Spinal Tumors

Disease/Disorder

Definition

A spinal tumor is a malignant or benign growth that develops within or near the spine and/or spinal cord. Although relatively uncommon, these tumors can result in substantial morbidity often impacting motor and sensory function and causing significant pain. Both benign and malignant tumors can increase in size and compress adjacent tissues, while malignant tumors can additionally invade adjacent tissues and metastasize to other locations. Spinal tumors are often classified by their location: intradural-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.

Etiology

All malignant and most benign tumors are the result of abnormal, excessive tissue growth following damages to genes.¹ Primary spinal tumors originate from the tissues of the spine or spinal cord and can be cancerous or non-cancerous. Secondary spinal tumors are metastases from cancers originating elsewhere in the body. Cancers that commonly metastasize to the spine include prostate, lung, breast, kidney.

Epidemiology including risk factors and primary prevention

The annual incidence of primary spinal cord tumors is estimated to be 4,100 cases with the majority seen in adults.²  Although the exact incidence is unknown, metastatic disease to the central nervous system is approximately tenfold more common than benign disease.³ When categorized by anatomical location, about 55-60% of all spinal tumors are extradural and 40-45% of them are intradural.⁴ Among the intradural tumors, 30% are intramedullary (or 5-10% of all spinal tumors), while the remainder are extramedullary.⁵ Secondary tumors from metastatic disease most commonly present in the extradural space and are located in the thoracic spine (70%) followed by the lumbar spine (20%) and less commonly the cervical and sacral spine.⁶

Although there is limited evidence regarding risk factors for developing spinal cord tumors, it is thought that exposure to chemicals, radiation, certain infections, and hereditary conditions such as neurofibromatosis and von Hippel-Lindau may increase risk.¹

Patho-anatomy/physiology

Primary adult intradural-intramedullary spinal cord tumors are the least common type of spinal tumor accounting for 2-5% of all tumors and are most commonly ependymomas (50%-60%) followed by astrocytomas (25%-35%), hemangioblastomas (3%-8%), and oligodendrogliomas.⁷ Primary adult intradural-extramedullary spinal cord tumors usually arise from the spinal nerves and dura, with meningiomas (20-25%) and schwannomas (15-50%) being the most common in this location.⁸ Although uncommon, primary tumors can originate in the extradural space and can be both benign and malignant including osteomas, plasma cell tumors and hemangiomas, as well as chordomas, chondrosarcomas, Ewing’s sarcoma, osteosarcoma, plasma cell neoplasms and lymphoma, respectively.⁹ The most common pediatric spinal tumors are neurodevelopmental tumors (31%), astrocytomas (29%), and neuroblastomas (14%).¹⁰

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. Both invasion and compression of the cord can result in clinical presentation of a spinal cord injury.

Table 1: Categorization of Spinal Tumors by Anatomical Location⁹

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 deficits, weakness, poor balance, 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 may progressively worsen with continued tumor growth.

Chronic/stable: With continued tumor growth or prolonged spinal cord compression, irreversible neurological dysfunction from spinal cord injury can result. This may include persistent sensory deficits, weakness, neurogenic bowel, and neurogenic bladder.

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 reflect the spinal cord level and somatotopic area affected and can result in motor deficits, sensory deficits, autonomic dysfunction, neurogenic bowel and bladder, sexual dysfunction, and neuropathic pain. Other complications include venous thromboembolism, pathologic fracture, pressure injuries, spasms, and spasticity that if not adequately addressed can result in joint contractures.

Essentials of Assessment

History

The two most common presenting symptoms for patients with spinal tumors are pain and neurologic dysfunction. Pain can manifest as back pain, radicular pain, or central pain depending on the size and location of the tumor. Neurologic dysfunction can present as weakness, sensory loss, changes in reflexes, ambulatory difficulties and/or loss of voluntary control of bowel/bladder. The pattern of weakness, sensory loss, and functional deficits can assist in localization of the neurologic injury. Oncologic history and history of associated symptoms that may suggest cancer, such as decreased appetite, night pain, and unexpected weight loss, should be assessed.

Physical examination

A thorough neurologic examination should be conducted, with emphasis on proprioception, sensation to pinprick, light touch, and temperature as well as 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 anal contraction 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.

Functional assessment

The neurological level of injury will drive neurological and functional deficits. The patient’s mobility should be evaluated, including ambulation, transfers, gait pattern, and any need for assistance or assistive devices. Activities of daily living, such as feeding, dressing, toileting, and grooming, should also be evaluated. Physical and occupational therapists are highly skilled in performing these assessments and should be involved as early as possible in the care of these patients .

Laboratory studies

Checking tumor markers may assist diagnosis of cancers and monitor disease progression.

