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Spinal cord tumors

Spinal cord tumors
Literature review current through: Jan 2024.
This topic last updated: Jun 28, 2023.

INTRODUCTION — Spinal cord tumors can occur within or adjacent to the spinal cord. They are considered to be intraaxial in location and can be either primary or metastatic. Primary spinal cord tumors account for 2 to 4 percent of all primary central nervous system (CNS) tumors, one-third of which are located in the intramedullary compartment.

The clinical manifestations and general approach to spinal cord tumors will be reviewed here. Clinical features, diagnosis, and treatment of epidural spinal cord compression from metastatic spinal tumors are reviewed separately. (See "Clinical features and diagnosis of neoplastic epidural spinal cord compression" and "Treatment and prognosis of neoplastic epidural spinal cord compression".)

ANATOMY — Spinal cord tumors can be classified according to their anatomic location (figure 1):

Intramedullary – Intramedullary tumors arise within the spinal cord itself. Most primary intramedullary tumors are either ependymomas or astrocytomas. Metastases are being recognized with increasing frequency, primarily because of improvements in imaging modalities.

Intradural extramedullary – Tumors arising within the dura but outside the actual spinal cord are termed "intradural extramedullary." The most common tumors in this group are meningiomas and nerve sheath tumors. (See "Epidemiology, pathology, clinical features, and diagnosis of meningioma" and "Intradural nerve sheath tumors".)

Extradural – Extradural tumors are usually metastatic and most often arise in the vertebral bodies. Metastatic lesions can cause spinal cord compression either by epidural growth that results in extrinsic spinal cord or cauda equina compression or, less frequently, by intradural invasion. (See "Clinical features and diagnosis of neoplastic epidural spinal cord compression".)

The relative frequency of primary spinal tumors is illustrated by a Surveillance, Epidemiology, and End Results (SEER) and National Program of Cancer Registries analysis that included 11,712 cases identified over a four-year period [1]. Of these, 9136 were benign (78 percent) and 2576 were malignant (22 percent). By histology, the three most common tumors were meningioma, tumors of spinal nerves, and ependymoma (33, 27, and 21 percent, respectively).

PRESENTATION

Symptoms — Tumors within or extrinsic to the spinal cord can cause symptoms through disruption of normal neural elements and pathways, producing both local and distal effects.

The most frequent local effect is pain that causes nocturnal awakening. Patients often describe this pain as gnawing and unremitting [2]. The site of this may provide an indication of the anatomic location of the tumor.

Neurologic dysfunction distal to the lesion is due to interruption of ascending and descending spinal cord pathways. The most common sequelae are sensory dysesthesias and muscular weakness, especially of the iliopsoas musculature. Patients often report progressive difficulty in ambulation. Severe distal sensory loss and sphincter dysfunction also may occur. Although neurologic manifestations may begin unilaterally, they can progress to involve both sides of the spinal cord and thereby produce bilateral symptoms and signs.

History — A prior history of cancer may suggest a diagnosis of metastasis to the spinal column, which may cause axial or radiating pain. Referred pain (eg, to the shoulder or neck) is also common with spinal metastases [3].

The spine is a common metastatic site for many tumor types. As an example, vertebral metastases have been found at autopsy in 90 percent of patients who died of prostate cancer, 74 percent with breast cancer, and 45 percent with lung cancer [4,5].

Physical examination — A thorough physical examination is necessary to define probable sites of tumor involvement, document preoperative neurologic deficits, and determine progressive neurologic deterioration. An assessment of the patient's ambulatory status is also necessary since this carries important prognostic significance.

Imaging — Contrast-enhanced magnetic resonance imaging (MRI) of the spine is currently the diagnostic study of choice, providing excellent delineation of the spinal cord and surrounding structures. Almost all primary spinal cord tumors and metastases enhance with gadolinium. (See "Clinical features and diagnosis of neoplastic epidural spinal cord compression", section on 'Magnetic resonance imaging of the spine'.)

INTRAMEDULLARY TUMORS — The majority of intramedullary primary spinal cord tumors are gliomas. The term "glioma" refers to a tumor bearing a histologic resemblance link to normal glial cells. The major types of glial tumors are ependymomas, astrocytomas, and oligodendrogliomas. Spinal cord gliomas are rare compared with cerebral lesions, probably because of the relative paucity of glial tissue in the spinal cord.

