NEURO-ONCOLOGICAL EMERGENCY

2011

Outline

• Introduction• Clinical Diagnosis• Emergency Management of Supratentorial Tumours• Emergency Management of Posterior Fossa Tumours• Pituitary Apoplexy• Spinal Emergency: Spinal Cord Compression• Postsurgical Craniospinal Emergencies

Introduction

• Neuro-oncological patients are at risk of a wide array of medical and surgical emergencies that can present with different symptoms and signs.

• These symptoms and signs may arise due to either the direct local effects of their tumours and metastasis on involved tissues or from generalised effects associated with their disease that are popularly known as paraneoplastic syndromes.

• Because such conditions may require specific emergency treatment, the recognition of these syndromes by clinicians is critical as quite often these emergencies are predictable and can be prevented or adequately managed.

Introduction

• Patient with Cancer, but not all symptoms (and etiologies) necessarily pertain to cancer

• New Symptoms Related to the Cancer– Headache– Mental Status Changes– Pain– Weakness

• Complication of Therapy– Neutropenia– Diarrhea– Vomiting

Clinical Diagnosis: Hydrocephalus

• Obstructive– Mass Lesions

• Intraventricular• Posterior fossa• Brainstem• Cerebral hemisphere• Diencephalon• Pineal region

Clinical Diagnosis : Hydrocephalus

• Treatment– Tumour debulking / excision– Ventriculoperitoneal shunt– External ventricular drainage– Ventriculo-subgaleal drainage– Endoscopic third ventriculostomy

Clinical Diagnosis : Intracranial Hypertension

• Mass lesion (Tumour and perilesional edema)• Tumoural bleed or infarction• Obstructive hydrocephalus

Clinical Diagnosis : Intracranial Hypertension

• Treatment– High dose dexamethasone– Mannitol – Hyperventilation– CSF diversion procedure– Tumour excision / debulking (decompression)

Clinical Diagnosis : New Onset Seizures

• Possible oncological etiologies: – Tumoural location, size and cortical infiltration– Tumoural bleed– Obstructive hydrocephalus

Emergency Management of Supratentorial Tumours

• The emergency management of supratentorial brain tumours is dependent on the location of the tumour, its size, and associated edema. – Immediate initiation of corticosteroids, – Temporary CSF diversion

• For those patients with focal obstruction CSF (trapped ventricles), • Tumour excision to open up CSF pathways. • For patients with primary intraventricular tumors, surgery may be easier

through dilated ventricles, and immediate surgery is undertaken without an attempt at external CSF drainage.

– Hyperventilation and/or mannitol• For control of increased ICP prior to definitive treatment in cases due to

the mass effect of tumor and associated edema. – Emergency tumour resection

• If the ICP cannot be controlled or progressive signs of neurologic deterioration occur, is required.

Emergency Management of Posterior Fossa Tumours

• Control of intracranial pressure (ICP). – Initiation of corticosteroids, usually dexamethasone,

• Initial intravenous bolus dose of 1 mg per kg followed by a maintenance dose of 1 to 2 mg/kg/day in divided doses

– For those patients with hydrocephalus, emergency CSF diversion may be required.

• This has usually been done by ventriculostomy and external ventricular drainage but currently is often handled by performing an endoscopic third ventriculostomy and leaving an external drain for pressure monitoring.

• The risk of upward herniation of the posterior fossa contents because of lowering the supratentorial pressure is a rare complication of acute CSF drainage, but is more likely with cystic tumours

Emergency Management of Posterior Fossa Tumours

• Emergency tumour resection– Especially in children with cystic tumours and suppressed

consciousness or pressure waves (episodic severe headache, bradycardia, intermittent visual loss with the headache), steroids are unlikely to have much effect on the local raised pressure

– CSF drainage and monitoring is continued after surgery until it is clear that CSF flow is adequate.

– With the current treatment, <50% of patients will require permanent CSF diversion procedure.

– Following surgery, there is often postoperative edema and corticosteroids are maintained for a few days and then carefully tapered.

Pituitary Apoplexy

• Rare yet potentially fatal disease, clinically characterized by the abrupt onset of severe headache accompanied by neurological and or endocrine deterioration.

• Rapid intervention may halt and even reverse neurological deficits and a life-threatening situation.

• It occurs secondary to sudden expansion of a mass within the sella turcica, usually as a result of hemorrhage and/or infarction.

