Spinal Cord Tumors – Management of Intradural Intramedullary Neoplasms

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Intradural or primary spinal cord tumors (neoplasms) are uncommon lesions and fortunately affect only a minority of the population. However, when lesions grow, they result in compression of the spinal cord, which can cause limb dysfunction, motor and sensation loss, and, possibly, lead to death. Spinal tumors are classified based on their anatomic location as related to the dura mater (lining around the spinal cord) and spinal cord (medullary) as epidural, intradural extramedullary, or intradural intramedullary. Primary spinal tumors are typically intradural in location, where extradural spinal tumors are typically due to metastatic disease.

This article focuses on the evaluation and management of intradural intramedullary spinal cord tumors. The typical histopathological types that account for 95% of these intramedullary neoplasms include astrocytomas, ependymomas, and hemangioblastomas. Spinal cord astrocytomas and ependymomas can be further classified as glial cell neoplasms. [1, 2, 3, 4, 5] See the image below.

In 1887, Sir Victor Horsley performed the first successful resection of an intradural neoplasm diagnosed by William Gower. This lesion was an intradural extramedullary tumor that compressed the spinal cord. However, the patient did not survive the intervention. Due to limited operative techniques, the first successful removal of an intradural intramedullary tumor, performed by Eiselsberg-Renzi, did not occur until 1907.

In 1925, Charles Elsberg published the first large series of patients who underwent resections of primary spinal cord neoplasms. Unfortunately, again because of the limited operative techniques of this period, these patients endured significant surgical morbidity and mortality. However, subsequent advances in imaging, sterile operative techniques, microsurgical dissection, operating microscopy, and neurophysiologic monitoring have facilitated removal of these lesions with substantially improved clinical results.

Spinal cord parenchyma consists of both gray (neurons and supporting glial cells) and white matter (axonal) and tracts that transmit impulses between the brain and . These tracts, or circuits, control posture, movement, sensation, and autonomic system function, including bowel, bladder, and sexual function.

Neurologic dysfunction develops as the spinal cord tumors enlarge and compress adjacent healthy neural tissue, disrupting these pathways. Upon further compression, patients can lose complete motor function and sensation below the lesion. In addition to weakness and sensory loss, patients may experience pain, particularly at night. This pain is believed to be related to disturbances in venous outflow by the tumor, causing engorgement and swelling of the spinal cord.

Intramedullary spinal cord tumors account for approximately 2% of adult and 10% of pediatric central nervous system neoplasms. In adults, 85-90% of intramedullary tumors are the glial subtypes, astrocytoma or ependymoma. Ependymomas account for approximately 60-70% of all spinal cord tumors found in adults, while, in children, 55-65% of intramedullary spinal cord tumors are astrocytomas. Hemangioblastomas account for 5% of tumors, whereas paragangliomas, oligodendrogliomas, and gangliogliomas account for the remaining lesions.

The pathogenesis of spinal neoplasms is unknown, but most arise from normal cell types in the region of the spinal cord in which they develop. A genetic predisposition is likely, given the higher incidence in certain familial or syndromic groups (neurofibromatosis). Astrocytomas and ependymomas are more common in patients with neurofibromatosis type 2, which is associated with an abnormality on chromosome 22. In addition, spinal hemangioblastomas can develop in 30% of patients with von Hippel-Lindau syndrome, which is associated with an abnormality on chromosome 3.

The spinal cord consists of numerous nerve bundles that descend from and ascend to the brain. Electrical impulses are carried and transmitted to facilitate movement and sensation. With intramedullary spinal cord tumors, compression and stretching of these fiber tracts results in loss of the motor and sensory function. As the tumors grow, the patient’s neurologic function further deteriorates.

Patients with intramedullary glial spinal cord tumors (ie, ependymomas, astrocytomas) typically present with back pain referred from the level of the lesion, sensory changes, or worsening function. The symptoms can be of a long duration, since these lesions tend to grow slowly and typically have a benign histopathology. Patients with low-grade astrocytomas tend to experience symptoms over a mean duration of 41 months. This is in contrast to patients with malignant astrocytomas, whose symptoms persist for a mean duration of only 4-7 months before diagnosis.

Ependymomas are associated with the following:

Mean at presentation of 43 years

Slight female predominance

Pain localized to the spine (65%)

Pain worse at night or upon awakening

Dysesthetic pain (burning pain)

Long history of symptoms

Myxopapillary variant (mean of presentation of 21 y; slight male predominance)

Astrocytomas are associated with the following:

Equal male and female prevalence

Pain localized to spine

Pain worse at night or upon awakening

Paresthesias (abnormal sensation)

Hemangioblastomas are associated with the following:

Onset of symptoms by the fourth decade of life, 80% symptomatic by age 40 years

Familial disorder (ie, von Hippel-Lindau syndrome) present in a third of patients

Decreased posterior column sensation

Back pain localized over lesion

Sensory findings include the following:

Decreased touch, pain, and/or temperature sensation

Hyperesthesias

Decreased proprioception (inability to localize limbs in space)

Abnormal sensation below the level of lesion

Abnormal sensation only at level of lesion (suspended level)

Hyperreflexia findings include the following:

Hoffman sign for cervical lesions

Clonus

Extensor plantar response (Babinski sign)

Other findings include the following:

Motor weakness (late finding)

Spasticity

Increased tone

Muscle atrophy (late finding)

Intramedullary spinal cord neoplasms or tumors are typically histopathologically “benign” or slow growing. However, patients can have more aggressive neoplasms as well as morbidity due to the location of the lesion. Consequently, compared with similar intracranial neoplasms, patients may have a prolonged survival after diagnosis.