Imaging

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. Computed tomography (CT) and 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 specific tumor, its histologic type, extent of surgical resection, and its location will also influence prognosis and functional outcomes.¹¹ Patients with primary spinal tumors tend to show faster and greater gains in motor function than patients with secondary spinal tumors.

There are many scoring systems that estimate the prognosis and survival of patients with metastatic spinal tumors using multiple factors. These scales include the revised Tokuhashi score, Tomita scale and Katagari scale.¹² Although these scales can be helpful, they should not be the only factor as they do not account for factors such as quality of life.

The International Standards for Neurological Classification of Spinal Cord Injuries (ISNCSCI) examination describes neurological level of injury (NLI) as well as completeness of spinal cord injury. It has been shown to have prognostic value in the traumatic spinal cord injury population in terms of mobility and functional outcome.  In general, incomplete spinal cord injuries have better functional outcomes than complete injuries. The ISNCSCI exam should also be used for those with non-traumatic spinal cord injuries, such as compression from spinal tumors. While helpful in describing these injuries, it should not be used for prognostic purposes given that its prognostic value is still under investigation in the non-traumatic spinal cord injury population.

Environmental

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; this way, the family can focus on spending quality time with the patient. Although home nursing is usually available, its cost or the 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. When appropriate, a short course of acute inpatient rehabilitation can 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.

Professional issues

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.

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 2011.¹³  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.

Several scoring systems have been developed over the years to assist with guidance for surgical decision-making including the Epidural Spinal Cord Compression (ESCC) grade, which bases recommendations on the degree of spinal cord compression and the Spinal Instability Score (SINS), which assess for stability of the spine.¹⁴

According to the Cochrane review, radiotherapy is an essential component of treatment in adults with metastatic extradural spinal cord compression. For those with radiosensitive tumors, radiotherapy could be the primary treatment for ambulatory patients with stable spines and for those who cannot tolerate surgical treatment.¹⁵ Improving quality of life, relief of pain, and maintenance of neurological functions would be the goal of treatments.

There are no general treatment guidelines for primary intradural-intramedullary and intradural-extramedullary spinal tumors. Treatment plans should be individualized based on histologic type and the patient’s condition.

At different disease stages

New onset/acute Surgery is often the preferred treatment, especially for intramedullary tumors.

• Acute cord compression should be treated emergently, with consideration for surgery and steroids (with or without radiation).
• Surgery is often the preferred treatment for benign tumors.
• Provide pain management. Corticosteroid may be considered both preoperatively and postoperatively to reduce edema.
• Consider acute inpatient rehabilitation hospital admission.

Subacute

• Incorporate adjunctive radiation therapy and/or chemotherapy for chemosensitive tumors.
• Continue pain management.
• Transition to outpatient rehabilitation therapies.

Chronic/stable

• Monitor for tumor recurrence or progression.
• Tumor debulking may be considered as a palliative measure.
• Continue pain management.
• Initiates a home exercise program.

Pre-terminal or end of life care

• Provide pain management.
• Palliative care should be involved in order to discuss the goals of care and establish advance care planning. It can play a significant role in managing the symptoms caused by cancers and providing support to the patients and families.
• Nutritional support, bowel and bladder care, and skin care should be offered early and continue throughout treatment.

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, physiatry, primary care medicine, physical therapy, occupational therapy, and vocational counseling. For certain patients, palliative care teams are also 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 pressure injuries. Caregivers should be educated and trained to assist in proper mobility techniques and other activities of daily living.

Emerging/unique interventions

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, transfers, and self-care should be advocated. Various studies have found that rehabilitation can help patients  achieve their goals, increase the likelihood of  returning home, and  lower pain level and depression.¹⁶

Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

Tumor histologic type and location are major determinants in functional outcomes and mortality.¹¹ Early diagnosis is important as no one “red flag” question can accurately indicate malignancy in low back pain. MRI is the preferred imaging tool. Biopsy and staging are the keys to determine future management.

Cutting Edge/Emerging and Unique 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.¹⁷

Stereotactic body radiotherapy or spine radiosurgery-based ablative treatment has been utilized to delivery high-dose radiation for local control of metastatic spinal tumors.¹⁸ In selected patients, stereotactic radiosurgery can be implemented as an alternative or adjuvant treatment to surgery.There is evidence for brachytherapy as a possible modality for pain control, but more studies are needed to establish its effect on local tumor control and survival.¹⁹ Finally, nanomedicine is a new area that requires further research, but early results have been promising for development of localized and targeted treatments for spinal cord tumors.²⁰

Gaps in the Evidence-Based Knowledge

There are many proposed modalities for neurorehabilitation. These include partial or full body-weight-supported treadmill, functional electrical 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, and optimal dosage of radiation therapy in the primary treatment of both primary and secondary spinal tumors.

