Spinal tumors are neoplasms located in either the vertebral column or the spinal cord. There are three main types of spinal tumors classified based on their location: extradural and intradural (intradural-intramedullary and intradural-extramedullary). Extradural tumors are located outside the dura mater lining and are most commonly metastatic. Intradural tumors are located inside the dura mater lining and are further subdivided into intramedullary and extramedullary tumors. Intradural-intramedullary tumors are located within the dura and spinal cord parenchyma, while intradural-extramedullary tumors are located within the dura but outside the spinal cord parenchyma. The most common presenting symptom of spinal tumors is nocturnal back pain. Other common symptoms include

muscle Skeletal muscles (commonly referred to as muscles) are organs of the vertebrate muscular system and typically are attached by tendons to bones of a skeleton. The muscle cells of skeletal muscles are much longer than in the other types of muscl ...

weakness, sensory loss, and difficulty walking. Loss of bowel and bladder control may occur during the later stages of the disease. The cause of spinal tumors is unknown. Most extradural tumors are metastatic commonly from breast, prostate, lung, and kidney cancer. There are many genetic factors associated with intradural tumors, most commonly neurofibromatosis 1 (NF1),

neurofibromatosis 2 Neurofibromatosis type II (also known as MISME syndrome – multiple inherited schwannomas, meningiomas, and ependymomas) is a genetic condition that may be inherited or may arise spontaneously, and causes benign tumors of the brain, spinal cord, ...

(NF2), and Von-Hippel Lindau (VHL) syndrome. The most common type of intradural-extramedullary tumors are

meningioma Meningioma, also known as meningeal tumor, is typically a slow-growing tumor that forms from the meninges, the membranous layers surrounding the brain and spinal cord. Symptoms depend on the location and occur as a result of the tumor pressing o ...

s and nerve-sheath tumors. The most common type of intradural-intramedullary tumors are ependymomas and astrocytomas. Diagnosis involves a complete medical evaluation followed by imaging with a CT or MRI. A biopsy may be obtained in certain cases to categorize the lesion if the diagnosis is uncertain. Treatment often involves some combination of surgery, radiation, and chemotherapy. Observation with follow-up imaging may be an option for small, benign lesions. Steroids may also be given before surgery in cases of significant cord compression. Outcomes depend on a number of factors including whether the tumor is benign or malignant, primary or metastatic, and location of the tumor. Treatment is often

palliative Palliative care (derived from the Latin root , or 'to cloak') is an interdisciplinary medical caregiving approach aimed at optimizing quality of life and mitigating suffering among people with serious, complex, and often terminal illnesses. Wit ...

for the vast majority of metastatic tumors.

Signs and symptoms

The symptoms of spinal tumors are often non-specific, resulting in a delay in diagnosis. Spinal nerve compression and weakening of the vertebral structure cause the symptoms. Pain is the most common symptom at presentation. Other common symptoms of

spinal cord compression Spinal cord compression is a form of myelopathy in which the spinal cord is compressed. Causes can be bone fragments from a vertebral fracture, a tumor, abscess, ruptured intervertebral disc or other lesion. When acute it can cause a medical ...

include muscle weakness,

sensory loss Many types of sense loss occur due to a dysfunctional sensation process, whether it be ineffective receptors, nerve damage, or cerebral impairment. Unlike agnosia, these impairments are due to damages prior to the perception process. Vision loss ...

, numbness in hands and legs, and rapid onset

paralysis Paralysis (also known as plegia) is a loss of motor function in one or more muscles. Paralysis can also be accompanied by a loss of feeling (sensory loss) in the affected area if there is sensory damage. In the United States, roughly 1 in 50 ...

. Bowel or

bladder incontinence Urinary incontinence (UI), also known as involuntary urination, is any uncontrolled leakage of urine. It is a common and distressing problem, which may have a large impact on quality of life. It has been identified as an important issue in geria ...

often occur in the later stages of the disease. Children may present with spinal deformities such as

scoliosis Scoliosis is a condition in which a person's spine has a sideways curve. The curve is usually "S"- or "C"-shaped over three dimensions. In some, the degree of curve is stable, while in others, it increases over time. Mild scoliosis does not t ...

