Spinal tumor

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Spinal tumors are neoplasms located in either the vertebral column or the spinal cord. [1] 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. [2] 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. [1] Other common symptoms include muscle weakness, sensory loss, and difficulty walking. [1] Loss of bowel and bladder control may occur during the later stages of the disease. [3]

Contents

The cause of spinal tumors is unknown. Most extradural tumors are metastatic commonly from breast, prostate, lung, and kidney cancer. [4] There are many genetic factors associated with intradural tumors, most commonly neurofibromatosis 1 (NF1), neurofibromatosis 2 (NF2), and Von-Hippel Lindau (VHL) syndrome. [1] The most common type of intradural-extramedullary tumors are meningiomas and nerve-sheath tumors. [5] The most common type of intradural-intramedullary tumors are ependymomas and astrocytomas. [6] 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. [1] [4] [5]

Treatment often involves some combination of surgery, radiation, and chemotherapy. [1] [4] [5] Observation with follow-up imaging may be an option for small, benign lesions. [4] Steroids may also be given before surgery in cases of significant cord compression. [1] 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 for the vast majority of metastatic tumors. [5]

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. [1] [3] [7] Other common symptoms of spinal cord compression include muscle weakness, sensory loss, numbness in hands and legs, and rapid onset paralysis. Bowel or bladder incontinence often occur in the later stages of the disease. [3] Children may present with spinal deformities such as scoliosis. [1] [2] The diagnosis is challenging, primarily because symptoms often mimic more common and benign degenerative spinal diseases. [8] [9]

Spinal cord compression is commonly found in patients with metastatic malignancy. [10] Back pain is a primary symptom of spinal cord compression in patients with known malignancy. [11] Back pain may prompt a bone scan to confirm or exclude spinal metastasis. Rapid identification and intervention of metastatic spinal cord compression is necessary to preserve neurologic function. [12]

Causes

The cause of the majority of spinal tumors is currently not known. Primary spinal tumors are associated with a few genetic syndromes. [1] [5] Neurofibromas are associated with neurofibromatosis 1 (NF1). [1] Meningiomas and schwannomas are associated with neurofibromatosis 2 (NF2). [1] Intramedullary hemangioblastomas can be seen in patients with von Hippel-Lindau disease. [5] Spinal cord lymphomas are commonly seen in patients with suppressed immune systems. [5] The majority of extradural tumors are due to metastasis, most commonly from breast, prostate, lung, and kidney cancer. [4]

Pathophysiology

The spinal cord is a long, cylindrical anatomical structure that is located within the vertebral cavity. It runs from the foramen magnum of the skull to the conus medullaris at the lumbar spine. [1] Most symptoms from spinal tumors occur due to compression of the spinal cord as it plays a primary role in motor and sensory function. [13] The spinal cord is surrounded by three layers known as the spinal meninges. [13] These are the dura mater, arachnoid mater, and pia mater. Spinal cord tumors are classified based on their location within the spinal cord: intradural (intradmedullary and extramedullary) and extradural tumors.[ citation needed ]

Intradural tumors are located within the dura mater. [1] 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. [14] [6] [7] Intradural-extramedullary tumors are located within the dura but outside of the spinal cord parenchyma, with the most common being meningiomas and nerve sheath tumors (e.g. schwannomas, neurofibromas). [5] [14] [7] Extradural tumors are located outside the dura mater most commonly in the vertebral bodies from metastatic disease. [1]

Common primary cancers in metastatic spinal tumors includes breast, prostate, lung, and kidney cancer. [1] [4] [8] It is important to diagnose and promptly treat metastatic tumors as they can lead to long-term neurologic deficit from epidural spinal cord compression. [1] Primary extradural tumors are rare and most arise from surrounding bony and soft tissue structures, including Ewing's sarcoma, osteosarcoma, and vertebral hemangioblastomas. [1]

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. [5] Patients with either benign degenerative spinal disease or spinal tumors often present with back pain. A patient with radiculopathy or myelopathy raises suspicion for a more serious condition. [15]