Precipitants of apoplexy

(1) Reduced blood flow in the gland,

(2) An acute increase in blood flow in the pituitary gland,

(3) Overstimulation of the pituitary gland,

(4) The anticoagulated state.

Pituitary Apoplexy: Clinical features

• Headache (95% of cases) – Sudden and frequently retro-orbital in location. – Accompanied by vomiting,

• with the mechanism attributed to meningeal irritation and/or increased intracranial pressure

• Visual deficits (64%) and the ocular paresis (78%)– Proximity of the pituitary gland to the visual apparatus and ocular

motility cranial nerves (i.e., the cavernous sinus)– Visual field deficit is a bitemporal superior quadrantanopsia.– The oculomotor nerve is involved most commonly, which results in

a unilateral dilated pupil, ptosis, and an inferiorly and laterally deviated globe.

• Decreased mental status – Secondary to hydrocephalus or hyponatremia (Addisonian crisis).

Pituitary Apoplexy: Radiographic evaluation

• Cranial CT may show a hemorrhagic mass in the region of the sella turcica; however,

• MRI is the imaging modality of choice, because it clearly demonstrates the features of hemorrhage and infarction, the suprasellar extension, compression of the chiasm, and extension into the cavernous sinuses.

• Cerebral angiography is sometimes needed to differentiate pituitary apoplexy from aneurysmal SAH.

Pituitary Apoplexy: Treatment

• Immediate initiation of steroid replacement is paramount in the treatment of patients presenting with pituitary apoplexy. – 100 milligrams of hydrocortisone should be administered IV every 8

h.

• Definitive treatment for pituitary apoplexy is surgery – For decompression, especially in patients with decreases in visual

acuity or visual field deficits, a decreased level-of-consciousness, or progressive deterioration of visual or oculomotor abilities.

– The trans-sphenoidal surgical route is adequate in most instances. – The visual outcome is related to the duration of impairment, severity

of the initial visual defect, the appearance of the optic disc, and early decompression.

Spinal Emergency: Spinal Cord Compression

• Causes of spinal cord dysfunction in patients with cancer:– Epidural compression

• Tumour• Abscess• Hematoma• Disc herniation• Vertebral hemangioma

– Intramedullary processes• Metastases• Abscess• Hematoma• Syrinx

– Myelopathy• Radiation• Intrathecal chemotherapy• Paraneoplastic

– Leptomeningeal metastases– Spinal arachnoiditis

Pathophysiology of Spinal Cord Compression

• Hematogenous spread.• Usually in the vertebral body.• Compression of cord microvasculature by bone or tumors.

Circulatory disturbance.

Secondary change (cord edema, ischemia, or infarction).

Neurologic signs.

Clinical Presentation of Cord Compression

Tumor type Occurrence(%)

Breast 20.6Lung 17Lymphoma 9Prostate 7Sarcoma 6.6Myeloma 6Kidney 5.6Others 27

Malignant Epidural Spinal Cord Compression (ESCC)

• Neoplastic invasion of the space between vertebrae and spinal cord (epidural invasion)– Usually from bone metastases

• Compresses thecal sac of spinal cord• Frequent complication of malignancy• Can cause pain• Can cause irreversible loss of neurologic function

Malignant Epidural Spinal Cord Compression (ESCC)

• Causes– Metastatic tumor from any primary site– Tumors with predilection to metastasize to spinal column– Prostate, breast, and lung carcinoma

• 15-20% of cases

– Renal cell, non-Hodgkin’s lymphoma, or myeloma• 5-10% of cases

Vertebral metastases

• More common than Epidural Spinal Cord Compression

– Prostate cancer 90%– Breast Cancer 74%– Lung Cancer 45%– Lymphoma 29%– Renal cell 29%– GI 25%

Spinal Cord Compression: Clinical Presentation

Thoracic 70%Lumbosacral 20%Cervical 10%

One vertebral body 46%

Several contiguous 26%

Multiple nonconstiguous 28%

•Local pain(90%),constant, dull, aching, and progressive.•Weakness(80%), neurologic signs.