Optimal treatment options depend on the patient’s clinical symptoms and neurologic finding. When and whether to treat these lesions as well as perform radiosurgery or surgical excision of lesions remains controversial. However, cures have been reported only after complete surgical resection. Therefore, patients with and confirmatory findings from imaging studies may benefit most from surgical excision, with the surgical goal of total gross resection of the lesion.

Understanding the normal spinal cord vascular supply is essential to treating intramedullary spinal cord lesions; specifically because these vessels may have a variable and inconsistent distribution.

The great vessels (aorta, carotids) contribute arterial supply to the spinal cord via segmental arteries, which further branch into medullary and radicular arteries. The radicular artery provides extramedullary blood supply to the nerve root and dura; the medullary artery bifurcates into and posterior divisions to form the spinal arteries. One anterior and 2 posterior spinal arteries then transverse the longitudinal axis of the spinal cord and provide the blood supply to the spinal cord. Neoplasms acquire their blood supply by leaching blood from these vessels.

The venous plexus of the spinal column, termed the Batson plexus, is unlike other venous systems in the because the veins do not contain valves. Therefore, blood can have pathologic retrograde flow. This retrograde flow blood can back up and cause venous congestion. This can manifest as venous hypertension. Because oxygenated blood cannot pass through the spinal cord because of the congestion of outflow, patients present with progressive neurologic dysfunction.

The spinal cord parenchyma consists of a central canal surrounded by an H-shaped gray matter region that contains neurons. Outer myelinated nerve tracts, termed white matter, surround the central gray matter. The central canal represents an embryologic remnant from neurulation of the neural plate and is lined with ependymal cells. Ependymomas arise from these cells and, therefore, are typically located centrally in the spinal cord parenchyma. In contrast, astrocytes support gray matter neurons and white matter axons. Neoplastic transformation of these supporting cells results in the development of astrocytomas and may occur almost anywhere within the cord.

Observation with serial imaging studies over a variable period is a treatment option for patients who pose a high surgical risk, who are elderly, and/or who only have minimal neurologic signs. Patients in whom pathology tissue shows a malignant neoplasm may be best treated with radiotherapy because they are expected to have an accelerated deterioration and complete surgical resection is not possible.

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James S Harrop, MD Associate Professor, Departments of Neurological and Orthopedic Surgery, Jefferson Medical College of Thomas Jefferson University

James S Harrop, MD is a member of the following medical societies: American Association of Neurological Surgeons, American College of Surgeons, American Spinal Injury Association, Cervical Spine Research Society, Congress of Neurological Surgeons, North American Spine Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Depuy spine consulant, Asterias, Bioventus, Tejin serve on advisory boardsbr/>Received research grant from: DOD, PICORIbr/>Received income in an amount equal to or greater than $250 from: Stryker honorarium.

Tristan Blase Fried Student Medical Researcher, Thomas Jefferson Hospital; Student Researcher (STAR), Department of Dermatology, Hahnemann University

Disclosure: Nothing to disclose.

Zachary J Senders, MD Resident Physician, Department of General Surgery, Case Western/UH Case Medical Center, Case Western Reserve University School of Medicine

Disclosure: Nothing to disclose.

George M Ghobrial, MD Resident Physician, Department of Neurological Surgery, Thomas Jefferson University Hospital

Disclosure: Nothing to disclose.

Ashwini D Sharan, MD Assistant Professor of Neurosurgery, Assistant Professor of Neurology, Thomas Jefferson University School of Medicine

Ashwini D Sharan, MD is a member of the following medical societies: American Medical Association, Association for the Advancement of Medical Instrumentation, International Parkinson and Movement Disorder Society, Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Brian H Kopell, MD Associate Professor, Department of Neurosurgery, Icahn School of Medicine at Mount Sinai

Brian H Kopell, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, American Society for Stereotactic and Functional Neurosurgery, Congress of Neurological Surgeons, International Parkinson and Movement Disorder Society, North American Neuromodulation Society

Disclosure: Received consulting fee from Medtronic for consulting; Received consulting fee from Abbott Neuromodulation for consulting.

The authors and editors of Medscape Reference gratefully acknowledge the contributions of Jennifer Malone, MD, to the development and writing of this article.

Spinal Cord Tumors – Management of Intradural Intramedullary Neoplasms

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