What Are the Signs, Symptoms, and Treatments of Spinal Cord Compression?


Introduction and Scope of the Problem

Malignant spinal cord compression occurs in 2.5% to 5% of patients with cancer in the last 5 years of life. It presents as the initial manifestation of malignancy in up to 20% of new cancer diagnoses. The cumulative incidence varies by cancer type and is likely to continue to increase with improved cancer survival rates. Systemic cancers more likely to lead to spinal cord compression include prostate, breast, renal, and lung cancer; lymphoma; sarcoma; and multiple myeloma ( Table 60.1 ). The thoracic spine is the most common site for spinal cord metastases (70%), with the lumbar spine being second (20%); multiple levels are involved about one-third of the time.

Table 60.1
Cancers Commonly Associated With Spinal Cord Compression
Account for 15% to 25% of those presenting with MSCC
  • Prostate cancer

  • Lung cancer

  • Breast cancer

Account for 5% to 10% of those presenting with MSCC
  • Kidney or renal cancer

  • Lymphoma

  • Myeloma

Account for less than 5% of those presenting with MSCC
  • Colorectal cancer

  • Tumors of unknown primary

  • Melanoma

  • Sarcoma

MSCC , Malignant spinal cord compression.

Although in most instances spinal cord compression occurs in cancer as a result of metastatic spread to the spinal cord, it can also occur as a result of ischemic or hemorrhagic insults to the circulation involving the spinal cord, complications of radiation or chemotherapy, infections, or paraneoplastic syndromes. Occasionally, spinal cord compression occurs in the setting of primary spinal cord tumors such as astrocytomas and ependymomas. The focus of this chapter is metastatic spread to the spinal cord or malignant spinal cord compression (MSCC). The treatment of spinal cord compression is considered a medical emergency.

Historically, a diagnosis of MSCC was felt to portend an overall poor prognosis; however, it is known that patients with MSCC are a heterogenous population, with subsets of patients who share certain characteristics having improved survival. In a retrospective study of 176 patients with MSCC referred for surgical resection, median survival was 9.4 months after surgery. Primary cancer type was significant in predicting survival with lung, colorectal, bladder, esophageal, liver, and stomach cancers associated with a poorer prognosis and breast cancer, lymphoma, multiple myeloma, kidney, prostate, and thyroid cancers associated with a better prognosis. Other factors associated with decreased survival included older age, additional comorbidities, pathological spinal fractures, poor performance status, neurological deficits, and metastasis to the lung, liver, or brain.

In light of these findings, patient-based prognostic calculators have recently emerged in an effort to help with individual patient survival prediction. In the same retrospective study by Ahmed and colleagues, nine of these scoring systems were assessed for their accuracy in predicting short-term (30- and 90-day) and long-term (1-year) survival in patients by cancer type. Results from this analysis showed that the Skeletal Oncology Research Group nomogram developed by Paulino Pereira and colleagues incorporating different variables ranging from patient age to different lab values demonstrated the highest accuracy at predicting short-term survival. Tokuhashi and colleagues developed a prognostic scoring system that incorporates patient factors such as presence of metastatic disease and performance status to predict long-term survival. These tools can assist surgeons in a more personalized estimate of patient prognosis preoperatively.

Finally, in addition to better prognostication from improved understanding of the MSCC population, research has shown that treatment has increased the postoperative survival of patients with MSCC over the past 20 years. In a retrospective study of 1,515 patients who underwent surgery for MSCC from 1998 to 2017, patients with renal cancer had a 60% improvement in survival, patients with lung cancer had a 40% improvement in survival, and patients with colon cancer doubled their postoperative survival time.

Relevant Pathophysiology

MSCC usually spreads into the epidural space through extension of the tumor or through hematogenous spread via Batson’s venous plexus. Other, less common modes of spread into the spinal cord are through leptomeningeal and intramedullary spread. Leptomeningeal spread can take place through seeding of the meninges by the primary tumor (as can be seen in lung and breast cancer, melanoma, and lymphoma). Multiple myeloma typically presents with lytic bone lesions that extend into soft tissue as a result of activation of osteoclasts. Signs and symptoms are similar to those seen with epidural spread (see later discussion); however, lower-extremity weakness and paresthesias may be more pronounced. Intramedullary spread is relatively rare; in this instance, the primary tumor metastasizes into the spinal cord itself. Signs and symptoms are similar to those in other types of MSCC; however, weakness may be unilateral. Solid and hematological malignancies can also cause weakening of the bone leading to vertebral compression fractures. Bony fragments from a compression fracture may lead to compression of the spinal cord directly.

