Hospital Medicine

Spinal cord tumors and compression

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I. What every physician needs to know.

Diagnosis of spinal cord compression is usually urgent given the dense concentration of motor and sensory pathways in a small area. Delay in diagnosis can lead to lifelong immobility, bladder dysfunction and decreased survival irrespective of the underlying pathological diagnosis. Early recognition may reverse neurological deficits and because therapy is usually well tolerated in ambulatory patients, the results can be gratifying even in those with very limited overall prognosis. One in five patients with cancer can present for the first time with cord compression.

Spinal cord compression due to trauma, epidural abscess, hematomas, etc. will not be discussed here.

II. Diagnostic Confirmation: Are you sure your patient has spinal cord tumor and compression?

Reliable, consistent elicitation of a clear horizontal level on the trunk below which sensory, motor and autonomic dysfunction is impaired is characteristic of spinal cord involvement and quickly helps to exclude peripheral neuropathy or muscle diseases as possible causes for patient's symptoms. The temporal course of symptoms is one of relentless progressive quadriplegia or paraplegia. On the other hand, if you are convinced about history of improvement in symptoms, one should cast a doubt on the diagnosis of cord compression.

A. History Part I: Pattern Recognition:

In spinal epidural cord compression (ESCC) 90% of patients have back pain which is persistently gnawing and worse on coughing, sneezing or movement. Recumbency commonly exacerbates the pain and sitting up relieves it in contrast to the pain of degenerative spondylotic myelopathy which is relieved with rest.

Cervical cord lesions may have pain radiating down the arms. Patients with thoracic cord lesions may complain about "girdle like tightness" around the trunk. Ask about leg stiffness or heaviness. They may trip over minor obstacles, so called "tripping over match sticks". Coming down the stairs or stepping off a curb will reveal ankle clonus felt as "vibrations" by patient. Sudden involuntary jerking movements of upper or lower extremities is called flexor spasms which are a feature of upper motor neuron (UMN) lesion.

Sensory symptoms of tingling and numbness in the local dermatome are common and ascending numbness with paresthesias is frequent. Occasinally extradural cervical cord lesions may be associated with "Lhermittes sign" which is a sudden shooting electric shock-like sensation traveling down the spine and arms when the neck is flexed.

Bladder and bowel dysfunction with urinary retention, incontinence, constipation can occur at any level of cord involvement. In those with suspected cauda equina syndrome, absence of post-voidal residual urine virtually excludes it (99.99% negative predictive value).

B. History Part 2: Prevalence:

Patients with recent onset weakness and sensory symptoms in extremities or the trunk with or without bladder/bowel dysfunction should raise suspicions. Recent diagnosis of breast, prostate, lung cancer, multiple myeloma, or ongoing treatment for such malignancies strengthens the clinical settings. Patients with multiple neurofibromatosis are at high risk for spinal cord neurofibromas, acoustic schwannomas and ependymomas.

Most common epidural tumors are secondary metastases, and the top three primary sites are prostate, breast and lung. Renal cell carcinoma, non-Hodgkin's lymphoma and myeloma make up the rest. Most common intradural extramedullary tumors are meningiomas, schwannomas and neurofibromas. Most common intra-medullary tumors are ependymomas.

C. History Part 3: Competing diagnoses that can mimic spinal cord tumors and compression.

Differential diagnosis includes degenerative cervical spondylotic myelopathy, spinal epidural abscess/hematoma and a group of non- compressive myelopathies including acute tranverse myelopathy, examples include:

  • Vascular: Acute spinal cord infarction

  • Infectious: Preceding acute viral illness

  • Multiple Sclerosis (MS)

  • Idiopathic transverse myelitis.

In the setting of severe hypotension, dissecting aortic aneurysm, rapidly progressive quadriplegia, or paraplegia with sharp midline back pain suggests anterior spinal artery syndrome. Acute spinal MS is a relatively painless febrile illness.

Radiation myelopathy may occur if the spinal cord is included in the treatment ports. It is a chronic progressive condition occurring 1-2 years after radiotherapy.

