Clinical features and diagnosis of neoplastic epidural spinal cord compression, including cauda equina syndrome
- David Schiff, MD
David Schiff, MD
- Professor of Neurology, Neurological Surgery, and Medicine
- University of Virginia School of Medicine
- Section Editor
- Reed E Drews, MD
Reed E Drews, MD
- Section Editor — Complications of Cancer
- Associate Professor of Medicine
- Harvard Medical School
- Deputy Editors
- April F Eichler, MD, MPH
April F Eichler, MD, MPH
- Deputy Editor — Neurology and Sleep Medicine
- Assistant Professor of Neurology
- Harvard Medical School
- Diane MF Savarese, MD
Diane MF Savarese, MD
- Senior Deputy Editor — UpToDate
- Deputy Editor — Oncology and Palliative Care
- Clinical Instructor of Medicine
- Harvard Medical School
Neoplastic epidural spinal cord compression (ESCC) is a common complication of cancer that can cause pain and potentially irreversible loss of neurologic function. The degree of thecal sac compression required for the designation of ESCC has been variably defined; we and others consider any radiologic evidence of indentation of the thecal sac to be evidence for ESCC [1,2].
In adults, the tip of the spinal cord usually lies at the L1 vertebral level; below this level, the lumbosacral nerve roots form the cauda equina, which floats in cerebrospinal fluid (CSF). Since the pathophysiology of compression of the thecal sac at the level of the cauda equina does not differ significantly from that of more rostral compression, compression of the cauda equina is still generally referred to by the slightly inaccurate name of ESCC.
The epidemiology, pathophysiology, clinical features, and diagnosis of ESCC will be reviewed here. The treatment and prognosis of this disorder are discussed separately. (See "Treatment and prognosis of neoplastic epidural spinal cord compression, including cauda equina syndrome".)
Many cancer patients have asymptomatic or unrecognized ESCC, while others develop ESCC after the decision has been made to forgo extensive diagnostic testing or therapy. For these reasons, the incidence of this complication can only be estimated. One population-based study of spinal cord compression reported that the likelihood of a patient with cancer suffering cord compression in the five years before death was 2.5 percent, ranging from 0.2 percent in pancreatic cancer to 7.9 percent in myeloma . A study of malignant spinal cord compression in hospitalized patients with cancer identified an annual incidence of 3.4 percent .
Autopsy studies suggested that 5 percent of patients dying with cancer have ESCC . Similar findings were noted in a Danish study based upon referrals to a regional treatment center, in which the incidence of ESCC in cancer patients rose from 4.4 to 6 percent between 1979 and 1985 .
- Loblaw DA, Laperriere NJ. Emergency treatment of malignant extradural spinal cord compression: an evidence-based guideline. J Clin Oncol 1998; 16:1613.
- Schiff D, O'Neill BP, Wang CH, O'Fallon JR. Neuroimaging and treatment implications of patients with multiple epidural spinal metastases. Cancer 1998; 83:1593.
- Loblaw DA, Laperriere NJ, Mackillop WJ. A population-based study of malignant spinal cord compression in Ontario. Clin Oncol (R Coll Radiol) 2003; 15:211.
- Mak KS, Lee LK, Mak RH, et al. Incidence and treatment patterns in hospitalizations for malignant spinal cord compression in the United States, 1998-2006. Int J Radiat Oncol Biol Phys 2011; 80:824.
- BARRON KD, HIRANO A, ARAKI S, TERRY RD. Experiences with metastatic neoplasms involving the spinal cord. Neurology 1959; 9:91.
- Bach F, Larsen BH, Rohde K, et al. Metastatic spinal cord compression. Occurrence, symptoms, clinical presentations and prognosis in 398 patients with spinal cord compression. Acta Neurochir (Wien) 1990; 107:37.
- Constans JP, de Divitiis E, Donzelli R, et al. Spinal metastases with neurological manifestations. Review of 600 cases. J Neurosurg 1983; 59:111.
- Schiff D, O'Neill BP, Suman VJ. Spinal epidural metastasis as the initial manifestation of malignancy: clinical features and diagnostic approach. Neurology 1997; 49:452.
- Posner JB. Neurologic Complications of Cancer, FA Davis, Philadelphia 1995.
- Klein SL, Sanford RA, Muhlbauer MS. Pediatric spinal epidural metastases. J Neurosurg 1991; 74:70.
- Cole JS, Patchell RA. Metastatic epidural spinal cord compression. Lancet Neurol 2008; 7:459.
- Helweg-Larsen S, Hansen SW, Sørensen PS. Second occurrence of symptomatic metastatic spinal cord compression and findings of multiple spinal epidural metastases. Int J Radiat Oncol Biol Phys 1995; 33:595.
- Lada R, Kaminski HJ, Ruff R. Metastatic spinal cord compression. In: Handbook of Clinical Neurology, Part III, 69, Vecht C (Ed), Elsevier, Amsterdam 1997. Vol 25, p.167.
- Siegal T. Spinal cord compression: from laboratory to clinic. Eur J Cancer 1995; 31A:1748.
- Husband DJ. Malignant spinal cord compression: prospective study of delays in referral and treatment. BMJ 1998; 317:18.
- Greenberg HS, Kim JH, Posner JB. Epidural spinal cord compression from metastatic tumor: results with a new treatment protocol. Ann Neurol 1980; 8:361.
- Gilbert RW, Kim JH, Posner JB. Epidural spinal cord compression from metastatic tumor: diagnosis and treatment. Ann Neurol 1978; 3:40.
- Solberg A, Bremnes RM. Metastatic spinal cord compression: diagnostic delay, treatment, and outcome. Anticancer Res 1999; 19:677.
