clınıcal neurosciences

Clınıcal NeurosciencesSpine & Spinal Cord TumorsVolume 3 Number 1Winter 2009

I s s u e s I n

A Q u a r t e r l y P u b l i c a t i o n o f t h e N e u r o s c i e n c e s I n s t i t u t e s a t R o o s e v e l t H o s p i t a l

Leading the way to the future...

TM

2

Issues in Clinical neurosciencestm

3

spine & spinal Cord Tumors

WelcomeA variety of tumors,

both primary and second-ary, involves the spinal column. These tumors may arise from bone, nerve roots, meninges, or the spi-nal cord.

Tu mor s of t he spine are uncommon in the general population. Primary tumors are very rare, although metastases

to the spine from systemic cancer are more common. Treatment of these conditions may be performed using many

different methods, consisting of single or combined approaches, open or minimally invasive techniques, and may or may not require subsequent stabilization. A substantial degree of judgment and experi-ence is required to achieve the best possible outcome for the patient.

Our surgeons’ skills are augmented by superb intra-operative image guidance, real-time neurophysiologic monitoring, and excel-lent neuro-anesthesia. The postoperative attention to each patient is optimized with an advanced intensive care and rehabilitative program within our hospital.

In our Neurosurgery Department, we are fortunate to be able to offer our patients with spine and spinal cord tumors state-of-the-art treatment in all aspects of care.

This issue illustrates some interesting and challenging cases treated by our multidisciplinary team.

Sincerely yours,

Chandranath Sen, MD

Chandranath Sen, MD Chairman, Department of Neurosurgery, Co-Director, Center for Cranial Base Surgery

© 2009. Issues in Clinical Neurosciences™

A Quarterly Publication of the Department of Neurosurgery at Roosevelt Hospital www.roosevelt-neurosurgery.com1000 Tenth Avenue, Suite 5G-80

New York, NY 10019All rights reserved.

Graphic Design: State of the Art. Cover Illustration: iStock ImagesOnline version: www.roosevelt-neurosurgery.com/publications/

Journal Contents

3 CHAIRMAN’S NOTE

Dr. Chandranath Sen introduces our audience to the topic of spine and spinal cord tumors

4-5 INTRODUCTION: Dr. Charles Ippolito

The molecular basis of malignancies, metastasis to the spine, and the epidemiology of spinal tumors are discussed. Clinical evaluation, radiologic studies, and other diagnostic procedures are brought into focus as a foundation for the surgical approaches that are reviewed in the various case presentations.

6-7 CASE PRESENTATION # 1: Dr. Noel Perin

Neurophysiologic monitoring and dorsal column mapping in intramedullary spinal cord surgery are discussed with an illustrative case report.

8 CASE PRESENTATION # 2: Dr. Noel Perin

An intramedullary spinal cord tumor was found in a 35-year-old female, who had been treated for 4 years for a degenerative spinal disorder; she developed progressive weakness and numbness with gait difficulty.

9-10 CASE PRESENTATION # 3: Dr. Chan Roonprapunt

A 49-year-old female patient was diagnosed with a cervical heman-gioblastoma that was successfully resected.

10-13 CASE PRESENTATION # 4: Dr. Chandranath Sen and

Dr. Noel Perin

A 35-year-old man was diagnosed with a cervical chordoma. His tumor was resected, and his cervical spine was reconstructed with stabilization and fusion. Postoperatively, the patient showed no evidence of residual tumor.

14 NEUROSURGICAL STAFF

Phone numbers and e-mail addresses of our neurosurgical staff are provided for consultation and referral purposes.

15 AFFILIATES, RESIDENTS, FELLOWS & EDITORIAL

A list of professionals affiliated with the Department of Neurosurgery is provided.

4


5


In cases where the spinal canal is compromised, additional imaging studies (including myelography with postmyelography CT scanning, angiography, or radionuclide bone scanning) may be required. Since many spinal tumors are vascular, angiography may help decide if embolization might be useful to help reduce the amount of intraoperative blood loss.

Staging of Benign and Malignant Tumors

Once a diagnosis has been established, it is important to categorize or stage the tumor, since it provides the clinician with a rationale upon which to base treatment and prognosis. In this process, both anatomic and histologic data are integrated. In the case of skeletal tumors, the staging system devised by Enneking has been quite useful.