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 (MRI), myelography, and bone scans. [1] [5] MRI is the imaging of choice for spinal tumors. [1] The MRI protocol that is most frequently used includes T1-weighted and T2-weighted sequences, including contrast enhanced T1-weighted sequences. [1] Short-TI Inversion Recovery (STIR) is also commonly added to the MRI protocol for detecting spinal cord tumors. [1] Myelography may be used as a substitute when the patient cannot undergo an MRI or it is unavailable. [1] X-rays and CT are more commonly used to view the bony structures. [5] They are less frequently used for spinal cord tumors, however, since they cannot reliably detect them. [5] [16] Bone scanning may be used as a supplementary imaging modality for tumors involving bony structures of the spine. [5]

Treatment

Treatment greatly varies depending on the type of spinal cord tumors, goals of care, and prognosis. [5] The primary forms of treatment include surgical resection, radiotherapy, and chemotherapy. [5] Steroids (e.g. corticosteroids) [9] may be administered if there is evidence of spinal cord compression. 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.[ citation needed ]

Surgery

Surgery has several indications depending on the type of tumor, which includes complete resection, decompression of the nerves, and stabilization. [4] An attempt at total gross resection for a possible cure is an option for patients with primary spinal cord tumors. [4] Extramedullary tumours are more amenable to resection than intramedullary tumours, and even possible to be operated through microendoscopic or pure endoscopic approaches. [17] [7] In patients with metastatic tumors, treatment is palliative with the goal of improving the patient's quality of life. [5] In these cases, indications for surgery include pain, stabilization, and spinal cord decompression. [5]

Non-Surgical Treatment

Observation, chemotherapy, and radiotherapy 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. [4] 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. [4] 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. [9]

The combination of minimally invasive surgery and radiation or chemotherapy is a new technique for treating spinal tumors. [18] This treatment can be tailored to the particular tumor of the spine, either metastatic or primary. [19] 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. [20] [21]

Related Research Articles

<span class="mw-page-title-main">Brain tumor</span> Neoplasm in the brain

A brain tumor occurs when abnormal cells form within the brain. There are two main types of tumors: malignant tumors and benign (non-cancerous) tumors. These can be further classified as primary tumors, which start within the brain, and secondary tumors, which most commonly have spread from tumors located outside the brain, known as brain metastasis tumors. All types of brain tumors may produce symptoms that vary depending on the size of the tumor and the part of the brain that is involved. Where symptoms exist, they may include headaches, seizures, problems with vision, vomiting and mental changes. Other symptoms may include difficulty walking, speaking, with sensations, or unconsciousness.

<span class="mw-page-title-main">Prostate cancer</span> Male reproductive organ cancer

Prostate cancer is the uncontrolled growth of cells in the prostate, a gland in the male reproductive system just below the bladder. Early prostate cancer usually causes no symptoms. As the cancer develops, one or more tumors can damage nearby organs causing erectile dysfunction, blood in the urine or semen, and trouble urinating. For some patients, the cancer eventually spread to other areas of the body, particularly the bones and lymph nodes. There, tumors cause severe bone pain, leg weakness or paralysis, and eventually death.

<span class="mw-page-title-main">Sarcoma</span> Medical condition

A sarcoma is a malignant tumor, a type of cancer that arises from cells of mesenchymal origin. Connective tissue is a broad term that includes bone, cartilage, fat, vascular, or other structural tissues, and sarcomas can arise in any of these types of tissues. As a result, there are many subtypes of sarcoma, which are classified based on the specific tissue and type of cell from which the tumor originates. Sarcomas are primary connective tissue tumors, meaning that they arise in connective tissues. This is in contrast to secondary connective tissue tumors, which occur when a cancer from elsewhere in the body spreads to the connective tissue. Sarcomas are one of five different types of cancer, classified by the cell type from which they originate. The word sarcoma is derived from the Greek σάρκωμα sarkōma 'fleshy excrescence or substance', itself from σάρξsarx meaning 'flesh'.