The distribution of histologic subtypes of spinal gliomas also differs considerably from intracranial gliomas. Although glioblastomas account for the majority of intracranial gliomas, they comprise only 5 percent of spinal lesions. By contrast, ependymomas comprise approximately 60 to 80 percent of spinal gliomas compared with 3 percent of intracranial gliomas [6]. (See "Classification and pathologic diagnosis of gliomas, glioneuronal tumors, and neuronal tumors".)

Ependymomas — Ependymomas are intramedullary tumors that may be located anywhere along the spinal cord. Approximately one-half occur in the lumbosacral spinal cord or filum terminale; the other 50 percent occur anywhere in the cervical or thoracic spinal cord (image 1) [7]. Extraneural metastases are uncommon [8]. (See "Intracranial ependymoma and other ependymal tumors".)

In the World Health Organization (WHO) classification of brain tumors, ependymal tumors are classified by anatomic site (supratentorial, posterior fossa, spinal), histology (subependymoma, myxopapillary ependymoma, ependymoma), and molecular alterations (table 1) [9]. Spinal ependymomas are now classified according to amplification status of MYCN, a protooncogene that encodes a transcription factor that regulates expression of genes involved in cell growth. It is not yet known how MYCN contributes to the pathophysiology of MYCN-amplified ependymomas.

Spinal ependymomas, MYCN-nonamplified — Ependymomas are the most common intramedullary spinal cord tumors in adults, with a peak age at presentation between 30 and 40 years [9]. The cellular subtype is most common. Most spinal ependymomas are WHO grade 2; grade 3 (anaplastic) tumors are rare [9].

Patients often have localized pain for months to years prior to developing other symptoms. Neurologic deficits may include lower extremity spasticity, loss of pain and temperature sensation, lower extremity and truncal sensory diminution to light touch and vibration, and gait ataxia. Ependymomas tend to occur centrally within the cord, expanding it symmetrically as they grow [10]. The spinal cord may be expanded along several segments, and a tumor-associated cyst (syrinx) is common [11]. These lesions generally enhance intensely on MRI.

Optimal management consists of gross total resection by an experienced surgeon. Although these are infiltrative tumors, a total or near-total resection can frequently be achieved without causing further neurologic deficits. Consensus-based guidelines published by the National Comprehensive Cancer Network (NCCN) suggest adjuvant radiation therapy (RT) for incompletely resected grade 2 tumors and for grade 3 tumors [12]. Grade 3 spinal ependymomas have been associated with higher recurrence rate and poorer survival, although data are limited [9,13,14].

The most extensive data on the natural history and response to treatment come from an observational series of 126 patients with spinal ependymoma, treated over a 47-year period at six institutions [13]. The most frequent subtype was cellular ependymoma, which accounted for approximately 60 percent of classified cases; approximately 70 percent were moderate grade. Myxopapillary ependymomas comprised 17 cases in this series (13 percent).

Treatment included complete resection in 63 cases (50 percent); in 12 of these (19 percent), postoperative RT was given [13]. Among patients in whom a partial resection or biopsy only was performed, 47 of 58 (81 percent) had RT as part of their initial management. Median follow-up was approximately four years. Progression-free and overall survival rates at 15 years were 35 and 75 percent, respectively. Multivariate analyses supported the importance of complete resection, but did not demonstrate a benefit from adjuvant RT. Other large retrospective studies have reported similar findings [15].

There is no proven role for chemotherapy in recurrent or metastatic spinal cord ependymomas. However, the authors have occasionally seen disease stabilization in patients with advanced disease treated with carboplatin-based regimens. Bevacizumab may be useful in NF2-related schwannomatosis (NF2)-associated spinal ependymomas. (See "NF2-related schwannomatosis (formerly neurofibromatosis type 2)", section on 'Intramedullary spinal tumors'.)

Spinal ependymoma, MYCN-amplified — MYCN-amplified spinal ependymoma is a newly recognized tumor as of the 2021 revision of the WHO classification of central nervous system (CNS) tumors [9]. Most tumors have high-grade histopathologic features, although a WHO grade has not yet been assigned due to inadequate clinical studies.

Based on available data, MYCN-amplified ependymomas are aggressive tumors associated with early metastasis and dissemination throughout the neuraxis [16-19]. All reported patients with follow-up data have relapsed despite standard treatment [9].

