Doctor123.org

Tumor Compression Syndromes, Emergency Medicine

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.

- 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

- Spinal cord compression: - History of malignancy - Back or neck pain: - Prolonged - Worse with rest - Most commonly affects the thoracic spine

- 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

- 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

- 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)

- 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.

  • 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 yearOccurs in 5 " 14% of cancer patientsMore 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 plexusRecurrent laryngeal nerve compression by mediastinal lymph nodes
  • Superior vena cava (SVC) syndrome:Obstruction of returning blood flow in the SVC by compression, infiltration, or thrombosisVenous hypertension within the area ordinarily drained by the SVCIn severe cases, gradual elevation of the intracranial pressure (ICP), with altered mental status and coma60 " 85% caused by malignancy

Etiology

  • Spinal cord compression:Prostate cancerBreast cancerLung cancerRenal cell carcinomaMultiple myelomaMelanomaThyroid cancerLymphomaSarcoma
  • Brachial plexus compression:0.4% of cancers2 " 5% of those who receive radiation treatmentLung cancerBreast 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 malignancyBack or neck pain:ProlongedWorse with restMost commonly affects the thoracic spineParesthesiasDifficulty ambulatingConstipationUrinary retentionUrinary or fecal incontinenceWeight 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:OrthopneaDyspneaTightness of the shirt collarCoughChest painHeadacheFacial swellingHead fullnessBlurred visionDizzinessSyncope

Physical Exam

  • Spinal cord compression:Loss of rectal toneLoss of anal winkWeakness in 60 " 85% of patientsSensory findings less common
  • Laryngeal nerve compression:HoarsenessVocal cord paralysis
  • Brachial plexus:Ulnar paresthesiasWeakness and wasting of intrinsic hand musclesPan-plexopathyHorners syndrome
  • SVC syndrome:Periorbital edemaConjunctival suffusionFacial swellingFacial plethoraUpper extremity edemaFindings exacerbated by recumbent or stooped-over positionUsually worse in the early morning hoursICP may be elevated in severe cases:Altered mental statusComaPapilledema

Diagnosis Tests & Interpretation

Imaging

  • Chest radiograph:Spinal cord compression:May identify a primary lung tumorHelpful in excluding tuberculous spondylitisSVC compression:Mass present in 10%Pleural effusion in 25%Plain spinal radiographyWill show 85% of metastases causing compressionA 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 syndromeMay identify mass and impingement in vena cava obstruction
  • MRI:Study of choice for spinal cord compressionIndicated in patients with back or neck pain and:History of cancerBowel or bladder dysfunctionLower extremity weaknessSensory lossSaddle 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:BronchoscopyMediastinoscopyScalene node biopsyLimited thoracotomyVideo-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:GoiterPericardial constrictionPrimary thrombosisIdiopathic sclerosing aortitisTuberculous mediastinitisFibrosing mediastinitisHistoplasmosisIndwelling 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 modalityPain medication with narcoticsOncology, radiotherapy, and neurosurgical consultation for further management of tumor/malignancyConsider 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)