increased ICP

Symptoms

- headache

- nausea

- vomiting

- increased BP

- decreased about time, and then location and people as the pressure worse

- double vision

- pupil that do not respond to changes in light

- shallow breathing

- seizure

- loss of consciousness

- coma

 

Causes

- too mach cerebrospinal fluid

- bleeding into the brain

- swelling of the brain

- aneurysm

- blood pooling in some part of the brain

- brain or head injury

- brain tunor

- infections such as encephalitis or meningitis

- hydrocephalus

- high BP

- stroke

Symptoms

Intracranial tumors

- supratentorial tumor

- infratentorial tumor

brain injury from

- mass effect

- dysfunction or destruction of adjacent neural structures

- swelling

- abnormal electrical activity (seizure)

Supratentorial tumor

- focal neurologic deficit

* contralateral limb weakness

- visual field deficit

- headache

- seizure

Infratentorial tumor

- hydrocephalus (from compression 4th ventricle)

* headache

* nausea

* vomiting

* diplopia

- cerebellar hemisphere or brain stem dysfunction

* ataxia

* nystagmus

* CN palsies

 

Intracranial tumor

- glial tumor

- neural tumor and mixed tumor

- primary tumor

- secondary tumor: metastatic tumors

* most: lung, breast, kidney, GI tract, melanoma

Glial tumor

- astrocytoma

- oligodendroglioma

- ependymoma

- choroid plexus papilloma

Astrocytoma

- most common primary CNS neoplasm

- Grade I-IV

- infiltrate adjacent brain except Juvenile pilocytic astrocytomas, Pleomorphic xanthoastrocytomas (low grade, well circumscribe, good prognosis)

- Histologic features: hypercellularity, nuclear atypia, endovascular hyperplasia, necrosis (specific for GBM)

Treatment

 

Oligodendroglioma

- often presents with seizures

- calcification and hemorrhage on CT or MRI suggest diagnosis

- Grade I-IV

Treatment

 

Ependymoma

- pediatric: supratentorial ependymoma (lateral and 3rd ventricle)

- adult: infratentorial ependymoma (4th ventricle)

- most common symptoms: increased ICP (headache, nausea, vomiting, vertigo)

- spread through CSF: drop mets in spinal canal

Treatment

 

Choroid plexus papilloma

- infants: supratentorial ependymoma (lateral ventricle)

- adult: infratentorial ependymoma (4th ventricle)

- well circumscribed and vividly enhance

- most common symptoms: increased ICP (headache, nausea, vomiting, vertigo)

Treatment

Neural tumor and mixed tumor

- medulloblastoma

- Ganglioglioma

Medulloblastoma

- most common types: primitive neuroectodermal tumor

- first decade of life or around age 30 yr

* most common malignant pediatric brain tumor

- location: usually midline, cellebellum

- most common symptoms: increased ICP (headache, nausea, vomiting, vertigo)

* from obstructive hydrocephalus

- Histologic features: densely packed small, round cells with large nuclei and scant cytoplasm

- generally not encapsulated

- frequently disseminate within CNS

- CT scan: usally hyperdense and cysts formation or necrosis

* older patients: calcification

Treatment

 

Ganglioglioma

- mixed tumor: Neuron + glial cells

- first three decades of life

- often in medial temporal lobe

- most common symptoms:temporal lobe epilepsy

Treatment

Embryologic tumor

- craniopharyngioma

- epidermoid

- dermoid

- teratoma

Craniopharyngioma

- benign cystic tumor

- most frequently in children and elderly (age 50 yr)

* calcification: all pediatri and half of adult

- symptoms: compression of adjacent structures

* optic chiasm: bitemporal hemianophia

* pituitary or hypothalamic dysfunction: endocrine hypofunction

* 3rd ventricle: hydrocephalus

Treatment

 

Epidermoid

- cystic lesions with stratified squamous epithelial walls from trapped ectodermal cell rests

- Grow slowly and linearly by desquamous into cyst cavity

- cyst contain keratin, cholesterol, cellular debris

- most common location: cerebellopontine angle

- complication:

* Mollaret's meningitis (release of irritative cyst contents into subarachnoid space)

Treatment

 

Dermoid

- less common

- contain midhair follicles, sebaceous glands

- location: anywhere along the craniospinal axis (more commonly line structures)

- associated with anomalies, trauma (eg. lumbar puncture)

- symptoms: mass effect

* compression of adjacent structures (eg. optic chiasm rupture)

* leakage of sebum into subarachnoid space (aseptic chemical meningitis)

* variable: headache, seizure, vasospasm, death

Treatment

 

