A spinal tumor is a malignant or benign growth that develops within or near the spine and/or spinal cord. Tumors can increase in size and compress adjacent tissues. Malignant tumors can additionally invade adjacent tissues and metastasize to other locations. One way spinal tumors are classified is by their location: intramedullary tumors occur within the spinal cord; intradural extramedullary tumors occur between the spinal cord and its protective sheath, the dura; and extradural tumors occur outside the dura.
All malignant and most benign tumors are the result of abnormal, excessive tissue growth following damage to genes, which could have occurred spontaneously or after exposure to chemicals, radiation, or infections. Primary spinal tumors originate from the tissues of the spine or spinal cord. Secondary spinal tumors are metastases from cancers originating elsewhere in the body. Cancers that commonly metastasize to the spine include prostate, lung, breast, and kidney.
Epidemiology including risk factors and primary prevention
About 10,000 new cases of spinal tumors occur in the United States annually,1 with about 850 to 1700 of these cases being adult primary spinal cord tumors.2 The annual incidence of pediatric spinal cord tumors is low, about 3 per million population in the United States.3 Secondary tumors make up over 70% of all spinal tumors. Fifty-five percent of all spinal tumors are extradural, secondary tumors. Between 55% and 65% of all spinal tumors are benign. Only 5% to 10% of all spinal tumors are intramedullary. Spinal tumors are more common in individuals with a history of cancer (particularly lung, breast, prostate, or kidney cancer), neurofibromatosis type 2, von Hippel-Lindau disease, compromised immune system, or a family history of spinal tumors.
Primary adult intradural intramedullary spinal cord (ILSC) tumors include ependymomas (50%-60%), astrocytomas (25%-35%), hemangioblastomas (3%-8%), and oligodendrogliomas. Primary adult intradural extramedullary spinal cord (IESC) tumors include meningiomas (50%), schwannomas (30%), and neurofibromas (20%). Primary adult extradural spinal cord (ESC) tumors include sarcomas (bone), chondrosarcomas (cartilage), leiomyosarcomas (smooth muscle), chordomas (notochord remnants), and lymphomas. The most common pediatric spinal tumors are neurodevelopmental tumors (31%), astrocytomas (29%), and neuroblastomas (14%).3
Spinal tumors cause neurologic injury by invading or compressing nerve tissues of the spinal cord or spinal nerves. Edema around the tumor can increase compression.
Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time)
New onset/acute: New spinal tumors are usually asymptomatic and unnoticed until they compromise a neurologic structure and cause progressively worsening pain and/or neurologic deficits, including sensory changes, balance difficulties, weakness, and bowel or bladder dysfunction. Spinal tumors can also be found incidentally during imaging workup for other disease conditions.
Subacute: Pain and/or neurologic deficits will progressively worsen with continued tumor growth.
Chronic/stable: If the tumor continues to grow, irreversible spinal cord injury, including paralysis, bowel and bladder incontinence, and loss of sensation below the level of the tumor could result.
Pre-terminal: Spinal tumors can result in life-threatening conditions, such as respiratory weakness, excessive tumor burden, and complications from neurologic dysfunction.
Specific secondary or associated conditions and complications
Neurologic presentations are a reflection of the area affected and can result in motor deficits, sensory deficits, autonomic dysfunction, neurogenic bowel and bladder, sexual dysfunction, and spasticity. Other complications include venous thromboembolism, pathologic fracture, pressure ulcers, and joint contractures.
2. ESSENTIALS OF ASSESSMENT
Pain is the most common presenting symptom (72%), and it manifests as back pain, radicular pain, or central pain in 27%, 25%, and 20% of the cases, respectively. Weakness is the second most common presenting symptom (55%), followed by sensory loss (39%). The pattern of weakness, sensory loss, and functional deficits can assist in localization of the neurologic injury. Bladder or bowel incontinence is a less common presenting symptom (15%). Oncologic history and history of associated symptoms that may suggest cancer, such as decreased appetite, night pain, and unexpected weight loss, should be assessed.
A thorough neurologic examination should be conducted, with emphasis on proprioception, sensation to pinprick, temperature, and light touch, strength, reflexes, and muscle tone. Any skin characteristics that may indicate a congenital disease should be noted. Spinal deformities or palpable masses may be present. In the case of paraplegia or tetraplegia, rectal examination to evaluate voluntary sphincter tone and deep anal pressure is warranted. A specific examination according to the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI), which includes some of the previously mentioned components, should be conducted.