Myxopapillary ependymoma — Myxopapillary ependymomas are biologically and morphologically distinct from other ependymomas. These tumors most commonly arise in the lumbosacral spinal cord and filum terminale (image 2). Immunohistochemistry may be needed to differentiate myxopapillary ependymomas from either chordomas or chondrosarcomas. (See "Chordoma and chondrosarcoma of the skull base".)

Myxopapillary ependymomas are WHO grade 2 slow-growing glial tumors that typically are found in young adults and are slightly more common in males than in females [20-23]. The median age at diagnosis is 35 to 39 years [20,22,23]. Myxopapillary ependymomas generally present with low back pain, with or without radicular features [22]. The vast majority of these tumors are located in the lumbosacral or thoracolumbar spine [20,22]. Due to the propensity for seeding of the neuraxis, consensus guidelines from the NCCN suggest obtaining complete brain and spine MRI and cerebrospinal fluid analysis at the time of diagnosis [12].

Initial management of these tumors consists of laminectomy with attempted complete surgical resection. These tumors frequently can be totally resected, and many patients are cured following gross total resection. However, some patients recur locally or with leptomeningeal tumor dissemination as much as 20 years after the initial surgery (image 2).

Subtotal resection may be necessary, particularly in patients with unencapsulated tumors [20,21,24]. Consensus-based guidelines published by the NCCN suggest postoperative RT in patients who have undergone subtotal resection or biopsy of a myxopapillary ependymoma [12]. Craniospinal RT is reserved for patients with evidence of leptomeningeal dissemination at the time of initial staging.

Most observational studies have found that postoperative RT is associated with improved local control and progression-free survival, but the effect on overall survival is less clear [20-22,24]. There are no randomized trials addressing the role of postoperative RT. There is a suggestion that higher doses of RT (50.4 to 54 Gy) are more effective than lower doses [22].

In large series, the estimated 10-year overall survival exceeds 90 percent, but up to one-third of patients recur at a median of approximately two years from diagnosis [20,22,23]. The most common pattern of treatment failure is local (85 percent), followed by distant spinal and brain alone [22]. Risk factors for decreased local control and disease-free survival include young age (<36 years) and subtotal resection [22]. Comprehensive genomic analysis suggests that tumors may cluster into two molecular subtypes, with differing age at diagnosis and risk of recurrence [25]. These subtypes have not yet been validated prospectively, however.

Long-term morbidity and late complications of therapy have not been well studied in patients with myxopapillary ependymoma. In the largest series to date, late adverse events were reported in approximately one-quarter of patients and included chronic pain, motor paraplegia, hypoesthesia, and urinary and/or bowel sphincter dysfunction [22].

Astrocytomas — Astrocytomas occur throughout the spinal cord. The pathologic features of spinal astrocytic lesions are predictive of the biologic behavior and clinical course. Approximately one-half of spinal cord astrocytomas are pilocytic and one-half are infiltrative astrocytomas [26]. (See "Classification and pathologic diagnosis of gliomas, glioneuronal tumors, and neuronal tumors".)

Pilocytic astrocytomas are well circumscribed and low grade with nonaggressive clinical behavior. On MRI these tumors enhance intensely with gadolinium (image 3) [26-29].

Diffuse fibrillary astrocytomas of the spinal cord usually appear as nonencapsulated lesions that enhance minimally or heterogeneously on MRI. Approximately one-third are histologically high grade (anaplastic astrocytoma or glioblastoma) (image 4). Approximately half of these tumors harbor H3K27M mutations in histone coding genes and are now classified as H3K27M-mutant midline gliomas [9]. (See "Diffuse intrinsic pontine glioma".)

There are no randomized trials defining the optimal approach to the management of these tumors. The most extensive experience comes from a retrospective series of 136 patients (69 with pilocytic lesions and 67 with infiltrative astrocytomas) treated at the Mayo Clinic over a 43-year period [26]:

At diagnosis, the median age was 35 years, and symptoms were present for a median of eight months.

Gross total resection was feasible in 16 percent of patients, subtotal resection in 25 percent, and biopsy only in 59 percent. There were no differences between pilocytic and infiltrative lesions in the extent of resection.