Teratoma

- Germ cell tumors

- location: midline, often in pineal region

- contain all 3 embryonal layers: ectoderm, mesoderm, endoderm

* more primitive structure: more malignant

!!! prognosis: very poor in malignant

* more differentiated tissue: more benign

Treatment

Miscellaneous tumors

- meningiomas

- vestibular schwannoma

- pituitary adenoma

- hemagioblastoma

- lymphoma

Meningioma

- derived from arachnoid cap cells of arachnoid mater

- rise from dura mater: dural-based tumors

- slow growing, encapsulated, benign tumor

- aggressive atypical or malignant: invade adjacent bone or cerebral cortex

- most common location: along falx, convexities, sphenoid wings

- !!!previous cranial irradiation increases incidence of meningiomas

- after treatment: may develop recurrences from

* surgical bed

* distant de novo tumors

Treatment

 

Vestibular Schwannoma (Acoustic neuroma)

- arise from superior half of vestibular portion of vestibulocochlear nerve (CN VIII)

- symptoms: tinnitus, progressive hearing loss, balance difficulty, brain stem compression (large tumor), obstructive hydrocephalus (large tumor)

*** bilateral acoustic neuromas are pathognomonic for neurofibromatosis type 2 (NF2)

Treatment

 

Pituitary adenoma

- arise from anterior pituitary gland (adenohypophysis)

- Types by diameter

1. < 1 cm: microadenoma

2. > 1 cm: macroadenoma

- Types by function

1. functional: secrete endocrinologically active compounds at pathologic levels

( Cushing's disease-ACTH, Forbes-Albright syndrome-prolactin, Acromegaly-GH)

2. nonfunctional (diagnosis as large lesion that results to mass effect)

(compress optic chiasm-visual field deficits, compress pituitary gland-panhypopituitarism

- Pituitary apoplexy: hemorrhagic pituitary adenoma

*abrupt symptoms: headache, visual disturbance, decreased mental status, endocrine dysfunction

Treatment

 

Hemagioblastoma

- cystic tumor with an enhancing tumor on cyst wall (Mural nodule), abundant thin-walled vascular channels

- almost in posterior fossa

- 20% with von Hippel-Lindau disease (VHL)

* VHL: multisystem neoplasm disorder (other tumor associated with VHL are renal cell carcinoma, pheochromocytoma, and retinal angiomas)

Treatment

 

CNS Lymphoma

- primary: in CNS

- secondary: from systemic disease, growing transplant and AIDS populations

- symptoms: mental status change, headache (increased ICP), CN palsy-from lymphomatous meningitis

- CT scan: hyperdense, enhancing with contrast

- confirm diagnosis by Stereotactic needle biopsy specimen

Treatment

Spinal tumors

- Extradural spinal tumor: metastatic tumor, primary tumor

- Intradural spinal tumor

* Extramedullary tumor: meningioma, Schwannoma, Neurofibroma

* Intramedullary tumor: ependymoma, astrocytoma

Symptoms: tumor affect to

- stability: vertebral column, posterior ligamentous complex

- neural compression: myelopathy, radiculopathy

Extradural tumors

Metastatic tumor

- most common extradural tumor

- primary tumors from lymphoma, lung, breast, prostate

- most: thoracic and lumbar vertebral bodies

- symptoms: aching pain in bones, radicular pain (awaking pain), pain with movement (vertebral fracture), neurological (weakness, tingling or numbness in arms or legs)

Treatment

 

Primary tumor

- Hemangioma: benign vascular tumor in vertebral body (thoracolumbar)

mid-life, Women > men

often asymptomatic

* symptoms: large hemangioma resulting to destabilize then fracture, hemorrhage resulting pain and neurodeficit

- Aneurysm bone cysts (ABC)

nonneoplastic osteolytic lesion

expansile, contain thin-walled blood cavities

children and adolescent

common locations: lamina or spinous process in cervicothoracic spine

* symptoms: pain swelling, fracture

Treatment

Intradural tumors

- Extramedullary tumor: meningioma, Schwannoma, Neurofibroma

- Intramedullary tumor: ependymoma, astrocytoma

- arise from meninges or nerve root elements

- typically brnign, slow growing and well circumscribed

- symptoms: myelopathy (compress spinal cord), radiculopathy (compress nerve roots)

Intradura-Extramedullary tumor:

- Meningioma

- Schwannoma

- Neurofibroma

Meningioma

- origin: arachnoid mater

- 5th-7th decade female, increased incidence in pt with NF2

- most common: posterolaterally of thoracic spine

- symptoms: cord compression, progressive myelopathy

- MRI: dural tail sign

Treatment

 

Schwannoma

- origin: peripheral nerve sheath schwann cells

- benign, encepsulated, rarely undergo malignancy

- symptoms: radiculopathy, often presenting as pain myelopathy

- multiple schwannoma likely have NF2

Treatment

 

Neurofibroma

- benign, not encapsulated, fusiform

- similarly to schwannoma (difficult to differentiate on imaging)

- salvage of the parent nerve to improve the likelihood of total resection (thoracic and high cervical nerve root maybe with minimal deficits)