Spinal Cord Compression: Clinical Features

• Important to recognize• Early recognition leads to better outcomes• Efficacy of treatment depends most on patient’s neurological

function at presentation• Median time from symptoms to diagnosis is around 2

months• More than half of patients who present to hospital are non-

ambulatory

RED FLAGS

• First Red Flag: Pain– Usually first symptom

• 80-90% of the time

– Usually precedes other neurologic symptoms by seven weeks

• Increases in intensity

– Severe local back pain– Aggravated by recumbency

• Distension of venous plexus

– May become radicular

RED FLAGS

• Second Red Flag: Motor – Weakness: 60-85%13

– At or above conus medularis• Extensors of the upper extremities

– Above the thoracic spine• Weakness from corticospinal dysfunction

• Affects flexors in the lower extremities

– Patients may be hyperreflexic below the lesion and have extensor plantars

– Weakness tends to be symmetrical– Progressive weakness is followed by lost of gait function then

paralysis– The severity of weakness is greatest with thoracic metastases

RED FLAGS

• Third Red Flag: Sensory– Less common than motor findings– Still present in majority of cases– Ascending numbness and parathesias

RED FLAGS

• Fourth Red Flag: Bladder and Bowel Function – Loss is late finding– Autonomic neuropathy presents usually as urinary

retention• Rarely sole finding

Intramedullary Metastases

• Less common• Often present with hemicord symptoms

– Unilateral weakness below lesion– Contralateral diminution of pain and temperature

sensation– Can progress to bilateral dysfunction

Clinical pathway for patients with metastatic spinal cord compression

Spinal Cord Compresion: Evaluation

• Physical Examination!• MRI: The new gold standard

– Approx 95% sensitivity/44-93% specificity• Myelogram: The old gold standard• Plain X-Ray• Bone scan may help find other locations of disease

Treatment of Spinal Cord Compression

• Initial treatment: steroid plus radiation. And consulted neurosurgery, neurology.

• Indication of surgical intervention.– Unknown diagnosis.– Spinal instability.– Failure of radiation therapy.

• Aggressive surgery– New data shows that all patients should be considered for decompressive

radical resection (Malignant spinal cord compression)

• Chemotherapy: chemosensitive tumour.• Steroid

– IV dexamethasone 10mg q6h for 2 days, followed 4mg oral q6h. – Improve peaked at day 2 and diminished by day 4 and tapered every 4

days.

Emergency Management of Spinal Cord Tumours

• Outcome especially extramedullary tumors, is highly dependent on the degree of neurologic compromise present at the time of intervention.

• Those patients who are ambulatory and have good neurologic function at the time of diagnosis and emergency treatment tend to maintain function.

• For those patients with significant motor deficits at diagnosis, time is of essence, as intervention within the first 24 hours of neurologic compromise is associated with better outcome and regaining of neurologic function.

• The time frame outlined is predominantly based on the adult experience with metastatic lesions and children may have neurologic recovery even when deficits are present for >24 hours.

Emergency Management of Spinal Cord Tumours

• Steps in the emergency management:– The use of corticosteroids

• usually dexamethasone, at an intravenous bolus of 2 mg per kg, up to 50 to 100 mg, followed by the use of dexamethasone at 1 to 2 mg/kg/day in divided doses.

– Emergency spinal decompression • is usually indicated for patients with extradural masses,

especially in patients with possible metastatic disease without a known primary.

• In certain circumstances, as in children with known primary disease with neuroblastoma and lymphoma and minimal to moderate neurologic dysfunction, treatment with emergency radiation or chemotherapy can be used instead of emergency surgical decompression and tumor removal.

Sub-optimal acute care of metastatic spinal cord compression

Other Neuro-Oncologic Emergencies

• Neutropenic Fever• Metabolic/Electrolyte Changes

– Tumor Lysis Syndrome– Hypercalcemia

• SVC Syndrome• Chemotherapy Complications

Postsurgical Craniospinal Emergencies

• Cranial Emergencies– Haemorrhages– Acute oedema – hydrocephalus, – infections– infarct

Postsurgical Craniospinal Emergencies

• Spinal Emergencies– Haemorrhages

• epidural haematomas, especially following decompressive laminectomies in patients with vertebral neoplasia

– Acute oedema – CSF fistulae with associated pseudo-meningocele– Infections

• Epidural enhancement may be observed associated with mass effect and, if severe, spinal cord compression

– Malpositioning of vertebral screws, • Direct injury to the neural structures adjacent to the surgical site

as well as to regional vascular structures such as the vertebral arteries

Thank You