Survival was significantly longer for patients with pilocytic tumors compared with those with infiltrative astrocytomas (10-year survival 78 versus 17 percent). Even for patients with infiltrative lesions, there appeared to be a plateau in the survival curve with some long-term survivors.

The most important factor associated with a better prognosis was low tumor grade (WHO grade I). Other factors associated with a better prognosis included tumor location other than the cervical region, limited extent of tumor involvement along the spinal cord, and longer (>180 days) duration of symptoms. Multivariate analysis could not demonstrate an advantage for more aggressive surgical resection.

Postoperative RT did not affect outcomes in patients with pilocytic astrocytomas, although it did appear to improve the outcome for infiltrative astrocytomas.

The initial step in the management of a patient with a symptomatic or enlarging presumed primary intramedullary spinal cord tumor is a surgical procedure for tissue diagnosis and resection to the maximum extent possible. Pilocytic astrocytomas can often be completely or near-completely resected without causing further neurologic deficits. Diffuse astrocytomas are more infiltrative, and meaningful resection is often precluded by lack of clear tissue planes and risk of neurologic morbidity [26,30].

Molecular testing (table 2) should be performed to reach an integrated molecular diagnosis whenever possible (table 3). In particular, astrocytic tumors should be tested for isocitrate dehydrogenase (IDH) mutations (which are rare but have been reported in spinal tumors [31,32]), histone K27M mutations, which are enriched in midline (including spinal) diffuse astrocytic tumors, and BRAF variants and fusions, which are common in pilocytic astrocytomas and may have therapeutic implications for targeted therapies. Glioblastoma specimens of sufficient size should also be tested for O6-methylguanine-DNA methyltransferase (MGMT) promoter methylation status. (See "Classification and pathologic diagnosis of gliomas, glioneuronal tumors, and neuronal tumors", section on 'Key molecular diagnostic tests'.)

Given the rarity of spinal cord diffuse astrocytomas, there are no randomized trials to guide recommendations for subsequent treatment, and practice varies [33]. Our approach is to use fractionated RT for low-grade tumors that are incompletely resected and for all high-grade tumors.

The role of chemotherapy in this disease is undefined. There have been no trials of temozolomide or other agents for spinal cord astrocytomas. For patients with an adequate performance status who have an IDH-wildtype glioblastoma or IDH-mutant astrocytoma, it is reasonable to use temozolomide in combination with RT, particularly if the tumor is MGMT methylated, based on data in supratentorial glioblastoma. In a multicenter series of 33 patients with spinal cord glioblastoma, use of concurrent and adjuvant temozolomide was associated with improved survival on univariate analysis, although the result lost statistical significance on multivariable analysis (adjusted odds ratio [OR] 0.35, 95% CI 0.12-1.05) [34]. None of the 14 tumors for which MGMT methylation status was known were methylated, and 20 of 20 tumors tested for IDH mutations were wildtype. Median overall survival was 13.1 months.

Metastases — Intramedullary spinal cord metastases are rare, although the increased use of MRI has resulted in more frequent recognition of such lesions (image 5) [35-37]. Approximately one-half of cases are associated with lung cancer. Breast cancer, renal cell carcinoma, lymphoma, and melanoma are other tumor sites that metastasize to the spinal cord.

Intramedullary metastases are usually observed in patients with widespread metastatic disease. The majority of patients also have brain and lung metastases, while leptomeningeal metastases are seen in approximately 25 percent [35].

Patients typically present with weakness, numbness, and pain. The Brown-Sequard syndrome of hemicord dysfunction is a common initial finding and may raise suspicion of this diagnosis as compared with the more common epidural spinal cord compression. Contrast-enhanced MRI is generally diagnostic (image 6). A computed tomography (CT) myelogram can be useful in patients who cannot undergo MRI. (See "Anatomy and localization of spinal cord disorders", section on 'Brown-Sequard (hemicord) syndrome'.)

Management of intramedullary metastases generally consists of fractionated RT, which usually maintains but does not improve the pretreatment level of neurologic function. As with the treatment of brain metastases and epidural spinal cord compression, corticosteroids are used to diminish the effects of radiation-induced edema until RT is completed [35]. (See "Treatment and prognosis of neoplastic epidural spinal cord compression".)

INTRADURAL EXTRAMEDULLARY TUMORS — Both meningiomas and nerve sheath tumors (schwannomas and neurofibromas) can develop in the intradural extramedullary spinal compartment (figure 1).