References

1. National Cancer Institute. Adult Central Nervous System Tumors Treatment (PDQ®)–Health Professional Version.; March 2024. https://www.cancer.gov/types/brain/hp/adult-brain-treatment-pdq. Accessed April 24, 2024.
2. Ostrom QT, Price M, Neff C, et al. CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2015-2019. Neuro Oncol. 2022;24(Suppl 5):v1-v95. doi:10.1093/neuonc/noac202
3. Boire A. Metastasis to the Central Nervous System. Continuum (Minneap Minn). 2020;26(6):1584-1601. doi:10.1212/CON.0000000000000939
4. Arnautovic K, Arnautovic A. Extramedullary intradural spinal tumors: a review of modern diagnostic and treatment options and a report of a series. Bosn J Basic Med Sci. 2009;9 Suppl 1(Suppl 1):S40-S45. doi:10.17305/bjbms.2009.2755
5. Rasras S, Kiani A. Intradural Extramedullary Spinal Tumors. In: Roy Morgan L, Birol Sarica F, eds. Brain and Spinal Tumors – Primary and Secondary. IntechOpen; 2020. doi:10.5772/intechopen.85360
6. Gokaslan ZL, Fisher CG, Boriani S, Vialle LR, AOSpine International, eds. Metastatic Spinal Tumor. Thieme; 2015.
7. M Das J, Hoang S, Mesfin FB. Intramedullary Spinal Cord Tumors. In: StatPearls. StatPearls Publishing; 2024. Accessed April 24, 2024. http://www.ncbi.nlm.nih.gov/books/NBK442031/
8. Puac-Polanco P, Guarnizo A, Cruz JP, Rodriguez FR, Torres CH. Intradural Extramedullary Tumors and Associated Syndromes. Neuroimaging Clin N Am. 2023;33(3):407-422. doi:10.1016/j.nic.2023.03.002
9. Kumar N, Tan WLB, Wei W, Vellayappan BA. An overview of the tumors affecting the spine-inside to out. Neurooncol Pract. 2020;7(Suppl 1):i10-i17. doi:10.1093/nop/npaa049
10. Wilson PE, Oleszek JL, Clayton GH. Pediatric spinal cord tumors and masses. J Spinal Cord Med. 2007;30 Suppl 1(Suppl 1):S15-20. doi:10.1080/10790268.2007.11753963
11. 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(5):972-981; discussion 972-981.
12. Denisov AA, Zaborovsky NS, Ptashnikov DA, Mikhailov DA, Masevnin SV, Smekalenkov OA. Comparison of prognostic scales for patients with metastatic spine disease. Orthop Rev (Pavia). 2020;12(4):8822. doi:10.4081/or.2020.8822
13. 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(2):312-317. doi:10.1016/j.ijrobp.2012.01.014
14. Tsukamoto S, Kido A, Tanaka Y, et al. Current Overview of Treatment for Metastatic Bone Disease. Curr Oncol. 2021;28(5):3347-3372. doi:10.3390/curroncol28050290
15. 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;2015(9):CD006716. doi:10.1002/14651858.CD006716.pub3
16. Raj VS, Lofton L. Rehabilitation and treatment of spinal cord tumors. J Spinal Cord Med. 2013;36(1):4-11. doi:10.1179/2045772312Y.0000000015
17. 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(1):117-125. doi:10.1227/NEU.0b013e3182532e71
18. de Moraes FY, Taunk NK, Laufer I, et al. Spine radiosurgery for the local treatment of spine metastases: Intensity-modulated radiotherapy, image guidance, clinical aspects and future directions. Clinics (Sao Paulo). 2016;71(2):101-109. doi:10.6061/clinics/2016(02)09
19. Zuckerman SL, Lim J, Yamada Y, Bilsky MH, Laufer I. Brachytherapy in Spinal Tumors: A Systematic Review. World Neurosurg. 2018;118:e235-e244. doi:10.1016/j.wneu.2018.06.166
20. Tobin MK, Geraghty JR, Engelhard HH, Linninger AA, Mehta AI. Intramedullary spinal cord tumors: a review of current and future treatment strategies. Neurosurg Focus. 2015;39(2):E14. doi:10.3171/2015.5.FOCUS15158

Original Version of the Topic

Tommy C. Yu, MD, Sunlung Suen, MD. Spinal tumors. 7/11/2013.

Previous Revision(s) of the Topic

Tommy C. Yu, MD. Spinal tumors. 8/22/2016.

Tommy C. Yu, MD, Matthew Weinstein, DO. Spinal Tumors. 5/24/2021

Author Disclosures

Mitra McLarney, MD
Nothing to Disclose

Courtney Gilbert, MD
Nothing to Disclose