. The diagnosis is challenging, primarily because symptoms often mimic more common and benign degenerative spinal diseases. Spinal cord compression is commonly found in patients with metastatic malignancy. Back pain is a primary symptom of spinal cord compression in patients with known malignancy. Back pain may prompt a

bone scan A bone scan or bone scintigraphy is a nuclear medicine imaging technique of the bone. It can help diagnose a number of bone conditions, including cancer of the bone or metastasis, location of bone inflammation and fractures (that may not be visi ...

to confirm or exclude spinal metastasis. Rapid identification and intervention of metastatic spinal cord compression is necessary to preserve neurologic function.

Causes

The cause of the majority of spinal tumors is currently not known. Primary spinal tumors are associated with a few

genetic syndromes A syndrome is a set of medical signs and symptoms which are correlated with each other and often associated with a particular disease or disorder. The word derives from the Greek σύνδρομον, meaning "concurrence". When a syndrome is paired ...

. Neurofibromas are associated with neurofibromatosis 1 (NF1). Meningiomas and schwannomas are associated with neurofibromatosis 2 (NF2). Intramedullary hemangioblastomas can be seen in patients with von Hippel-Lindau disease. Spinal cord lymphomas are commonly seen in patients with suppressed immune systems. The majority of extradural tumors are due to metastasis, most commonly from breast, prostate, lung, and kidney cancer.

Pathophysiology

The spinal cord is a long, cylindrical anatomical structure that is located within the vertebral cavity. It runs from the

foramen magnum The foramen magnum ( la, great hole) is a large, oval-shaped opening in the occipital bone of the skull. It is one of the several oval or circular openings (foramina) in the base of the skull. The spinal cord, an extension of the medulla oblon ...

of the skull to the conus medullaris at the lumbar spine. Most symptoms from spinal tumors occur due to compression of the spinal cord as it plays a primary role in motor and sensory function. The spinal cord is surrounded by three layers known as the spinal

meninges In anatomy, the meninges (, ''singular:'' meninx ( or ), ) are the three membranes that envelop the brain and spinal cord. In mammals, the meninges are the dura mater, the arachnoid mater, and the pia mater. Cerebrospinal fluid is located in th ...

. These are the dura mater,

arachnoid mater The arachnoid mater (or simply arachnoid) is one of the three meninges, the protective membranes that cover the brain and spinal cord. It is so named because of its resemblance to a spider web. The arachnoid mater is a derivative of the neural c ...

, and pia mater. Spinal cord tumors are classified based on their location within the spinal cord: intradural (intradmedullary and extramedullary) and extradural tumors. Intradural tumors are located within the dura mater. These are further broken down into intramedullary and extramedullary tumors. Intradural-intramedullary tumors are located within the spinal cord itself, with the most common being ependymomas, astrocytomas, and hemangioblastomas. Intradural-extramedullary tumors are located within the dura but outside of the spinal cord parenchyma, with the most common being

s and nerve sheath tumors (e.g. schwannomas, neurofibromas). Extradural tumors are located outside the dura mater most commonly in the vertebral bodies from metastatic disease. Common primary cancers in metastatic spinal tumors includes breast, prostate, lung, and kidney cancer. It is important to diagnose and promptly treat metastatic tumors as they can lead to long-term neurologic deficit from epidural

. Primary extradural tumors are rare and most arise from surrounding bony and soft tissue structures, including Ewing's sarcoma, osteosarcoma, and vertebral hemangioblastomas.

Diagnosis

Medical Examination

The diagnosis of spinal tumors is challenging, as the symptoms can be non-specific and often mimic more common and benign degenerative spinal diseases. A comprehensive medical examination is necessary to look for signs or symptoms that may point towards a more serious condition. This includes a complete neurological exam focusing on any motor or sensory deficits. Patients with either benign degenerative spinal disease or spinal tumors often present with back pain. A patient with

radiculopathy Radiculopathy, also commonly referred to as pinched nerve, refers to a set of conditions in which one or more nerves are affected and do not work properly (a neuropathy). Radiculopathy can result in pain (radicular pain), weakness, altered sensati ...

or myelopathy raises suspicion for a more serious condition.