- Kovner F, Spigel S, Rider I, et al. Radiation therapy of metastatic spinal cord compression. Multidisciplinary team diagnosis and treatment. J Neurooncol 1999; 42:85.
- Helweg-Larsen S, Sørensen PS, Kreiner S. Prognostic factors in metastatic spinal cord compression: a prospective study using multivariate analysis of variables influencing survival and gait function in 153 patients. Int J Radiat Oncol Biol Phys 2000; 46:1163.
- Helweg-Larsen S, Sørensen PS. Symptoms and signs in metastatic spinal cord compression: a study of progression from first symptom until diagnosis in 153 patients. Eur J Cancer 1994; 30A:396.
- Bilsky MH. New therapeutics in spine metastases. Expert Rev Neurother 2005; 5:831.
- Fourney DR, Frangou EM, Ryken TC, et al. Spinal instability neoplastic score: an analysis of reliability and validity from the spine oncology study group. J Clin Oncol 2011; 29:3072.
- Fagius J, Westerberg CE. Pseudoclaudication syndrome caused by a tumour of the cauda equina. J Neurol Neurosurg Psychiatry 1979; 42:187.
- Lossos A, Siegal T. Electric shock-like sensations in 42 cancer patients: clinical characteristics and distinct etiologies. J Neurooncol 1996; 29:175.
- Newton HB, Rea GL. Lhermitte's sign as a presenting symptom of primary spinal cord tumor. J Neurooncol 1996; 29:183.
- Hainline B, Tuszynski MH, Posner JB. Ataxia in epidural spinal cord compression. Neurology 1992; 42:2193.
- van der Sande JJ, Kröger R, Boogerd W. Multiple spinal epidural metastases; an unexpectedly frequent finding. J Neurol Neurosurg Psychiatry 1990; 53:1001.
- Ruff RL, Lanska DJ. Epidural metastases in prospectively evaluated veterans with cancer and back pain. Cancer 1989; 63:2234.
- Hagenau C, Grosh W, Currie M, Wiley RG. Comparison of spinal magnetic resonance imaging and myelography in cancer patients. J Clin Oncol 1987; 5:1663.
- Godersky JC, Smoker WR, Knutzon R. Use of magnetic resonance imaging in the evaluation of metastatic spinal disease. Neurosurgery 1987; 21:676.
- Sarpel S, Sarpel G, Yu E, et al. Early diagnosis of spinal-epidural metastasis by magnetic resonance imaging. Cancer 1987; 59:1112.
- Rodichok LD, Harper GR, Ruckdeschel JC, et al. Early diagnosis of spinal epidural metastases. Am J Med 1981; 70:1181.
- Portenoy RK, Galer BS, Salamon O, et al. Identification of epidural neoplasm. Radiography and bone scintigraphy in the symptomatic and asymptomatic spine. Cancer 1989; 64:2207.
- Dunne JW, Harper CG, Pamphlett R. Intramedullary spinal cord metastases: a clinical and pathological study of nine cases. Q J Med 1986; 61:1003.
- Schiff D, O'Neill BP. Intramedullary spinal cord metastases: clinical features and treatment outcome. Neurology 1996; 47:906.
- Bleyer WA, Byrne TN. Leptomeningeal cancer in leukemia and solid tumors. Curr Probl Cancer 1988; 12:181.
- Wasserstrom WR, Glass JP, Posner JB. Diagnosis and treatment of leptomeningeal metastases from solid tumors: experience with 90 patients. Cancer 1982; 49:759.
- Jaeckle KA. Nerve plexus metastases. Neurol Clin 1991; 9:857.
- Goldwein JW. Radiation myelopathy: a review. Med Pediatr Oncol 1987; 15:89.
- Schultheiss TE, Stephens LC. Invited review: permanent radiation myelopathy. Br J Radiol 1992; 65:737.
- Zevgaridis D, Büttner A, Weis S, et al. Spinal epidural cavernous hemangiomas. Report of three cases and review of the literature. J Neurosurg 1998; 88:903.
- Alexiadou-Rudolf C, Ernestus RI, Nanassis K, et al. Acute nontraumatic spinal epidural hematomas. An important differential diagnosis in spinal emergencies. Spine (Phila Pa 1976) 1998; 23:1810.
- Chen CJ, Hsu WC. Imaging findings of spontaneous spinal epidural hematoma. J Formos Med Assoc 1997; 96:283.
- Aydingöz U, Oto A, Cila A. Spinal cord compression due to epidural extramedullary haematopoiesis in thalassaemia: MRI. Neuroradiology 1997; 39:870.
- Dibbern DA Jr, Loevner LA, Lieberman AP, et al. MR of thoracic cord compression caused by epidural extramedullary hematopoiesis in myelodysplastic syndrome. AJNR Am J Neuroradiol 1997; 18:363.
- Sasaki S, Nakamura K, Oda H, et al. Thoracic myelopathy due to intraspinal rheumatoid nodules. Scand J Rheumatol 1997; 26:227.
- Weissman MN, Lange R, Kelley C, et al. Intraspinal epidural sarcoidosis: case report. Neurosurgery 1996; 39:179.
- Dhôte R, Roux FX, Bachmeyer C, et al. Extradural spinal tophaceous gout: evolution with medical treatment. Clin Exp Rheumatol 1997; 15:421.
- - ESCC as initial manifestation of malignancy
- Localization within the spine
- Clinical features
- - Pain
- - Motor findings
- - Sensory findings
- - Bladder and bowel dysfunction
- - Ataxia
- Radiologic confirmation
- - Magnetic resonance imaging
- - Myelography
- - Other modalities
- DIFFERENTIAL DIAGNOSIS
- Musculoskeletal disease
- Spinal epidural abscess
- Metastatic disease
- Radiation myelopathy
- INFORMATION FOR PATIENTS