In this system, histologic architecture, radiology, and the natural history of the tumor are used to stage benign lesions. Stage 1 lesions are immobile and tend to heal spontaneously. Stage 2 lesions are radiographically more aggressive in appearance but histologically immature, showing evidence of growth progression. Stage 3 lesions tend to be locally aggressive, histologically immature, and show growth progression that is not limited by natural barriers (e.g., cartilage, cortical bone, or major fascial planes).5

Histologic architecture is used to grade sarcomas, which are designated low-grade Stage-I (Broders grades 1 and 2) or high-grade Stage-II (Broders grades 3 and 4). Lesion location is specified as “A” when intracompartmental and “B” when extracompartmental. Using this system, malignant bone tumors are classified as IA, IB, IIA, IIB.6

REFERENCES

1. Chiang AC, Massagué J. Molecular origins of cancer: molecular basis of metastasis. N Engl J Med. 2008;359:2814-2823.

2. Roodman GD. Mechanisms of bone metastasis. N Engl J Med. 2004,350:1655-1664.

3. Barrenechea IJ, Perin NI, Triana A, Lesser J, Costantino P, Sen C. Surgical management of chordomas of the cervical spine. J Neurosurg Spine. 2007;398-406.

4. Mallon MJ, Harrelson JM. Primary neoplasms of the spine. In: Wilkins RH, Rengachary SS (eds). Neurosurgery. 2nd Ed. Vol. II. New York: McGraw-Hill. 1996:1805-1813.

5. Sundaresan N, DiGiacinto GVD, Hughes JEO. Surgical treatment of spinal metastases. Clin Neurosurg. 1986;33:503-522.

6. Sundaresan N, Krol G, Hughes JEO, Hough L. Tumors of the spine: diagnosis and management. In: Tindall GT, Cooper PR, Barrow DL (eds). The Practice of Neurological Surgery. Vol. 1. Baltimore: Williams & Wilkins. 1996:1303-1322.

SPINAL TUMORS

VERTEBRAL

Benign

Posterior elements• Osteochondroma • Osteoid osteoma – small, night pain • Osteoblastoma – large, lumbar • Aneurysmal bone cyst – large lumbar

Anterior Elements• Giant Cell – sacrum and vertebral body • Hemangioma • Eosinophilic granuloma

Malignant

• Plasmacytoma – pedicle and vertebral body • Ewing’s sarcoma – sacrum • Lymphoma • Chondrosarcoma • Osteosarcoma • Chordoma

Metastatic

• Lung • Breast • Prostate • Thyroid • Kidney

EXTRADURAL

• Meningioma • Neurofibroma • Schwannoma

INTRADURAL/EXTRAMEDULLARY

• Meningioma • Neurofibroma • Schwannoma • Myxopapillary ependymoma

INTRAMEDULLARY

• Ependymoma • Astrocytoma • Hemangioblastoma • Paragangliomas • Oligodendrogliomas • Gangliogliomas

Table courtesy of Daniel S. Yanni, MD

IntroductionLesions of the Osseous Spine

The molecular transformation to malignant cells is a multistep process involving the deregulation of normal cellular growth to unbridled proliferation. Factors involved in the deregulation process are still under investigation and include loss of tumor suppressor genes and the presence of activated oncogenes. Taking into account the multifactorial molecular basis of this transformation from precancer-ous to malignant status, many pathologic conditions of the osseous spine can be considered premalignant (e.g., atypical osteoblastoma, enchondroma, hereditary multiple osteochondromatosis, Paget’s disease of bone).1,2 In addition, the vertebral column may serve as a common site for metastasis from distant primary malignancies.2 These malignancies can include lung, breast, prostate, kidney, and GI tract.

Primary Neoplasms

Primary neoplasms of the spine tend to be rare, and several large studies have shown that skeletal tumors arose in only 11% of all cases reviewed.3 Due to this low incidence, symptoms arising from a primary spinal tumor are often overlooked, since symptoms can resemble those arising from degenerative spinal disorders. Primary tumors, therefore, should be ruled out in the differential diagnosis of atypical symptoms often presenting as spondylosis.4

Epidemiology of Spinal Tumors

The American Cancer Society has reported that approximately 2000 new cases of bone cancer and 6000 cases of soft tissue sarcomas are diagnosed each year, with about 5% involving the spine. Certain tumors arise primarily in the vertebral column (e.g., osteoblastomas). Autopsy studies have shown that a wide range (from 5% to 30%) of all cancer patients have metastases to the spine. These studies have also shown that metastases to the spine involve four primary sites: prostate, breast, lung, and the hematopoeitic system. The tendency of some tumors to metastasize to the spine and skeletal system is known as osteotropism.4

Pathogenesis of Spinal Tumors

Embryonic origin is the main differentiator in the classification of primary tumors of the spine. This classification establishes the dif-ference between tumors arising from osteogenic cells (i.e., true bone tumors) and tumors arising from nonosseous cells in adjacent tissue.

