Spinal Tumors – What you need to know!

A spinal tumor is an abnormal mass of tissue within or surrounding the spinal cord and/or spinal column. These cells grow and multiply uncontrollably, seemingly unchecked by the mechanisms that control normal cells. Spinal tumors can be benign (non-cancerous) or malignant (cancerous). Primary tumors originate in the spine or spinal cord, and metastatic or secondary tumors result from cancer spreading from another site to the spine.

Primary spinal tumors originate in the spinal column, such as by growing in the bones, discs, nerves, or other elements within the spine. Primary spinal tumors are usually noncancerous (benign) and occur in younger people. Hemangiomas are among the most common benign primary spinal tumors. Osteosarcomas and multiple myeloma are two of the more common primary spinal tumors that are cancerous (malignant).

Secondary spinal tumors (metastatic tumors) are tumors that have spread to the spine from a cancer that has started elsewhere in the body. These tumors are the most common type in the spine and are cancerous (have the potential to spread further and are typically fast-growing). Cancerous cells from the lung, breast, and prostate are most likely to spread and become lodged in the spine.

Spinal tumors are referred to in two ways.

1. By the region of the spine in which they occur. These basic areas are cervical, thoracic, lumbar and sacrum.
2. By their location within the spine.
   • Intradural-extramedullary – The tumor is located inside the thin covering of the spinal cord (the dura), but outside the actual spinal cord. Frequency of occurrence in this location is 40%. The most common of these types of tumors develop in the spinal cord’s arachnoid membrane (meningiomas), in the nerve roots that extend out from the spinal cord (schwannomas and neurofibromas), or at the spinal cord base (filum terminale ependymomas). Although meningiomas are often benign, they can be difficult to remove and may recur. Nerve root tumors are also generally benign, although neurofibromas may become malignant over time. Ependymomas at the end of the spinal cord can be large, and the delicate nature of fine neural structures in that area may make removal difficult.
   • Intramedullary – These tumors grow inside the spinal cord. They typically derive from glial or ependymal cells (a type of glial cell) that are found throughout the interstitium of the spinal cord. Frequency of occurrence in this location is approximately 5%. Astrocytomas and ependymomas are the two most common types. Astrocytomas are more common in the thoracic region followed by the cervical. Ependymomas are most common in the filum (bottom region of the spinal cord), followed by the cervical region. They are often benign (compared to intracranial), but can be difficult to remove. Intramedullary lipomas are rare congenital tumors most commonly located in the cervicothoracic spinal cord.
   • Extradural – The tumor is located outside the dura, which is the thin covering surrounding the spinal cord. Frequency of occurrence in this location vs the ones above is approximately 55%. These lesions are typically attributed to metastatic cancer or less commonly schwannomas derived from the cells covering the nerve roots. Occasionally, an extradural tumor extends through the intervertebral foramina, lying partially within and partially outside of the spinal canal.

The bony spinal column is the most common site for bone metastasis. Estimates indicate that at least 30% and as high as 70% of patients with cancer will experience spread of cancer to their spine. The most common primary spine tumor (originated in the bony spine) is vertebral hemangiomas. These are benign lesions and rarely cause symptoms such as pain.

Common primary cancers that spread to the spine are lung, breast and prostate. Lung cancer is the most common cancer to metastasize to the bone in men, and breast cancer is the most common in women. Other cancers that spread to the spine include multiple myeloma, lymphoma, melanoma and sarcoma, as well as cancers of the gastrointestinal tract, kidney and thyroid. Prompt diagnosis and identification of the primary malignancy is crucial to overall treatment. Numerous factors can affect outcome, including the nature of the primary cancer, the number of lesions, the presence of distant non-skeletal metastases and the presence and/or severity of spinal-cord compression.

Pediatric Spinal Tumors

• Osteoid Osteoma
• Osteoblastoma
• Osteochondroma
• Osteosarcoma
• Ewing Sarcoma
• Eosinophilic Granuloma
• Aneurysmal Bone Cyst
• Ahordoma
• Mesenchymal Chondrosarcoma
• Giant-Cell Tumor Of Bone
• Fibrous Dysplasia
• Fibroma
• Angiosarcoma
• Hemangioma

Unlike adults, children have not achieved complete skeletal growth, which doctors must take into account when considering treatment. Other factors to consider are spinal stability, surgical versus nonsurgical interventions and preservation of neurological function.

