Basics
Description
- Complications arising from the compression of neural or vascular structures by solid tumors or their direct infiltration of such structures
- Spinal cord compression:
- Affects over 20,000 patients each year
- Occurs in 5 " 14% of cancer patients
- More than 50% of cases are metastases from lung, breast, or prostate cancer.
- Vertebral metastases are far more common than epidural spinal cord compression (ESCC).
- Approximately 20% of cases of ESCC represent the initial manifestation of malignancy.
- Other neurologic tumor compression:
- Brachial plexus
- Recurrent laryngeal nerve compression by mediastinal lymph nodes
- Superior vena cava (SVC) syndrome:
- Obstruction of returning blood flow in the SVC by compression, infiltration, or thrombosis
- Venous hypertension within the area ordinarily drained by the SVC
- In severe cases, gradual elevation of the intracranial pressure (ICP), with altered mental status and coma
- 60 " 85% caused by malignancy
Etiology
- Spinal cord compression:
- Prostate cancer
- Breast cancer
- Lung cancer
- Renal cell carcinoma
- Multiple myeloma
- Melanoma
- Thyroid cancer
- Lymphoma
- Sarcoma
- Brachial plexus compression:
- 0.4% of cancers
- 2 " 5% of those who receive radiation treatment
- Lung cancer
- Breast cancer
- SVC syndrome from tumor compression:
- Lung cancer (most common):
- Small cell lung cancer primarily
- Postirradiation fibrosis
- Lymphoma
- Breast cancer
- Testicular cancer
- See "Differential Diagnosis " for non malignant etiologies of the SVC syndrome.
In children with spinal cord compression, common causes are sarcoma, neuroblastoma, germ cell tumors, and lymphoma.
Diagnosis
Signs and Symptoms
History
- Spinal cord compression:
- History of malignancy
- Back or neck pain:
- Prolonged
- Worse with rest
- Most commonly affects the thoracic spine
- Paresthesias
- Difficulty ambulating
- Constipation
- Urinary retention
- Urinary or fecal incontinence
- Weight loss
- Brachial plexus compression:
- Neuropathic pain involving the medial aspect of the upper extremity
- Intrathoracic vagal nerve compression:
- Ipsilateral aching facial pain around the ear
- SVC syndrome:
- Orthopnea
- Dyspnea
- Tightness of the shirt collar
- Cough
- Chest pain
- Headache
- Facial swelling
- Head fullness
- Blurred vision
- Dizziness
- Syncope
Physical Exam
- Spinal cord compression:
- Loss of rectal tone
- Loss of anal wink
- Weakness in 60 " 85% of patients
- Sensory findings less common
- Laryngeal nerve compression:
- Hoarseness
- Vocal cord paralysis
- Brachial plexus:
- Ulnar paresthesias
- Weakness and wasting of intrinsic hand muscles
- Pan-plexopathy
- Horners syndrome
- SVC syndrome:
- Periorbital edema
- Conjunctival suffusion
- Facial swelling
- Facial plethora
- Upper extremity edema
- Findings exacerbated by recumbent or stooped-over position
- Usually worse in the early morning hours
- ICP may be elevated in severe cases:
- Altered mental status
- Coma
- Papilledema
Diagnosis Tests & Interpretation
Imaging
- Chest radiograph:
- Spinal cord compression:
- May identify a primary lung tumor
- Helpful in excluding tuberculous spondylitis
- SVC compression:
- Mass present in 10%
- Pleural effusion in 25%
- Plain spinal radiography
- Will show 85% of metastases causing compression
- A normal spine (or 1 showing just degenerative changes) on plain radiology does not exclude the diagnosis of possible cord compression.
- CT:
- Contrast CT is more sensitive and specific than plain radiography and radionucleotide imaging in distinguishing benign from malignant disease in spinal compression syndrome
- May identify mass and impingement in vena cava obstruction
- MRI:
- Study of choice for spinal cord compression
- Indicated in patients with back or neck pain and:
- History of cancer
- Bowel or bladder dysfunction
- Lower extremity weakness
- Sensory loss
- Saddle anesthesia
Diagnostic Procedures/Surgery
- CT myelography:
- Indicated for spinal cord compression when MRI is unavailable or contraindicated (pacemaker, metallic implants, severe claustrophobia)
- Minimally invasive techniques can often be used to establish a tissue diagnosis in cases of SVC syndrome.
- Occasionally an invasive procedure is required to obtain a tumor biopsy in patients with SVC syndrome:
- Bronchoscopy
- Mediastinoscopy
- Scalene node biopsy
- Limited thoracotomy
- Video-assisted thoracic surgery (VATS)
- Radiation therapy (RT) can be done to shrink the tumor:
- Should be done after tissue diagnosis is made, as RT can obscure tissue and make definitive diagnosis difficult.
- Endovascular stents can be used to achieve more rapid relief than can be achieved using RT.