Four primary cells are involved in osteogenesis: osteoblast, osteoclast, chondroblast, and fibroblast. Those tumors derived directly from the osteogenic process include the osteogenic sarcoma, osteo-blastoma, osteoid osteoma, and the osteoma. Tumors arising from

osteoblasts, the principal cell in the orchestration of osteogenesis, demonstrate active ossification and calcification. Tumors derived from chrondroblasts (e.g., chrondroscarcoma, chondroblastoma, osteochondroma, chondroma) demonstrate cartilage as part of their histologic architecture. Tumors that are derived from fibroblasts (e.g., fibrous histiocytoma, fibrosarcoma, fibroma) feature collagen (see Table). By definition, the osteoclast destroys bone; it is the progenitor to the giant cell tumor.

In the majority of patients with cancer, metastasis to the spine is generally accomplished via the hematogenous route. In this paradigm, the progressive replacement of red bone marrow by tumor cells eventually leads to invasion of the epidural space. Traditionally, the pedicle was believed to be the initial site of involvement. In the case of prostate tumors, emboli probably spread to the vertebral body via the basivertebral plexus.5

Clinical Evaluation and Diagnosis

In patients with a primary tumor of the spine, pain is often the chief presenting complaint. There is often a similarity between symptoms of herniated disk and spinal tumors, often making a dif-ferential diagnosis difficult. A distinguishing factor in differentiating the pain involved in these two conditions is that tumor pain seldom improves with rest and may actually get worse at night with patients in the supine position. In 80% to 100% of primary neoplasms, pain (the cardinal symptom of spinal tumors) can often be the only symptom at the time of initial diagnosis.

Along with pain, neurologic deficits are often pathognomonic and frequently develop when a tumor compresses or infiltrates the spinal cord, the nerve roots, or paraspinal nerve plexuses. Neurologic deficits are generally late manifestations of spinal tumors, which can be preceded by long periods of local or radicular pain.

Radiologic Evaluation

Currently, patients presenting with a longstanding history of axial and radicular pain (with or without neurologic deficits) will have an MRI scan done as the initial imaging study. If a primary or meta-static bone tumor is noted on MRI, a CT scan is obtained to evaluate the bony anatomy and bone destruction. If the lesion is thought to be metastatic in nature, a metastatic workup should be initiated in the absence of a known primary tumor

6


7


Figure 1.3. MRI showing intramedullary tumor enlarging the cord, isointense on T1, hyperintense on T2, hyperintense on proton density, and not contrast enhancing.

CASE PRESENTATION #1Chief Complaint, Presentation, and History of Present Illness

A 67-year-old female presented with a 4-year history of back pain and a 3-month history of increasing gait difficulty and right leg weakness. Neurological examination showed an antalgic gait, a spastic right leg with weakness 3/5, and diminution to pin sensation in the entire right lower extremity. The patient was grossly myelopathic in both lower extremities; joint position sense was absent in the right lower extremity and intact on the left. MRI scans revealed a large intramedullary tumor at T7-T8 enlarging the spinal cord. The tumor was isointense on T1, hyperintense on T2, without enhancement with gadolinium, and without a cystic component (Fig. 1.3)

Diagnosis, Surgical Approach, and Follow-Up

A standard thoracic laminectomy and durotomy were carried out, exposing the spinal cord and showing significant enlargement over 2 to 3 segments. We were unable to determine the extent of the midline macroscopically. By using the strip electrode for dorsal column mapping (Fig. 1.2), the neurophysiology team was able to locate the anatomic midline as lying between electrodes 6 and 7. The myelotomy was placed at the selected site, and a gross total removal of the tumor was achieved. The final pathology was gangliocytoma.

Postoperatively, the patient had transient increased weakness in the right lower extremity, but good strength in the left. Joint position sense was preserved on the left and absent on the right, and the patient was stable from her pre-operative exam. At follow-up, her right lower extremity strength continued to improve with physical therapy.

REFERENCES

1. Costa P, Bruno A, Bonzanino M, Massaro F, Caruso L, Vincenzo I, Ciaramitaro P, Montalenti E. Somatosensory- and motor-evoked potential monitoring during spine and spinal cord surgery. Spinal Cord. 2007;45(1):86-91.