D. Physical Examination Findings.

Broad patterns of physical exam findings include:

At the level of compression, patients may have Lower Motor Neuron (LMN) findings of muscle atrophy, fasciculations, loss of tone and reflexes. Below that level, Upper Motor Neuron (UMN) findings are those of spasticity, hyperreflexia with upgoing Plantar reflex also called Babinski's sign With Extradural lesion (i.e. spinal metastasis), early symptoms are motor and sensory in an asymmetric onset. In contrast, intramedullary tumors, Cauda equina, conus medullaris lesion, bladder and bowel symptoms occur earlier. In localizing the segmental level of spinal cord, location of back pain, root pain and atrophic muscle groups is of greater value than the level of hypoalgesia. In patients with severe spasticity, ankle clonus is prominent.

High cervical cord lesions may produce quadriparesis with diaphragmatic weakness. C5-C6 lesions may produce bicep weakness and loss of bicep reflex. C7 lesion produces triceps and wrist extension weakness. Horner's syndrome can occur at any level. Thoracic lesions usually have a sensory level on the trunk. Remember, nipples are at T4 and umbilicus is T10. Lesion at T9-T10 will spare upper abdominal muscles and when abdominal muscles contract, umbilicus will move up (Beevor's sign).

Two other rare patterns include the Brown-Sequard hemicord syndrome (same side motor sensory findings and opposite side loss of pain and temperature) and central cord syndrome which in the cervical cord will produce more arm over leg weakness, with dissociated sensory loss.Carefully elicited sensory level on the trunk, severe spasticity, ankle clonus and Babinski's sign with bladder involvement are all classic findings.

E. What diagnostic tests should be performed?

N/A

1. What blood test do we need to order?

Routine complete blood count and chemistry panel and coagulation screen may not offer much in the way of diagnostic clues but will be needed if surgery becomes an option. Anemia, markedly raised ESR, mild hypercalcemia with renal failure may suggest myeloma.

2. What imaging studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?

Magnetic resonance imaging (MRI) with gadolinium of the clinically suspected level is the diagnostic imaging of choice. Once cord compression is suspected, imaging should not be delayed and radiologist may need to be called for a wet reading. In chronic kidney disease/acute renal failure/hepatorenal syndrome, call radiologist regarding the risk of gadolinium. Spinal segmental level is important for focused imaging, but in patients with known epidural metastatic disease finding unsuspected disease at other levels is not unusual. So in this setting, MRI of the entire spine is recommended. Pain control and mild sedation in anxious/claustrophobic patient might provide good quality imaging.

If MRI is contraindicated, CT-myelography is the next best investigation. Rarely, lumbar puncture can cause an abrupt deterioration in cord compression. So if CT-myelography is considered, consult neurosurgery.

F. Over-utilized or “wasted” diagnostic tests associated with this diagnosis.

If MRI shows a lesion and it correlates with your clinical suspicion, electromyography (EMG) or lumbar puncture may not add vital information for further management. Of note, these tests should not delay MRI acquisition.

III. Default Management.

Basic principles of management include:

  • Pain control

  • Preserve or improve neurological deficit

  • Avoid further complications

  • Discuss long-term goals, quality of life issues, etc.

Currently available treatment options include: surgery, external beam radiotherapy, stereotactic body radiotherapy and in the case of chemosensitive tumors, systemic chemotherapy.

A. Immediate management.

In patients with moderate to severe pain, opioid patient controlled anesthesia (PCA) is a good starting point. In those with paresthesias or radicular neuropathic pain, anti-convulsants may be used in parallel with opiates, but start at lower doses to avoid excess sedation.

Intravenous steroids (dexamethasone 16-60mg in divided doses) should be given immediately on suspicion of cord compression. Do not wait until MRI confirmation. If MRI reveals retropropulsion of bone fragments onto spinal cord or in known patients with multiple vertebral body metastases and new neurological deficit, one should worry about spine instability and consult ortho-spine/neurosurgery service immediately. Consider a spinal brace, especially in those with intractable pain.

Once imaging confirms a cord compression, neurosurgery, radiation oncology or medical oncology consultants may need to be involved depending on the underlying diagnostic possibilities and radiological findings. In extradural spinal cord compression radiotherapy will serve the dual purpose of stabilizing epidural involvement or thecal sac indentation and also control pain. Coordinate with surgeons and start heparin or low-molecular weight heparin (LMWH) DVT prophylaxis and if it needs to be avoided, mechanical prophylaxis is the standard of care. For bedridden patients, skin care precautions are needed to prevent decubitus ulcers.