- multiple neurofibromas likely have NF1 (Cafe au lait spots, neurofibromas, bone defects, scoliosis, Lisch nodules, optic nerve or optic glioma)

Treatment

Intradural-Intramedullary tumors

- Ependymoma

- Astrocytoma

- arise from within parenchyma of spinal cord

- symptoms: local dysthesia, burning pain, radicular pain, sensory loss, weakness, sphincter dysfunction

 

Ependymoma

- most common intramedullary tumor in adult

- may contain cystic areas and hemorrhage

- well circumscribed

- central location in spinal cord

- more common symptoms: scoliosis and bony remodelling

Treatment

 

Astrocytoma

- most common intramedullary tumor in children, male > female

- more often in cervical cord, but may occur at all levels

- faster growing, worse prognosis

- nonencapsulated, infiltrative nature

- may interfere with CSF-cantaining central canal of spinal cord leading to dilated central canal (Syringomyelia)

- symptoms:

* common: pain, weaknessm sensory changes

* uncommon: bowel and bladder dysfunction

Treatment

Investigation

- MRI with and without gadolinium: all patient with symptoms

- metastatic tumor: CT scan chest + abdomen + pelvis and bone scan

Management

symptomatic patient inial mange with

- dexamethasone: reduce of vasogenic edema

- phenyltoin: if seizure

Treatment

Astrocytoma

- Gross total resection

!!!impossible: lesion from motor cortex, language centers, deep or midline structures or brain stem location

- radiotherapy: all grades (after total resection)

- chemotherapy (Temozolomide): typically reserved for GBM

 

Oligodendroglioma

- aggressive resection

- chemotherapy: procarbazine, lomustine (CCNU), vincristine (PCV), temozolomide

 

Ependymoma

- maximal resection

- postoperative radiotherapy: improve survival rate

 

Chorid plexus papilloma

- total surgical excision (curative)

 

Medulloblastoma

- surgical resection with radiotherpy with chemotherapy

 

Ganglioglioma

- complete surgical resection

 

Craniopharyngioma

- pimarily surgical resection

!!! complication: (basal frontal injury): visual loss, pituitary dysfunction, diabetes insipidus, cognitive impairment

 

Epidermoid

- surgical drainage and removal of cyst wall

!!!complication: severe chemical meningitis (intraoperative spillage of cyst contents)

 

Dermoid

- symptomatic patient: surgical resection

 

Teratoma

- surgical excision may be attempted

 

Meningioma

- total resection (curative)

- if small and asymptomatic

* follow up umtil symptomatic or until significant growth (document or serial imagings)

- if atypical and malignant

* surgery and postoperative radiation

 

Vestibular Schwannoma (Acoustic neuroma)

- microsurgical resection or stereotactic radiotherapy (gamma knife or linear accelerator technology)

!!!complication: damage to facial nerve (CN VII)

risk of facial nerve dysfunction increases with increasing tumor diameter

 

Pituitary adenoma

- symptomatic decompressed surgically via transphenoidal approach, endoscopic surgical techniques (through nose, minimally invasive)

except

- if prolactin-secreting tumor: usually shrink with Dopaminergic therapy alone

* consider surgery if persist mass effect or endocrinologic dysfunction in spite of adequate dopamine agonist therapy

 

Hemagioblastoma

- surgical resection (curative for sporadic (non-VHL associated) tumors): En bloc resection of the mural nodule

 

CNS Lymphoma

- steroid then whole brain radiation then chemotherapy (intrathecal methotrexate (option)

do not surgery

Spinal tumors

Metastatic tumors

- Goal: preserving spinal stability, reducing pain and maintaining neurological function

- nonsurgical treatment (palliative, non-curative)

* opioids, NSAIDS: reducing pain

* Dexamethasone: 10 mg IV for reducing edema, 100 mg IV for treatment

* Bisphosphonate: reducing osteolytic lesion

* radiotherapy: for radiosensitize tumor (lymphoma, multiple myeloma)

- surgical treatment

indication: failure of radiation therapy, spinal instability, recurrence after radiation therapy, for diagnosis in cases of unknown primary tumots, life expectancy of more than 6 months

* minimally invasive surgery: vertebral fracture treatments (vertebroplasty, kyphoplasty), transarterial chemoembolization

*open surgery: spinal stabilization surgery, spinal decompression surgery (corpectomy, laminectomy, costotransversectomy

 

Hemangioma

- if symptomatic: surgery, radiotherapy

 

Aneurysm bone cysts (ABC)

- spinal stabilization

- early surgery

 

Meningioma

- surgical excision

 

Schwannoma

- indication: symptomatoc lesions should be surgically resected

- preserving nerve root

 

Ependymoma

- surgical removal

- post-operative radiation therapy after subtotal resection

 

Astrocytoma

- surgical resection (almost always histologically incomplete because of infiltratuve nature)