Meningioma — Meningiomas can arise from arachnoidal cells anywhere along the neuraxis. They are occasionally found in association with neurofibromatosis [38-40]. Approximately 90 percent of the tumors occur within the cranial fossa. (See "Epidemiology, pathology, clinical features, and diagnosis of meningioma".)

Spinal meningiomas most commonly occur within the thoracic spine (image 7). The tumors are frequently adherent to the spinal dura, requiring dural resection for complete removal, and also grow along intradural and extradural components of the nerve roots.

Spinal meningiomas are typically slowly growing, invasive lesions and may remodel or erode bone. Calcifications may be suggestive of the histologic diagnosis of meningioma [41].

Pathologically, spinal meningiomas demonstrate the same features seen with intracranial lesions. Meningiomas can have areas of increased cellularity or vascularity, xanthomatous components, and areas of hemorrhage. Rarely, the tumors may be considered malignant or aggressive if they demonstrate a proclivity to recur.

The usual treatment for spinal meningiomas is resection, and complete resection can often be achieved. The dural origin is generally cauterized and occasionally resected. Thoracic spinal roots may be sacrificed as necessary to obtain a complete resection; cervical and lumbar nerve roots are preserved whenever possible.

Subtotally resected lesions are generally followed expectantly for regrowth. Symptomatic recurrences are generally treated with further surgery. Radiation therapy (RT) or radiosurgery may be useful in patients with tumor regrowth that is not amenable to further resection [42,43].

Nerve sheath tumors — Nerve sheath tumors constitute approximately 25 percent of tumors arising in the intradural extramedullary space and often extend laterally and enlarge the neural foramen (image 8). The clinical presentation, treatment, and prognosis of these tumors is discussed separately. (See "Intradural nerve sheath tumors".)

EXTRADURAL PRIMARY TUMORS — The most common tumors arising in the extradural space are metastases. Several uncommon primary tumors can also arise in the extradural space.

Metastases — Metastatic tumor from any primary site can involve the epidural area and can cause epidural spinal cord compression. The three most common primary tumors are prostate cancer, breast cancer, and lung cancer. The clinical features and management of this complication are discussed separately. (See "Clinical features and diagnosis of neoplastic epidural spinal cord compression" and "Treatment and prognosis of neoplastic epidural spinal cord compression".)

Chordomas — Chordomas are rare bone tumors that are locally invasive, frequently recur, and are relatively radioresistant. The tumors are remnants of the primitive notochord and may occur at the skull base (35 percent); cervical, thoracic, and lumbar spine (15 percent); and sacral regions (50 percent) (image 9). The presentation and management of patients with chordoma arising in the skull base are discussed separately. (See "Chordoma and chondrosarcoma of the skull base".)

Surgery and RT — The initial treatment of chordomas arising in the spine consists of wide local excision when possible [44-46]. Complete resection, although desirable, is not feasible in many cases because of anatomic constraints to surgical access and the proximity of adjacent critical normal structures. For this reason, postoperative radiation therapy (RT) is frequently used [45,47].

The results following surgery are illustrated by a retrospective series of 138 consecutive patients treated at two institutions over a 28-year period [44]. Lesions were in the sacrum, lumbar spine, or cervical-dorsal spine in 78, 15, and 7 percent of cases, respectively; none were in the skull base. Surgical resection was the initial therapy in 130 cases (94 percent). At a median follow-up of 12 years, the 10-year local relapse-free survival, distant relapse-free survival, and overall survival rates were 33, 72, and 54 percent, respectively. Although surgery was supplemented with RT in 31 percent of cases, doses were generally lower than those used in contemporary series.

Newer RT techniques, including stereotactic radiosurgery and charged particle irradiation (eg, protons, carbon ions), have been used to target the bone lesion while reducing the radiation exposure to the surrounding nerve roots and the cauda equina [48-53]. Even for patients with unresectable spine and sacral chordomas, high-dose definitive RT using advanced techniques may achieve durable local control and disease-free survival in a subset of patients [54].

Systemic therapy — Systemic therapy for the treatment of relapse after maximal surgery and/or RT has focused on molecularly targeted therapies [55].