The patient’s mobility should be evaluated, including ambulation, transfers, gait pattern, and any need for assistive devices. Activities of daily living, such as feeding, dressing, and grooming, should also be evaluated. The ability to perform necessary maneuvers for bowel and bladder cares should be examined. Physical therapists and occupational therapists are often involved in these assessments. The level of spinal cord injury will determine the neurologic and functional deficits and help focus the assessments.
Checking tumor markers may assist diagnosis of cancers and monitor disease progression.
Magnetic resonance imaging (MRI) is the recommended modality to evaluate spinal tumors. It can locate the spinal tumor, evaluate the extent of spinal cord compression, and help predict functional outcomes. MRI of other organ systems may locate the origin of secondary spinal tumors. CT scan with myelography is suggested for those who cannot undergo MRI. Skeletal imaging, such as bone scans, can locate bone metastases.
Supplemental assessment tools
Genetic evaluation may be useful in certain cases, such as neurofibromatosis and von Hippel-Lindau disease.
Early predictions of outcomes
The following MRI findings are associated with improved neurologic recovery: no intramedullary hemorrhage, limited cord edema, limited cord compression, and resolution of spinal cord signal abnormalities on serial MRI examinations. The specific tumor, its histologic type4, extent of surgical resection4, and its location will also influence prognosis and functional outcomes. Patients with primary spinal tumors tend to show faster and greater gains in motor function than patients with secondary spinal tumors.5
Tokuhashi score, revised in 2005, is a validated scoring system for outcome prediction in patients with spinal metastatic disease.6
ISNCSCI examination categorizes spinal cord injuries into classifications that have prognostic significance. In general, incomplete injuries have better functional outcomes than complete injuries.
ESCC in general has poor prognosis, with length of survival depending on ambulatory ability, primary tumor type, and the severity of metastasis.
A patient’s home setting may require modification to facilitate specific functional needs. Examples include installation of ramps, widening of doorways, adjustment of furniture heights, installation of grab bars, and acquisition of a hospital bed.
Social role and social support system
Patients often require assistance to return home. Medical and equipment costs are frequently the major barrier to successful home discharge. Health insurance coverage varies. Fortunately, there are means of decreasing costs, such as using recycled wheelchairs and adaptive equipment.
Medical assistance needs are another barrier for home discharge. If the patient requires palliative care or hospice service, home nursing is often provided to handle the patient’s medical needs; therefore, the family can focus on spending quality time with the patient. Although home nursing is usually available, the cost or frequency that it is provided may not meet the needs of individual patients and families.
When the amount of assistance required cannot be met by what can be provided at home, sometimes a stay at a skilled nursing facility is needed. However, this is often contrary to the patient’s and family’s wish to be at home. A short course of acute inpatient rehabilitation can sometimes address some of the medical issues, enhance independence of the patient, improve quality of life, and provide family education to facilitate the patient’s return home.
Patients may require accommodations to return to work. Other patients may require job skills training to obtain new work that they are able to perform. Vocational therapists can facilitate this process.
3. REHABILITATION MANAGEMENT AND TREATMENTS
Available or current treatment guidelines
For metastatic extradural spinal cord compression, a set of evidence-based clinical practice guidelines was updated in 2011.7 Highlights include the following: unless contraindicated, steroids are recommended for patients with neurologic deficits because of compression of neurologic structures; consider surgery if the patient has a good prognosis and is medically stable; otherwise, consider radiation therapy; and monitor patients for tumor recurrence. Tokuhashi score may provide guidance for surgical decision-making8, but should not be the only determination factor9.
According to the Cochrane review, radiotherapy is an essential component of treatment in adults with metastatic extradural spinal cord compression (MESCC). Radiotherapy could be the primary treatment for ambulatory patients with stable spines and for those who cannot tolerate surgical treatment.10
There are no general treatment guidelines for primary intradural spinal tumor. Treatment plan should be individualized based on histologic type and patient’s condition.
At different disease stages
- Acute cord compression should be treated emergently, with consideration for surgery and steroids (and radiation in nonsurgical candidates).