2. Deletis V, De Camargo BA. Interventional neurophysiological mapping during spinal cord procedures. Stereotact Funct Neurosurg. 2001;77(1-4):25-28.

3. Deletis V, Sala F. The role of intraoperative neurophysiology in the protection or documentation of surgically induced injury to the spinal cord. In: Slikker W Jr, Trembly W (eds), Neuroprotective Agents. Fifth International Conference. Ann. N. Y. Acad. Sci. 2001;939:137-144.

4. Epstein FJ, Farmer JP, Freed D. Adult intramedullary spinal cord ependymomas: the result of surgery in 38 patients. J Neurosurg. 1993;79(2):204-9.

5. Hoshimaru M, Koyama T, Hashimoto N, Kikuchi H. Results of microsurgical treatment for intramedullary spinal cord ependymo-mas: analysis of 36 cases. Neurosurgery. 1999;44(2):264-269.

6. Kržan M, Deletis V, Isgum V. Intraoperative neurophysiologi-cal mapping of dorsal columns. A new tool in the prevention of surgically induced sensory deficit? Electroenceph Clin Neurophysiol. 1997;102, 37P.

7. Manzano G, Green BA, Vanni S, Levi AD. Contemporary man-agement of adult intramedullary spinal tumors--pathology and neurological outcomes related to surgical resection. Spinal Cord. 2008;46(8):540-6.

8. Quiñones-Hinojosa A, Gulati M, Lyon R, Gupta N, Yingling C. Spinal cord mapping as an adjunct for resection of intramedullary tumors: surgical technique with case illustrations. Neurosurgery. 2002;51(5):1199-1207.

9. Sala F, Bricolo A, Faccioli F, Lanteri P, Gerosa M. Surgery for intramedullary spinal cord tumors: the role of intraoperative (neu-rophysiological) monitoring. Eur Spine J. 2007;16 Suppl 2:S130-9.

10. Shrivastava RK, Epstein FJ, Perin NI, Post KD, Jallo GI. Intramedullary spinal cord tumors in patients older than 50 years of age: management and outcome analysis. J Neurosurg Spine. 2005;2(3):249-55.

11. Smith M, Deacon P. Topographical anatomy of the posterior columns of the spinal cord in man. The long ascending fibers. Brain. 1984;107:671-698.

NEUROPHYSIOLOGICAL MONITORING AND DORSAL COLUMN MAPPING IN INTRAMEDULLARY SPINAL CORD SURGERYClinical Overview

The Neurosciences Group at Roosevelt Hospital works closely with the prestigious group of neurophysiologists led by Vedren Deletis, MD, PhD. Dr. Deletis is one of the pioneers in the development of motor-evoked potential monitoring in spinal cord surgery. He gained extensive experience working with the late Dr.Fred Epstein on many hundreds of spinal cord tumor surgeries ay NYU Medical Center, then at Beth-Israel North, and now at the Roosevelt Medical Center.

We collaborate closely with the neurophysiology team and have developed many techniques to monitor neural tracts to enable us to perform spinal surgery more precisely and safely. More recently, using dorsal column mapping to identify the physiological and functional midline of the spinal cord for myelotomy, this technique has enabled us to minimize injury to the dorsal columns. All patients undergoing intramedullary surgery have motor-evoked potential monitoring, somatosensory-evoked potential monitoring, dorsal column mapping, and the recording of “D” waves throughout the operation.1-4

Intramedullary spinal cord surgery carries significant risk for neurologic impairment. Intramedullary spinal cord tumors are rare neoplasms accounting for approximately 2% to 4% of central nervous system tumors. These tumors are primarily astrocytomas and ependymomas. Astrocytomas commonly occur in the pediatric population, whereas ependymomas are more frequently encountered in the adult population. These tumors are very slow growing and can reach significant proportions within the spinal cord before becoming symptomatic. They tend to expand the spinal cord and can distort the surface anatomy.4,5

Surgical resection is the definitive treatment for intramedullary spinal cord tumors.4,5,6,7 Resection of large centrally located intramed-ullary spinal cord tumors is achieved via a midline myelotomy. The midline in a normal cord is the dorsal median sulcus, located between the elevated posterior columns (Fig. 1.1). The midline can also be identified by following the dorsal median sulcal vein as it enters the midline raphe and also by locating a point midway between the root entry zones on either side.