A limited number of prospective phase II studies and multiple observational series have shown significant antitumor activity with imatinib as a single agent or in combination with other drugs [56-62]. In some instances, tumor responses have been manifested by necrosis or decreased uptake on positron emission tomography (PET) scan. Patients who have progressed after an initial response to imatinib have been reported to respond to combinations of imatinib plus cisplatin [60] or sirolimus [58]. Other targeted agents that may have activity include vascular endothelial growth factor (VEGF) receptor inhibitors (sunitinib, apatinib where available) and erlotinib [63-66].

Older series that used cytotoxic agents alone have not reported clinically significant activity. There is only limited experience with chemotherapy for the treatment of relapse after maximal radiation and surgery. Reported series generally include chordomas arising in the base of the skull as well as the spinal cord. The use of cytotoxic agents has not been demonstrated to have clinically significant activity [67,68].

Sarcomas — Sarcomas arise from mesenchymal elements and may erode into the bony spine. Rarely, they can arise directly from bony elements of the spine [69]. These lesions usually occur in younger patients, and are treated with a multimodality approach that includes surgery, radiotherapy, and chemotherapy. (See "Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma", section on 'Introduction'.)

Bone sarcomas, particularly osteosarcomas, may develop in patients with polyostotic Paget disease (pagetic sarcoma). This is a rare anaplastic malignancy with a peak incidence in the seventh and eighth decades of life. (See "Osteosarcoma: Epidemiology, pathology, clinical presentation, and diagnosis", section on 'Paget disease and other benign bone processes'.)

Paget osteosarcomas are usually sclerotic lesions, occasionally blastic. Treatment usually consists of vertebrectomy, followed by chemotherapy and radiation. The prognosis of this tumor is poor.

Chondrosarcoma is a malignant tumor of cartilage in which the matrix is entirely chondroid in nature. The presence of discrete calcified opacities is a radiographic hallmark of these lesions. Chondrosarcomas of the spine are relatively infrequent (5 percent of all spinal tumors). They typically originate in the vertebral body and extend into the adjacent soft tissue and spinal canal (image 10). These tumors have a predilection for the lower thoracic and lumbar regions. Surgery is the primary treatment for chondrosarcomas. RT and chemotherapy play a minor role and are only used in high-grade chondrosarcomas. (See "Chondrosarcoma" and "Chordoma and chondrosarcoma of the skull base".)

Leiomyosarcoma is a malignant mesenchymal tumor composed predominantly of spindle cells that exhibit smooth muscle differentiation. Primary leiomyosarcoma of the bone is extremely rare [70,71].

Ewing sarcoma is a small round cell tumor, which can arise in bone or soft tissue. The presentation, management, and prognosis of the Ewing sarcoma family of tumors are discussed separately. (See "Clinical presentation, staging, and prognostic factors of Ewing sarcoma".)

Lymphoma — Lymphomatous involvement of the spine is most often due to metastatic disease but occasionally can represent primary disease. Treatment can include surgery, radiotherapy, and chemotherapy.

Plasmacytomas and multiple myeloma — The spine can be the initial site of involvement for plasma cell neoplasms. Although plasmacytomas can be solitary, these lesions more commonly are part of a diffuse process. (See "Solitary plasmacytoma of bone" and "Solitary extramedullary plasmacytoma" and "Multiple myeloma: Clinical features, laboratory manifestations, and diagnosis".)

Langerhans cell histiocytosis (eosinophilic granuloma) — Langerhans cell histiocytosis (eosinophilic granuloma) can present at a variety of sites, including the vertebral bodies. (See "Clinical manifestations, pathologic features, and diagnosis of Langerhans cell histiocytosis", section on 'Lytic bone lesions'.)

Benign lesions — Benign lesions can arise in the spine and must be differentiated from malignant tumors [72]:

Osteoid osteomas – Osteoid osteomas are small lesions (<2 cm) that typically arise in long bones, although approximately 10 percent occur in the spine. Conservative management with salicylates is often sufficient. (See "Nonmalignant bone lesions in children and adolescents", section on 'Osteoid osteoma'.)

Osteoblastomas – Osteoblastomas are larger lesions (>2 cm) that also typically occur in young men. Because of their size, these lesions tend to cause neurologic symptoms. Although benign, these lesions tend to recur and generally are managed surgically. (See "Nonmalignant bone lesions in children and adolescents", section on 'Osteoblastoma'.)