- Surgery is often the preferred treatment, especially for intramedullary tumors.
- Provide pain management. Corticosteroid may be considered for postoperative edema.
- Consider acute inpatient rehabilitation hospital admission.
- Potentially adjunctive radiation therapy and chemotherapy for chemosensitive tumors.
- Continue pain management.
- Transition to outpatient rehabilitation therapies.
- Monitor for tumor recurrence or progression.
- Tumor debulking may be considered as a palliative measure.
- Continue pain management.
- Patient performs home exercise program.
Pre-terminal or end of life care
- Provide pain management.
- Nutritional support, bowel and bladder care, and skin care should continue, perhaps in a hospice setting.
Coordination of care
Patients with spinal tumors will benefit from an interdisciplinary, well-organized care plan. This potentially includes neurosurgery/spine orthopedics, neurology, oncology, radiation oncology, rehabilitation physicians, primary care physicians, physical therapy, occupational therapy, and vocational counseling. For certain patients, palliative care teams are crucial to optimizing patient care.
Patient & family education
Patient and family education should include disease process and prognosis, treatment goals, management of complications, such as respiratory dysfunction, sexual dysfunction, autonomic dysfunction, bowel and bladder dysfunction, contractures, and decubitus ulcers. Caregivers should be educated and trained to assist in proper mobility techniques and other activities of daily living.
Surveillance, Epidemiology, and End Results (SEER) database of the National Cancer Institute provides valuable epidemiologic information, especially for the relatively rare occurrence of primary spinal tumors. Subsequence research can be conducted to obtain further statistics about various histologic types of spinal tumors.
A short, directed course of multidisciplinary rehabilitation that focuses on improving mobility, transfer, self-care should be advocated. Various studies have found that it achieve the goals, improve returning to home, potentially lower pain level and depression, with maintenance of its effect until the time of death.
Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills
Tumor histologic type is a major determinant of mortality among the other factors.11 Tumor location is a major determinant of functional outcome.5Early diagnosis is important. No one “red flag” question can accurately indicate malignancy in low back pain.12 MRI is the preferred imaging tool. Biopsy and staging are the keys to determine future management.
4. CUTTING EDGE/EMERGING AND UNIQUE CONCEPTS AND PRACTICE
Cutting edge concepts and practice
Cancer stem cells and their roles in chemoresistance, radiation resistance, and metastasis have aroused significant scientific interests in novel therapies targeting these cells.13 Monoclonal antibodies and their neuroprotective effects in spinal cord injury are another area being studied that could impact future treatments.14
Stereotactic body radiotherapy (SBRT) or spine radiosurgery (SR)-based ablative treatment has been utilized to delivery high-dose radiation for local control of metastatic spinal tumors.15
5. GAPS IN THE EVIDENCE-BASED KNOWLEDGE
Gaps in the evidence-based knowledge
There are many proposed modalities for neurorehabilitation. These include partial or full body-weight-supported treadmill, functional electric stimulation, and other complementary modalities, such as acupuncture. However, no clear scientific studies have been conducted to show definitive effects of these treatments for recovery from spinal tumors.
Further studies are needed to determine the efficacy and safety of various minimally invasive surgical techniques, radiosurgery, optimal dosage of radiation therapy in the primary treatment of MESCC and intradural primary spinal tumors.
- American Association of Neurological Surgeons. Spinal tumors. July 2014. Available at: http://www.aans.org/Patient%20Information/Conditions%20and%20Treatments/Spinal%20Tumors.aspx. Accessed March 14, 2016.
- Campello C, Le Floch A, Parker F, et al. Neuroepithelial intramedullary spinal cord tumors in adults: study of 70 cases. Paper presented at: American Academy of Neurology Annual Meeting; 2009; Seattle, WA.
- Wilson PE, Oleszek JL, Clayton GH. Pediatric spinal cord tumors and masses. J Spinal Cord Med. 2007;30(Suppl 1):S15-S20.
- Raco A, Esposito V, Lenzi J, Piccirilli M, Delfini R, Cantore G. Long-term follow-up of intramedullary spinal cord tumors: a series of 202 cases. Neurosurgery. 2005;56:972-981.
- Tan M, New PW. Retrospective study of rehabilitation outcomes following spinal cord injury due to tumour. Spinal Cord. 2012;50:127-131.