B. Physical Examination Tips to Guide Management.

Once specific and appropriate therapy has been instituted, examine for improving neurological status or bladder function though this may take weeks in most cases and is largely dependent on pre-surgical functional status. Frequent timely communication with surgical team is essential in recognizing early post-operative complications of wound dehiscence, cerebrospinal fluid (CSF) leak, infection and intraabdominal vascular or visceral injury. Elderly are more susceptible.

D. Long-term management.

Formetastatic epidural disease,cure is usually beyond expectation and palliation is the main goal. Radiotherapy and surgical decompression are the keystones of management. In discussions with the patient and family it is important to make it clear that each of these options individually or in combination will be chosen depends on tumor activity, rate of progression and degree of neurological deficit, radiosensitivity, etc.

Generally speaking, radiotherapy is chosen if:

  1. There is no neurological deficit

  2. Pain is the main symptom

  3. Tumor is radiosensitive like lymphoma or myeloma

Surgical decompression is chosen if:

  1. There is concern for spinal instability or spinal cord by bone compression

  2. Neurological deficits fail to improve or worsen after radiotherapy

  3. Known radioresistance of tumor

  4. Concerns of current radiotherapy might exceed the spinal cord's tolerance in patients who received radiation therapy earlier

  5. When tumor recurs

  6. When diagnosis is in doubt

When patient is terminally ill with metastatic disease, pain control with radiation therapy or spinal morphine pump will improve quality of life. Transfer to hospice or arranging home hospice care may be needed.

In patients withintramedullary tumors, surgical resection is the main stay. Once surgically resected, the following general guidelines will help with patient and family discussions:

  1. Generally speaking, patients with severe neurological deficits before surgery are unlikely to recover significant neurological function post-operatively.

  2. In the first postoperative year, motor function improves more and over a longer period of time than sensory function.

  3. The postoperative quality of life depends on preoperative neurological status.

  4. In the immediate postoperative period the neurological deficits may be slightly worse before it gradually improves.

  5. In the recovery room immediately after surgery, paresthesias and hyperesthesia may be troublesome.

E. Common Pitfalls and Side-Effects of Management

Watch for steroid induced tremulousness, insomnia, delirium, especially in elderly/those with dementia and hyperglycemia or worsening diabetes control. Fluid retention due to steroids may become an issue in patients with chronic kidney disease or congestive heart failure. Look for oropharyngeal candidiasis and treat with nystatin or fluconazole if necessary. Treat constipation aggressively.

Too much sedation or inadequate pain control may interfere with ability to maintain fluid balance/nutrition or cooperate with physical therapy staff.

IV. Management with Co-Morbidities

N/A

A. Renal Insufficiency.

As above.

B. Liver Insufficiency.

As above.

C. Systolic and Diastolic Heart Failure

As above.

D. Coronary Artery Disease or Peripheral Vascular Disease

If patient is on aspirin or clopidogrel, the cardiology team may need to be consulted in addition and coordinated with surgical teams about timing of withholding, if at all, and safely reinitiating after surgery to avoid risk of post-operative epidural hematoma.

E. Diabetes or other Endocrine issues

As above.

F. Malignancy

As above.

G. Immunosuppression (HIV, chronic steroids, etc).

No change from standard management.

H. Primary Lung Disease (COPD, Asthma, ILD)

No change from standard management.

I. Gastrointestinal or Nutrition Issues

No change from standard management.

J. Hematologic or Coagulation Issues

If anticoagulation need to be reinitiated, it should be done based on discussions with surgical team. Deep Venous Thrombosis prophylaxis may be restarted 24 hours after the conclusion of the surgery if there were no intraoperative events which would dictate otherwise. In patients who were on anticoagulation for h/o thromboembolism, prophylactic temporary retrievable Inferior Venacava filter may need to be considered prior to surgery.

K. Dementia or Psychiatric Illness/Treatment

No change from standard management.

V. Transitions of Care

B. Anticipated Length of Stay.

Length of stay is dependent on surgical issues and radiotherapy regimens. Physical therapy will be an integral part of discharge planning and in cases of metastatic malignancy with overall poor prognosis or those who remain nonambulatory, palliative care team can help facilitate family discussions, living arrangements, identify health proxies and establish goals of therapy.

