Spinal tumors are

neoplasm A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

s located in either the

vertebral column The vertebral column, also known as the backbone or spine, is part of the axial skeleton. The vertebral column is the defining characteristic of a vertebrate in which the notochord (a flexible rod of uniform composition) found in all chordate ...

or the

spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the sp ...

. 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 In neuroanatomy, dura mater is a thick membrane made of dense irregular connective tissue that surrounds the brain and spinal cord. It is the outermost of the three layers of membrane called the meninges that protect the central nervous system. ...

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 mus ...

weakness, sensory loss, and difficulty walking. Loss of bowel and

bladder The urinary bladder, or simply bladder, is a hollow organ in humans and other vertebrates that stores urine from the kidneys before disposal by urination. In humans the bladder is a distensible organ that sits on the pelvic floor. Urine en ...

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 (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 ...

s and nerve-sheath tumors. The most common type of intradural-intramedullary tumors are

ependymoma An ependymoma is a tumor that arises from the ependyma, a tissue of the central nervous system. Usually, in pediatric cases the location is intracranial, while in adults it is spinal. The common location of intracranial ependymomas is the fourth ...

s 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 medi ...

include muscle weakness, sensory 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 5 ...

. 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 ty ...

. 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 Back pain is pain felt in the back. It may be classified as neck pain (cervical), middle back pain (thoracic), lower back pain (lumbar) or coccydynia (tailbone or sacral pain) based on the segment affected. The lumbar area is the most common ...

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 ...

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

lymphoma Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). In current usage the name usually refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include en ...

s 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

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 ''Conus'' is a genus of predatory sea snails, or cone snails, marine gastropod mollusks in the family Conidae.Bouchet, P.; Gofas, S. (2015). Conus Linnaeus, 1758. In: MolluscaBase (2015). Accessed through: World Register of Marine Species at ...

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 ...

. These are the

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 neura ...

, and

pia mater Pia mater ( or ),Entry "pia mater"
in
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

s, 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 An osteosarcoma (OS) or osteogenic sarcoma (OGS) (or simply bone cancer) is a cancerous tumor in a bone. Specifically, it is an aggressive malignant neoplasm that arises from primitive transformed cells of mesenchymal origin (and thus a sarcoma ...

, 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 sen ...

myelopathy Myelopathy describes any neurologic deficit related to the spinal cord. The most common form of myelopathy in humans, '' cervical spondylotic myelopathy (CSM)'', also called ''degenerative cervical myelopathy'', results from narrowing of the spin ...

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-ray An X-ray, or, much less commonly, X-radiation, is a penetrating form of high-energy electromagnetic radiation. Most X-rays have a wavelength ranging from 10 picometers to 10 nanometers, corresponding to frequencies in the range 30&nb ...

s, 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 and T2-weighted 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 ...

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 ...

(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 invol ...

) 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 Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs (chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemother ...

, 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. Rad ...

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 definitio ...

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 ...

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 (zoolo ...

, 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