<span class="mw-page-title-main">Bone tumor</span> Medical condition

A bone tumor is an abnormal growth of tissue in bone, traditionally classified as noncancerous (benign) or cancerous (malignant). Cancerous bone tumors usually originate from a cancer in another part of the body such as from lung, breast, thyroid, kidney and prostate. There may be a lump, pain, or neurological signs from pressure. A bone tumor might present with a pathologic fracture. Other symptoms may include fatigue, fever, weight loss, anemia and nausea. Sometimes there are no symptoms and the tumour is found when investigating another problem.

<span class="mw-page-title-main">Ependymoma</span> Medical condition

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 ventricle. Rarely, ependymomas can occur in the pelvic cavity.

<span class="mw-page-title-main">Meningioma</span> Type of tumor

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 on nearby tissue. Many cases never produce symptoms. Occasionally seizures, dementia, trouble talking, vision problems, one sided weakness, or loss of bladder control may occur.

<span class="mw-page-title-main">Plasmacytoma</span> Growth of a plasma cell tumour within soft tissue or the axial skeleton

Plasmacytoma is a plasma cell dyscrasia in which a plasma cell tumour grows within soft tissue or within the axial skeleton.

<span class="mw-page-title-main">Ganglioglioma</span> Medical condition

Ganglioglioma is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently occurs in the temporal lobes of children and young adults

<span class="mw-page-title-main">Spinal cord compression</span> Medical condition

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.

<span class="mw-page-title-main">Diastematomyelia</span> Medical condition

Diastematomyelia is a congenital disorder in which a part of the spinal cord is split, usually at the level of the upper lumbar vertebra in the longitudinal (sagittal) direction. Females are affected much more commonly than males. This condition occurs in the presence of an osseous, cartilaginous or fibrous septum in the central portion of the spinal canal which then produces a complete or incomplete sagittal division of the spinal cord into two hemicords. When the split does not reunite distally to the spur, the condition is referred to as diplomyelia, which is true duplication of the spinal cord.

<span class="mw-page-title-main">Leptomeningeal cancer</span> Medical condition

Leptomeningeal cancer is a rare complication of cancer in which the disease spreads from the original tumor site to the meninges surrounding the brain and spinal cord. This leads to an inflammatory response, hence the alternative names neoplastic meningitis (NM), malignant meningitis, or carcinomatous meningitis. The term leptomeningeal describes the thin meninges, the arachnoid and the pia mater, between which the cerebrospinal fluid is located. The disorder was originally reported by Eberth in 1870. It is also known as leptomeningeal carcinomatosis, leptomeningeal disease (LMD), leptomeningeal metastasis, meningeal metastasis and meningeal carcinomatosis.

<span class="mw-page-title-main">Bone metastasis</span> Medical condition

Bone metastasis, or osseous metastatic disease, is a category of cancer metastases that result from primary tumor invasions into bones. Bone-originating primary tumors such as osteosarcoma, chondrosarcoma, and Ewing sarcoma are rare; the most common bone tumor is a metastasis. Bone metastases can be classified as osteolytic, osteoblastic, or both. Unlike hematologic malignancies which originate in the blood and form non-solid tumors, bone metastases generally arise from epithelial tumors and form a solid mass inside the bone. Bone metastases, especially in a state of advanced disease, can cause severe pain, characterized by a dull, constant ache with periodic spikes of incident pain.

<span class="mw-page-title-main">Metastatic breast cancer</span> Type of cancer

Metastatic breast cancer, also referred to as metastases, advanced breast cancer, secondary tumors, secondaries or stage IV breast cancer, is a stage of breast cancer where the breast cancer cells have spread to distant sites beyond the axillary lymph nodes. There is no cure for metastatic breast cancer; there is no stage after IV.