- Tokuhashi Y, Matsuszaki H, Oda H, Oshima M, Ryu J. A revised scoring system for preoperative evaluation of metastatic spine tumor prognosis. Spine (Phila Pa 1976) 2005; 30: 2186-2191.
- Loblaw DA, Mitera G, Ford M, Laperriere NJ. 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;84:312-317.
- Quraishi NA, Manoharan SR, Arealis G, Khurana A, Elsayed S, Edwards KL, Ooszczyk BM. Accuracy of the revised Tokuhashi score in predicting survival in patients with metastatic spinal cord compression (MSCC). Eur Spine J. 2013 Mar;22 Suppl 1:S21-6.
- Eap C, Tardieux E, Goasgen O, Bennis S, Mireau E, Delalande B, Cvitkovik F, Baussart B, Aldea S, Jovenin N, Gaillard S. Tokuhashi score and other prognostic factors in 260 patients with surgery for vertebral metastases. Orthop Traumatol Surg Res. 2015 Jun;101(4):483-8.
- George R, Jeba J, Ramkumar G, Chacko AG, Tharyan P. Interventions for the treatment of metastatic extradural spinal cord compression in adults. Cochrane Database Syst Rev. 2015 Sep 4;9.
- Tan M, New P. Survival after rehabilitation for spinal cord injury due to tumor: a 12-year retrospective study. J Neurooncol. 2011;104:233-238.
- Downie A, Williams CM, Henschke N, Hancock MJ, Ostelo RW, de Vet HC, Macaskill P, Irwig L, van Tulder MW, Koes BW, Maher CG. Red flags to screen for malignancy and fracture in patients with low back pain.Br J Sports Med. 2014 Oct;48(20):1518.
- Hsu W, Mohyeldin A, Shah SR, Gokaslan ZL, Quinones-Hinojosa A. Role of cancer stem cells in spine tumors: review of current literature. Neurosurgery. 2012;71:117-125.
- Sharma A, Sharma HS. Monoclonal antibodies as novel neurotherapeutic agents in CNS injury and repair. Int Rev Neurobiol. 2012;102:23-45.
- Moraes FY, Taunk NK, Laufer I, Neves-Junior WF, Hanna SA, Carvalho Hde A, Yamada Y. Spine radiosurgery for the local treatment of spine metastases: Intensity-modulated radiotherapy, image guidance, clinical aspects and future directions. Clinics (Sao Paulo). 2016 Feb;71(2):101-9.
Bryce TN, Ragnarsson KT, Stein AB, Biering-Sorensen F. Spinal cord injury: anatomy, mechanics, and syndrome of traumatic injury. In: Braddom RL, Chan L, Harrast MA, et al, eds. Physical Medicine and Rehabilitation. 4th ed. Philadelphia, PA: Elsevier Saunders; 2011:1296-1301.
Stephen M, Selkirk and Robert Louis Ruff. Primary and Metastatic Tumors of the Spinal Cord and Spinal Canal. In: Kirshblum S, Campagnolo DL, et. al. Spinal Cord Medicine. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins, a Wolters Kluwer business; 2011: 575-587.
Chamberlain MC, Tredway TL. Adult primary intradural spinal cord tumors: a review. Curr Neurol Neurosci Rep. 2011;11:320-328.
Westwick HR, Shamju MF. Effects of sex on the incidence and prognosis of spinal meningiomas: a Surveillance, Epidemiology, and End Results study. J Neurosurg Spine. 2015. 23:368-373.
American Spinal Injury Association. International Standards for Neurological Classification of SCI (ISNCSCI) Exam. 2015. Available at: http://www.asiaspinalinjury.org/elearning/ISNCSCI.php. Accessed March 19,2016.
American Cancer Society. Brain and spinal cord tumors in adults. 2014. Available at: http://www.cancer.org/acs/groups/cid/documents/webcontent/003088-pdf.pdf. Accessed March 19, 2016.
Mayo Clinic. Spinal tumor. Available at: http://www.mayoclinic.org/diseases-conditions/spinal-cord-tumor/home/ovc-20117315. Accessed March 16, 2016.
Original Version of the Topic:
Tommy C. Yu, MD, Sunlung Suen, MD. Spinal tumors. Publication Date:2013/07/11.
Tommy C. Yu, MD
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