Symptoms

Back pain is the most common symptom seen in MSCC and occurs in 69% to 90% of cases. At presentation, many patients have already had pain for weeks or even months. Pain may be initially localized and then become more diffuse and radicular in nature. It is not uncommon for pain to radiate to the scapula, in a band across the chest or upper abdomen, or to present as hip pain in lower spinal cord disease. Pain may also be more intense after lying down because of the expansion of the venous plexus with recumbency, which leads many patients to report that it is worse at night or in the early morning. Pain that worsens while lying flat may also represent instability in the thoracic spine from unstable kyphosis.

Because early detection of MSCC is key to regaining mobility after treatment, MSCC must constantly be kept in the differential diagnoses of patients presenting with back pain, especially if the symptom is new, has changed in character, or involves the thoracic portion of the spine (because thoracic pain is relatively uncommon in typical osteoarthritis involving the spine). It is also important to keep in mind that in some cases, MSCC may be the initial presenting condition of a previously undiagnosed malignancy. However, pain is not as helpful in distinguishing MSCC from other conditions as other signs and symptoms. In a retrospective study of 342 episodes of suspected MSCC evaluated by computed tomography (CT), the presence of pain did not differentiate between those with and without MSCC. Instead, the symptom of weakness (inability to walk) and increased deep tendon reflexes is more predictive of MSCC.

Signs

Muscle weakness is a telltale sign in MSCC. It is present in 35% to 75% of patients at diagnosis; half are too weak to walk. Thoracic spine involvement is associated with the greatest amount of weakness. Motor deficits at diagnosis influence response to treatment and survival. Patients often do not complain of sensory deficits; these deficits, however, are usually present on careful examination. Work by Helwig-Larsen and colleagues suggests that sensory deficits are more common with lumbar spine metastases and motor deficits seen more in thoracic MSCC. Lhermitte sign, which is characterized by a shocklike sensation in the back, arms, and legs when the neck is flexed, can be seen with both cervical and thoracic MSCC. Changes in bowel and bladder function tend to occur late in MSCC. At diagnosis, up to half of patients have bladder dysfunction and are catheter dependent. Patients with cauda equina syndrome predictably present with decreased sensation in a saddle distribution, urinary retention, and lax anal sphincter tone. This presentation is sensitive and specific for the diagnosis, although confirmation imaging is needed if the diagnosis is suspected.

Summary of Evidence Regarding Diagnostic Workup

Whether to evaluate or confirm the diagnosis of MSCC is often the first diagnostic decision confronting a clinician. MSCC should always be considered early in a patient with cancer who presents with new back pain or new neurological deficits. Although imaging and treatment are often well tolerated, the burden of imaging in the setting of a frail patient with advanced cancer can be significant. MRI is the gold standard for diagnosis with a sensitivity of 93% and specificity of 97% but generally requires imaging time of well over 1 hour and necessitates sedation in some patients. The determination of whether to pursue confirmatory imaging should be made after clear dialogue with the patient and family. The decision should be based on the likelihood that the patient will benefit from treatment, that the burden of evaluation is acceptable, that significant pretest probability for MSCC exists, and that further evaluation and potential treatment are consistent with the patient’s goals. Pretest probability can be estimated with four factors on history and physical examination: an abnormal neurological examination, a known cancer diagnosis, a known vertebral metastasis at presentation, and new mid- to upper back pain. Patients with none of these four factors had a spinal cord compression incidence of 8% (notably not an insignificant percentage), and patients with three or four of these risk factors had an 81% likelihood of spinal cord compression.

Imaging

MRI should be the first imaging study performed when MSCC is suspected. It is broadly considered the current gold standard. In addition to excellent visualization of bony structures, MRI affords detection of intraforaminal and leptomeningeal disease. MRI of the complete spine is recommended because 30% of MSCC cases may involve noncontiguous spinal levels.

Other Diagnostic Considerations

In patients with spinal cord compression as the first manifestation of malignancy, CT-guided needle biopsy has been shown to be safe and effective. Biopsy of the spinal lesion in many of these cases is preferred because the tumor is often of unknown, lung, or hematological origin. Cerebrospinal fluid studies are of limited value in diagnosis of MSCC. Even in the often overlooked leptomeningeal variant of spinal cord compression, the initial cerebrospinal fluid sample fails to yield cells in 10% to 40% of cases. In most instances, further diagnostic evaluation beyond MRI is not needed to confirm the diagnosis of MSCC. Additional testing may be helpful to better define the extent of disease, which might have an impact on treatment choices and prognosis.

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