Imaging

Imaging is often the next step when the diagnosis is unclear or there is greater suspicion for a serious condition that may need immediate intervention. Common types of medical imaging include X-rays, computer tomography scan (CT),

Magnetic resonance imaging Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio wave ...

(MRI), myelography, and bone scans. MRI is the imaging of choice for spinal tumors. The MRI protocol that is most frequently used includes

T1-weighted Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio waves ...

and

T2-weighted Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. Physics of magnetic resonance imaging#MRI scanner, MRI scanners use strong magnetic ...

sequences, including contrast enhanced T1-weighted sequences. Short-TI Inversion Recovery (STIR) is also commonly added to the MRI protocol for detecting spinal cord tumors. Myelography may be used as a substitute when the patient cannot undergo an MRI or it is unavailable. X-rays and CT are more commonly used to view the bony structures. They are less frequently used for spinal cord tumors, however, since they cannot reliably detect them.

Bone scan A bone scan or bone scintigraphy is a nuclear medicine imaging technique of the bone. It can help diagnose a number of bone conditions, including cancer of the bone or metastasis, location of bone inflammation and fractures (that may not be visi ...

ning may be used as a supplementary imaging modality for tumors involving bony structures of the spine.

Treatment

Treatment greatly varies depending on the type of spinal cord tumors, goals of care, and prognosis. The primary forms of treatment include surgical resection, radiotherapy, and chemotherapy.

Steroids A steroid is a biologically active organic compound with four rings arranged in a specific molecular configuration. Steroids have two principal biological functions: as important components of cell membranes that alter membrane fluidity; and a ...

(e.g.

corticosteroids Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are involve ...

) may be administered if there is evidence of

. These do not affect the tumor mass itself, but tend to reduce the inflammatory reaction around it and decrease the overall volume of the mass impinging on the spinal cord.

Surgery

Surgery has several indications depending on the type of tumor, which includes complete resection, decompression of the nerves, and stabilization. An attempt at total gross resection for a possible cure is an option for patients with primary spinal cord tumors. Extramedullary tumours are more amenable to resection than intramedullary tumours, and even possible to be operated through microendoscopic or pure endoscopic approaches. In patients with metastatic tumors, treatment is palliative with the goal of improving the patient's quality of life. In these cases, indications for surgery include pain, stabilization, and spinal cord decompression.

Non-Surgical Treatment

Observation, chemotherapy, and

radiotherapy Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radia ...

are possible options as an adjunct to surgery or for tumors not amenable to surgery. Intradural-extramedullary tumors are often benign, so observation with follow-up imaging is an option in cases where the lesions are small and the patient is asymptomatic. Radiotherapy and chemotherapy may be administered alone or in conjunction with surgery. The choice of chemotherapy or radiotherapy is a multidisciplinary process and depends on the histological grade, type of tumor, and amount of surgical resection achieved. In cases where radiotherapy is chosen, radiation is usually delivered to the involved segment in the spinal cord and the uninvolved segment above and below the involved segment. The combination of

minimally invasive surgery Minimally invasive procedures (also known as minimally invasive surgeries) encompass surgical techniques that limit the size of incisions needed, thereby reducing wound healing time, associated pain, and risk of infection. Surgery by definition ...

and

radiation In physics, radiation is the emission or transmission of energy in the form of waves or particles through space or through a material medium. This includes: * ''electromagnetic radiation'', such as radio waves, microwaves, infrared, visi ...

or chemotherapy is a new technique for treating spinal tumors. This treatment can be tailored to the particular tumor of the

spine Spine or spinal may refer to: Science Biology * Vertebral column, also known as the backbone * Dendritic spine, a small membranous protrusion from a neuron's dendrite * Thorns, spines, and prickles, needle-like structures in plants * Spine (zoolog ...

, either metastatic or primary. Some suggest that direct decompressive surgery combined with postoperative radiotherapy, provide better outcomes than treatment with radiotherapy alone for patients with spinal cord compression due to metastatic cancer.

References

External links

{{DEFAULTSORT:Spinal Tumor Nervous system neoplasia