Incidence and Prevalence

Intracranial (brain) tumors account for 85-90% of all primary central nervous system (CNS) tumors. Primary tumors arising from the spinal cord, spinal nerve roots and dura are rare compared to CNS tumors that arise in the brain. Overall prevalence is estimated at one spinal tumor for every four intracranial lesions.

When speaking of tumors of the spine, there are different incidences of tumor types related to the neural/dural elements vs the surrounding vertebral bony support. Intramedullary tumors are rare compared to metastatic tumors of the spine. Vertebral hemangiomas are the most common benign bony primary spinal tumor. Spinal epidural metastases are the most common type of spinal tumor. They occur in up to 10% of cancer patients. Metastatic spinal tumors typically take hold in the bony spine and then grow to cause compression of the neural elements (spinal cord and nerve roots).

Causes

The cause of most primary spinal tumors is unknown. Some of them may be attributed to exposure to cancer-causing agents. Spinal cord lymphomas, which are cancers that affect lymphocytes (a type of immune cell), are more common in people with compromised immune systems. There appears to be a higher incidence of spinal tumors in particular families, so there is most likely a genetic component.

In a small number of cases, primary tumors may result from presence of these two genetic diseases:

Neurofibromatosis 2: In this hereditary (genetic) disorder, benign tumors may develop in the arachnoid layer of the spinal cord or in the supporting glial cells. However, the more common tumors associated with this disorder affect the nerves related to hearing and can inevitably lead to loss of hearing in one or both ears.

Von Hippel-Lindau disease: This rare, multi-system disorder is associated with benign blood vessel tumors (hemangioblastomas) in the brain, retina and spinal cord, and with other types of tumors in the kidneys or adrenal glands.

Symptoms

Non-mechanical back pain, especially in the middle or lower back, is the most frequent symptom of both benign and malignant spinal tumors. This back pain is not specifically attributed to injury, stress or physical activity. However, the pain may increase with activity and can be worse at night when lying down. Pain may spread beyond the back to the hips, legs, feet or arms and may worsen over time — even when treated by conservative, nonsurgical methods that can often help alleviate back pain attributed to mechanical causes. Depending on the location and type of tumor, other signs and symptoms can develop, especially as a tumor grows and compresses on the spinal cord, the nerve roots, blood vessels or bones of the spine.

Additional symptoms can include the following:

• Loss of sensation or muscle weakness in the legs, arms or chest
• Stiff neck or back
• Pain and/or neurologic symptoms (such as tingling) increase with Valsalva maneuver
• Difficulty walking, which may cause falls
• Decreased sensitivity to pain, heat and cold
• Loss of bowel or bladder function
• Paralysis that may occur in varying degrees and in different parts of the body, depending on which nerves are compressed
• Scoliosis or other spinal deformity resulting from a large and/or destructive tumor

Testing & Diagnosis

A thorough medical examination with emphasis on back pain and neurological deficits is the first step to diagnosing a spinal tumor. Radiological tests are required for an accurate and positive diagnosis.

• X-ray: Application of radiation to produce a film or picture of a part of the body can show the structure of the vertebrae and the outline of the joints. X-rays of the spine are obtained to search for other potential causes of pain, i.e. tumors, infections, fractures, etc. X-rays, however, are not very reliable in diagnosing tumors.
• Computed tomography scan (CT or CAT scan): A diagnostic image created after a computer reads X-rays, a CT/CAT scan can show the shape and size of the spinal canal, its contents and the structures around it. It also is very good at visualizing bony structures.
• Magnetic resonance imaging (MRI): A diagnostic test that produces three-dimensional images of body structures using powerful magnets and computer technology. An MRI can show the spinal cord, nerve roots and surrounding areas, as well as enlargement, degeneration and tumors.
• Bone Scan: A diagnostic test using Technectium-99. Helpful as an adjunct for identification of bone tumors (such as primary bone tumors of the spine), infection, and diseases involving abnormal bone metabolism.

Radiology studies noted above provide imaging findings that suggest the most likely tumor type. In some cases, however, a biopsy may be needed if diagnosis is unclear or if concern for malignancy vs benign tumor type. If the tumor is malignant, a biopsy also helps determine the cancer’s type, which subsequently determines treatment options.

Staging classifies neoplasms (abnormal tissue) according to the extent of the tumor, assessing bony, soft tissue and spinal canal involvement. A doctor may order a whole body scan utilizing nuclear technology, as well as a CT scan of the lungs and abdomen for staging purposes. To confirm diagnosis, a doctor compares laboratory test results and findings from the aforementioned scans to the patient’s symptoms.