Differential Diagnosis
Spinal Cord Compression
- Amyotrophic lateral sclerosis
- Arteriovenous malformations
- Epidural abscess
- Intervertebral disk disease
- Multiple sclerosis
- Neurologic diseases
- Osteoporotic vertebral fractures
- Primary bone tumors
- Spinal infarction
- Spondylitis
- Spondylosis
- Transverse myelitis
Superior Vena Cava Syndrome
- Pericardial tamponade
- Nephrotic syndrome
- Cor pulmonale
- Cirrhosis
- Nonmalignant etiologies of SVC syndrome:
- Goiter
- Pericardial constriction
- Primary thrombosis
- Idiopathic sclerosing aortitis
- Tuberculous mediastinitis
- Fibrosing mediastinitis
- Histoplasmosis
- Indwelling central venous catheters
Treatment
Initial Stabilization/Therapy
- Early diagnosis and treatment are the keys to an improved outcome.
- Level of neurologic dysfunction on presentation is a key factor in the prognosis for spinal cord compression.
- Avoid IV line placement in upper extremities if severe SVC compression is present.
Ed Treatment/Procedures
Spinal Cord Compression
- Corticosteroids (dexamethasone):
- Administer in ED.
- Higher doses alleviate the pain more rapidly, but studies indicate no significant difference in outcome with regard to sphincter function or ambulation between the dose schedules.
- Radiotherapy:
- Definitive treatment modality
- Pain medication with narcotics
- Oncology, radiotherapy, and neurosurgical consultation for further management of tumor/malignancy
- Consider empiric broad-spectrum antibiotics prior to the MRI if an epidural abscess is being considered.
- Urgent neurosurgical consultation
SVC Compression
- Manage the underlying malignancy with either radiotherapy or chemotherapy.
- Elevation of the head of the bed.
- Supplemental oxygen
- Administer steroids if there is respiratory compromise
- Judicious use of diuretics may transiently improve symptoms, but there is poor evidence to support efficacy.
- Urgent oncology referral
- Intravascular stents can relieve the obstruction more rapidly.
Medication
- For ESCC there is limited evidence suggesting steroids are beneficial, but it is still generally considered to be part of the standard regimen of treatment
- For paresis or paraplegia high dose dexamethasone: 1 mg/kg loading dose, then halve the dose every 3 days
- For patients with minimal neurologic dysfunction dexamethasone 10 mg followed by 16 mg daily initially in divided doses with a gradual taper once definitive treatment is underway
- For SVC syndrome steroids can reverse symptoms from steroid responsive malignancies such as lymphoma or thymoma.
- In patients undergoing RT steroids are often prescribed to prevent swelling
- Furosemide (Lasix): No prior use " 40 mg IVP; prior use " double 24 hr dose (80 " 180 mg IV)
- Hydrocodone/acetaminophen: 5/500 mg PO q4 " 6h
- Oxycodone/acetaminophen: 5/500 mg PO q4 " 6h
Follow-Up
Disposition
Admission Criteria
- Admission is advisable for all patients presenting with a tumor compression syndrome.
- Transfer to a center with neurosurgical capabilities may be needed for patients with spinal cord compression.
Discharge Criteria
None
Issues for Referral
- Radiation oncology should be consulted for patients presenting with tumor compression.
- Early neurosurgical consultation for patients with spinal cord compression
Pearls and Pitfalls
- Average life expectancy among patients who present with malignancy-associated SVC syndrome is ’ Ό6 mo.
- Presentations may be subtle and compression syndromes should always be considered in patients with known malignancy and unexplained complaints.
Additional Reading
- Cole JS, Patchell RA. Metastatic epidural spinal cord compression. Lancet Neurol. 2008;7(5):459 " 466.
- Graham PH, Capp A, Delaney G, et al. A pilot randomized comparison of dexamethasone 96 mg vs 16 mg per day for malignant spinal-cord compression treated by radiotherapy: TROG 01.05 Superdex study. Clin Oncol (R Coll Radiol). 2006;18:70 " 76.
- Lanciego C, Pangua C, Chac ³n JI, et al. Endovascular stenting as the first step in the overall management of malignant superior vena cava syndrome. AJR Am J Roentgenol. 2009;193(2):549 " 558.
- Loblaw DA, Mitera G, Ford M, et al. A 2011 updated systematic review and clinical practice guideline for the management of malignant extradural spinal cord compression. Int J Radiat Oncol Biol Phys. 2012;84(2):312 " 317.
- Wilson LD, Detterbeck FC, Yahalom J. Clinical practice. Superior vena cava syndrome with malignant causes. N Engl J Med. 2007;356:1862 " 1869.
Codes
ICD9
- 239.9 Neoplasm of unspecified nature, site unspecified
- 336.9 Unspecified disease of spinal cord
- 459.2 Compression of vein
ICD10
- D49.9 Neoplasm of unspecified behavior of unspecified site
- G95.29 Other cord compression
- I87.1 Compression of vein
SNOMED
- 413284005 Partial obstructing tumor (finding)
- 71286001 Spinal cord compression (disorder)
- 63363004 Superior vena cava syndrome (disorder)
- 193118002 Nerve root and plexus compressions in neoplastic disease (disorder)