<span class="mw-page-title-main">Brain metastasis</span> Cancer that has metastasized (spread) to the brain from another location in the body

A brain metastasis is a cancer that has metastasized (spread) to the brain from another location in the body and is therefore considered a secondary brain tumor. The metastasis typically shares a cancer cell type with the original site of the cancer. Metastasis is the most common cause of brain cancer, as primary tumors that originate in the brain are less common. The most common sites of primary cancer which metastasize to the brain are lung, breast, colon, kidney, and skin cancer. Brain metastases can occur in patients months or even years after their original cancer is treated. Brain metastases have a poor prognosis for cure, but modern treatments are allowing patients to live months and sometimes years after the diagnosis.

Neuro-oncology is the study of brain and spinal cord neoplasms, many of which are very dangerous and life-threatening. Among the malignant brain cancers, gliomas of the brainstem and pons, glioblastoma multiforme, and high-grade astrocytoma/oligodendroglioma are among the worst. In these cases, untreated survival usually amounts to only a few months, and survival with current radiation and chemotherapy treatments may extend that time from around a year to a year and a half, possibly two or more, depending on the patient's condition, immune function, treatments used, and the specific type of malignant brain neoplasm. Surgery may in some cases be curative, but, as a general rule, malignant brain cancers tend to regenerate and emerge from remission easily, especially highly malignant cases. In such cases, the goal is to excise as much of the mass and as much of the tumor margin as possible without endangering vital functions or other important cognitive abilities. The Journal of Neuro-Oncology is the longest continuously published journal in the field and serves as a leading reference to those practicing in the area of neuro-oncology.

Cancer pain can be caused by pressure on, or chemical stimulation of, specialised pain-signalling nerve endings called nociceptors, or by damage or illness affecting nerve fibers themselves.

<span class="mw-page-title-main">Congenital dermal sinus</span> Medical condition

Congenital dermal sinus is an uncommon form of cranial or spinal dysraphism. It occurs in 1 in 2500 live births. It occurs as a dermal indentation, found along the midline of the neuraxis and often presents alongside infection and neurological deficit. Congenital dermal sinus form due to a focal failure of dysjunction between the cutaneous ectoderm and neuroectoderm during the third to eight week of gestation. Typically observed in the lumbar and lumbosacral region, congenital dermal sinus can occur from the nasion and occiput region down.

<span class="mw-page-title-main">Vertebral hemangioma</span> Medical condition

Vertebral hemangiomas or haemangiomas (VHs) are a common vascular lesion found within the vertebral body of the thoracic and lumbar spine. These are predominantly benign lesions that are often found incidentally during radiology studies for other indications and can involve one or multiple vertebrae. Vertebral hemangiomas are a common etiology estimated to be found in 10-12% of humans at autopsy. They are benign in nature and frequently asymptomatic. Symptoms, if they do occur, are usually related to large hemangiomas, trauma, the hormonal and hemodynamic changes of pregnancy (causing intra-spinal bleeding), or osseous expansion and extra-osseous extension into surround soft tissues or epidural region of the spinal canal.

A central nervous system tumor is an abnormal growth of cells from the tissues of the brain or spinal cord. CNS tumor is a generic term encompassing over 120 distinct tumor types. Common symptoms of CNS tumors include vomiting, headache, changes in vision, nausea, and seizures. A CNS tumor can be detected and classified via neurological examination, medical imaging, such as x-ray imaging, magnetic resonance imaging (MRI) or computed tomography (CT), or after analysis of a biopsy.

Myxofibrosarcoma (MFS), although a rare type of tumor, is one of the most common soft tissue sarcomas, i.e. cancerous tumors, that develop in the soft tissues of elderly individuals. Initially considered to be a type of histiocytoma termed fibrous histiocytoma or myxoid variant of malignant fibrous histiocytoma, Angervall et al. termed this tumor myxofibrosarcoma in 1977. In 2020, the World Health Organization reclassified MFS as a separate and distinct tumor in the category of malignant fibroblastic and myofibroblastic tumors.

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