Osteochondromas – Osteochondromas are benign lesions that account for less than 4 percent of spinal tumors. These lesions consist of both healthy bone and a cartilaginous cap. Biologic behavior varies, and surgery should be considered only for lesions producing symptoms. The most important complication is malignant degeneration to a peripheral chondrosarcoma, the risk of which is less than 1 percent. (See "Nonmalignant bone lesions in children and adolescents", section on 'Osteochondroma and hereditary multiple osteochondromas' and "Chondrosarcoma", section on 'Osteochondroma'.)

Chondroblastomas – Chondroblastomas are tumors arising in bone and comprised of immature cartilage; only rarely have these been reported to arise in the spine. (See "Nonmalignant bone lesions in children and adolescents", section on 'Chondroblastoma'.)

Giant cell tumors – Giant cell tumors comprise 4 to 8 percent of all primary bone tumors; up to 10 percent may arise in the spine, particularly in younger individuals. Giant cell tumors tend to be larger, be very vascular, and have a high frequency of recurrence. (See "Giant cell tumor of bone".)

Vertebral hemangiomas – Vertebral hemangiomas are nonneoplastic lesions that are composed of thin-walled blood vessels. The term "hemangioma" is a misnomer since these are not neoplasms but low-flow venous malformations. These lesions are common, incidental findings, are typically confined to the vertebral body, and only rarely produce symptoms [73]. Rarely, "aggressive hemangiomas" may extend into the epidural space and compress the spinal cord (image 11).

Aneurysmal bone cysts – Aneurysmal bone cysts are nonneoplastic, expansile lesions, which account for up to 15 percent of primary spine tumors [74]. These lesions tend to be locally aggressive with a substantial potential for neurologic compromise. Thus, early surgical resection and spinal stabilization is generally required [75]. (See "Nonmalignant bone lesions in children and adolescents", section on 'Aneurysmal bone cyst'.)

SUMMARY AND RECOMMENDATIONS

Anatomy – Tumors affecting the spinal cord can be classified as intramedullary, extramedullary intradural, or extradural (figure 1). (See 'Introduction' above.)

Clinical presentation – Back pain, especially nocturnal, and progressive neurologic dysfunction are the most common presenting symptoms. Contrast-enhanced MRI of the spine is the best modality for imaging spinal cord tumors. (See 'Presentation' above.)

Primary intramedullary tumors – The most common intramedullary tumors include ependymomas (table 1), pilocytic astrocytomas, and diffuse astrocytomas. Myxopapillary ependymomas constitute a distinct subset of ependymomas that occur almost exclusively in the conus medullaris and filum terminale of the spinal cord. (See 'Intramedullary tumors' above.)

Maximal surgical resection is the initial step in the management of patients with intramedullary spinal cord tumors. Complete or near-complete resection is more often achieved for low-grade ependymoma and pilocytic astrocytoma than for high-grade ependymoma and diffuse astrocytoma. (See 'Ependymomas' above and 'Astrocytomas' above.)

For patients with World Health Organization (WHO) 1 or 2 intramedullary glial tumors in whom the initial surgery results in a complete resection, we suggest observation rather than adjuvant radiation therapy (RT) (Grade 2C). (See 'Spinal ependymomas, MYCN-nonamplified' above and 'Astrocytomas' above.)

For patients in whom complete resection has not been possible (ie, biopsy only or incomplete resection), we suggest postoperative adjuvant RT (Grade 2C). Temozolomide may be added in patients with high-grade astrocytic tumors, particularly if they are known to be O6-methylguanine-DNA methyltransferase (MGMT) methylated. RT may also be useful for patients with recurrent disease following initial resection. (See 'Spinal ependymomas, MYCN-nonamplified' above and 'Astrocytomas' above.)

Intradural extramedullary lesions – The most common intradural extramedullary tumors are meningiomas and nerve sheath tumors. The presentation and management of these tumors are discussed separately. (See "Epidemiology, pathology, clinical features, and diagnosis of meningioma" and "Intradural nerve sheath tumors".)

Extradural tumors – The vast majority of tumors occurring in the extradural space are metastases. These tumors are particularly important because of the risk of epidural spinal cord compression. The clinical features and management of this complication are discussed separately. (See "Clinical features and diagnosis of neoplastic epidural spinal cord compression" and "Treatment and prognosis of neoplastic epidural spinal cord compression".)

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Topic 5227 Version 34.0

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