C. When is the Patient Ready for Discharge.

  • Surgical and neurological stability

  • When pain adequately controlled

  • When spinal stability is agreed upon by surgical and radiation oncology staff

D. Arranging for Clinic Follow-up

N/A

1. When should clinic follow up be arranged and with whom.

  • Will need neurosurgery follow-up, especially needed to address post-surgical complications if any.

  • Radiotherapy and medical oncology for specific treatment protocols in those with epidural metastatic malignancies

  • Primary care Physician (PCP) follow-up in one to two weeks to address issues of steroid taper/pain control/drug side effects/warfarin follow-up, etc.

  • If prophylactic inferior vena cava (IVC) filter was placed, will need interventional radiology follow-up after the PCP follow-up, so that he/she could coordinate its removal after discussing with the neurosurgeon and oncologists.

2. What tests should be conducted prior to discharge to enable best clinic first visit.

None.

3. What tests should be ordered as an outpatient prior to, or on the day of, the clinic visit.

None.

E. Placement Considerations.

Patients with limited functional status before surgery are most likely to need placement and initiating disposition plans earlier will expedite discharge when medically ready.

F. Prognosis and Patient Counseling.

With epidural spinal metastases prognosis is generally poor. For example, median survival after radiotherapy for lung primary is 1.5 months while with breast cancer it is 9 months. Two out of three patients who could walk before treatment will continue to do so after treatment, while 1 out of 3 will become ambulatory if they had mild to moderate paraparesis before treatment. In patients who present with complete paraplegia, especially if it lasts longer than 24 hours, even with most optimistic views only about 1 in 4 patients may regain some ability to walk.

VI. Patient Safety and Quality Measures

A. Core Indicator Standards and Documentation.

None.

B. Appropriate Prophylaxis and Other Measures to Prevent Readmission.

  • If patient is being discharged to a skilled nursing facility/rehab center, instructions for falls precautions and DVT prophylaxis should be clear.

  • When appropriate, transition to oral opiate regimes at least 24- 48 hours before discharge to let patients assess the adequacy of pain control.

  • This will also let you adjust the bowel regimen accordingly to avoid constipation.

If a patient is being discharged home with palliative or hospice care, ensure all team members i.e. PCP, hospice MD are aware. Ensure that the patient and family members know who is going to follow-up at home, who they can call when needed, and that their expectations from those care providers is realistic and achievable. Specific detailed discussion will help to avoid a sense of abandonment which is an important aspect of preventing readmission.

What's the evidence?

Wilkins, R, Rengachary, SL. "Spinal Epidural Tumors". Neurosurgery. McGraw-Hill. 1996. pp. 1791-1804.

McGuire, EJ. "Urodynamics of the Neurogenic Bladder". Urol Clin N Am. vol. 37. 2010. pp. 507-516.

Abrahm, JL, Banffy, MB. "Spinal cord compression in patients with advanced metastatic cancer". JAMA. vol. 299. 2008. pp. 937-946.

Campbell, WW. DeJong's The Neurologial examination.. Lippincott Williams & Wilkins. 2005.

Patten, J. "The Anatomy, Physiology and Clinical features of Spinal cord Disease". In: Neurological Differential Diagnosis. 1996. pp. 139-144.

Khasraw, M, Posner, J. "Neurological complications of Cancer". Lancet Neurology. vol. 9. 2010. pp. 1214-1227.

Loblaw, DA, Laperriere, NJ. "Emergency treatment of Malignant extradural spinal cord compression: An evidence based guidline". J Clin Oncol. vol. 16. 1998. pp. 1613.

Klausner, AP, Steers, WD. "The Neurogenic Bladder: An update with management strategies for Primary care Physicians". Med Clinc N Am. vol. 95. 2011. pp. 111-120.

Braunwald, E, Fauci, AS, Kasper, DL, Hauser, SL, Longo, DL, Jameson, JL. Diseases of the Spinal Cord. In: Harrison's Principles of Internal Medicine. Mc Graw Hill. 2001. pp. 2425-2434.

Bradley, W. "Paraplegia and Spinal cord Syndromes". In: Neurology in Clinical Practice. E-Dition. 2008. pp. 353-364.

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