Treatment

Treatment decision-making is often multidisciplinary, incorporating the expertise of spinal surgeons, medical oncologists, radiation oncologists and other medical specialists. The selection of treatments including both surgical and non-surgical is therefore made keeping in mind the various aspects of the patient’s overall health and goals of care.

Non-Surgical Treatments

Nonsurgical treatment options include observation, chemotherapy and radiation therapy. Tumors that are asymptomatic or mildly symptomatic and do not appear to be changing or progressing may be observed and monitored with regular MRIs. Some tumors respond well to chemotherapy and others to radiation therapy. However, there are specific types of metastatic tumors that are inherently radioresistant (i.e. gastrointestinal tract and kidney): in those cases, surgery may be the only viable treatment option.

Surgery

Indications for surgery vary depending on the type of tumor. Primary (non-metastatic) spinal tumors may be removed through complete en bloc resection for a possible cure. In patients with metastatic tumors, treatment is primarily palliative, with the goal of restoring or preserving neurological function, stabilizing the spine and alleviating pain. Generally, surgery is only considered as an option for patients with metastases when they are expected to live 3 – 4 months or longer, and the tumor is resistant to radiation or chemotherapy. Indications for surgery include intractable pain, spinal-cord compression and the need for stabilization of pathological fractures.

For cases in which surgical resection is possible, preoperative embolization may be used to enable an easier resection. This procedure involves the insertion of a catheter or tube through an artery in the groin. The catheter is guided up through the blood vessels to the site of the tumor, where it delivers a glue-like liquid embolic agent that blocks the vessels that feed the tumor. When the blood vessels that feed the tumor are blocked off, bleeding can often be controlled better during surgery, helping to decrease surgical risks.

If surgery is considered, the approach to the tumor is determined by the tumor’s location within the spinal canal.  The posterior (back) approach allows for the identification of the dura and exposure of the nerve roots. This approach is commonly used for tumors in the posterior aspect of the spinal column or to expose tumors inside the dura.   Multiple levels can be decompressed, and multilevel segmental  fixation can be performed if necessary. The anterior (front) approach is excellent for tumors in the front of the spine. This approach also allows for the reconstruction of defects caused by removal of the vertebral bodies. This approach also allows placement of short-segment fixation devices. Thoracic and lumbar spinal tumors that affect both the anterior and posterior vertebral columns can be a challenge to resect completely. Not infrequently, a posterior (back) approach followed by a separately staged anterior (front) approach has been utilized surgically to treat these complex lesions.

Outlook

Outcome depends greatly on the age and overall health of the patient and on whether the spinal tumor is benign or malignant, primary or metastatic. In the case of primary tumors, the goal is to remove the tumor completely, leading optimally to the potential cure of the malignancy. In some primary tumor types (particularly those that arise inside the spinal cord-intramedullary) complete resection is not possible without significant neural damage.  In the case of metastatic tumors, the goal is almost always palliative, with treatment aimed at providing the patient with an improved quality of life and possibly prolonged life expectancy.  Treatment advances related to spine stereotactic body radiotherapy (SBRT) in combination with spinal epidural decompression (as indicated) have the opportunity to provide greater control of metastatic disease of the spine in certain patients. 

Surgery in adults for a variety of spinal tumor types has been associated with a risk for major complications (reports of up to 14%).  The most common complications are surgical site infection, systemic infections, and deep venous thrombosis.  Please note that post-operative complications are more common in patients who have significant co-morbidities (such as steroid use).  

Resources for More Information.

• KnowYourBack.org. (2019). Spinal Tumors.
• NIH. (2019). Brain and Spinal Tumors Information Page.
• Spine Tumors
• Spinal Tumors. American Association of Neurological Surgeons. https://www.aans.org/Patients/Neurosurgical-Conditions-and-Treatments/Spinal-Tumors. Visited October 25, 2019.
• Ciftdemir M, Kaya M, Selcuk E, Yalniz E. Spinal tumors of the spine. World J Orthop. 2016; 7(2): 109-116. doi: 10.5312/wjo.v7.i2.109.
• Ziu E, Mesfin FB. Cancer, Spinal Metastasis. [Updated 2019 Apr 23]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK441950/.
• Sun T, Plutynski A, Ward S, Rubin JB. Cell Mol Life Sci. 2015; 72(17):3323-42.
• 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: https://doi.org/10.3171/2015.5.FOCUS15158.