However, this anatomy is frequently distorted in cases of tumor, due to edema, neovascularization, or scar formation. The distortion can be a combination of rotation of the cord and asymmetric enlarge-ment, making identification of the surface anatomy extremely difficult (Fig. 1.1). Inadvertent dissection through the dorsal columns will cause postoperative sensory deficits, including the loss of proprioception, which can be disabling.2,5,6,7

Dorsal column dysfunction is the most common cause of postoperative morbidity following myelotomy for spinal cord tumors, reported in 43.6% of patients in one series.7 Many authors believe that the dysfunction following intramedullary spinal cord surgery is, at least in large part, a result of injury to the posterior columns.5,7

Figure 1.1. Artist rendering of normal spinal cord anatomy showing the elevated posterior columns and dorsal median sulcus

Figure 1.2 A miniature multi-electrode (1-8) grid is used intra-operatively to measure the amplitude gradient of conducted somatosensory-evoked potentials during functional mapping of the dorsal columns.

The standard microsurgical splitting of the dorsal columns from within the dorsal median sulcus is performed after identifying the midline via standard anatomical landmarks without any objective neurophysiological data. Injury to the dorsal columns during this dis-section can result in dysfunction manifesting as numbness, tingling, painful dysesthesias, or ataxic gait.8

This can be significantly incapacitating on the patient’s functional status and can affect the ability to rehabilitate. Decreasing the risk of dorsal column dysfunction remains a challenge in the treatment of intramedullary spinal cord lesions requiring a midline myelotomy.8,9,10

Together with the standard pre-operative radiographic studies and intra-operative ultrasound to identify the exact location of the tumor within the spinal cord, we utilized an intra-operative functional technique of mapping the dorsal columns to help locate the midline for the myelotomy.6,8 This is accomplished by defining the amplitude gradient of conducted somatosensory-evoked potentials (SEPs) using a miniature multi-electrode grid (Fig. 1.2). These signals are interpreted intra-operatively by the neurophysiology team correlating the surgical anatomy with the functional anatomy. We have found this technique particularly useful in patients with large intramedullary spinal cord tumors and syringomyelia.9,10,11

8


9


This sensory deficit improved when compared to her pre-operative status. Postoperative imaging revealed gross total resection with significant reduction in intramedullary edema formation (Figs. 3.4 & 3.5). No further adjuvant therapy was indicated.

Discussion

Hemangioblastomas are vascular tumors, which can be found throughout the neuraxis. They may occur as sporadic isolated lesions or multiple lesions in the cerebellum and retina and are often associ-ated with the dominantly inherited familial cancer syndrome known as von Hippel Lindau disease.

Although considered histologically benign, hemangioblasto-mas may cause significant neurological deficits, especially when they present in the spinal cord, where they comprise approximately 3% of all intramedullary spinal tumors. Advances in imaging, neurophysi-ologic monitoring, and microsurgery have markedly improved the treatment of these intraspinal lesions.

Spinal hemangioblastomas are rare intramedullary tumors and can present with symptoms similar to those associated with intramedullary astrocytomas and ependymomas. MRI is helpful for the differential diagnosis. T1-weighted images often reveal homoge-neously enhancing tumor with rostral/caudal cyst(s) and associated edema. Also, T2-weighted images typically demonstrate extensive edema not seen in other intramedullary neoplasms

CASE PRESENTATION #3

Cervical HemangioblastomaChief Complaint, Presentation, and History of Present Illness

A 49-year-old female presented to her primary care physician with a several-month history of neck pain and tingling in her fingers. Her symptoms were initially attributed to carpal tunnel syndrome. An MRI scan was performed when her dysesthesias progressed proximally to involve the deltoid region. The study revealed a large neoplasm in the cervical region (Figs. 3.1 & 3.2).

She underwent a selective spinal angiogram to delineate the vascular anatomy before surgery (Fig. 3.3). The dorsally situated hypervascular tumor received its blood supply from the lateral spinal arteries, and drainage through posterior surface veins.

Surgical Approach and Results

A C1-3 laminectomy was performed, with gross total resection of the neoplasm. Intra-operative somatosensory-evoked poten-tials and motor-evoked potentials were utilized during resection. Postoperatively, she had a transient increase in her sensory deficit, with impaired joint position sense in her right hand. Her motor strength remained normal.

Figure 3.1. Pre-operative MRI with gadolinium contrast of a large intramedullary hemagio-blastoma. T1-weighted sagittal image demonstrated a large homogeneously enhancing tumor.

Figure 3.2. Pre-operative T1-weighted axial image better demonstrated the intramedul-lary location of the neoplasm and the associated cyst.

Figure 3.3. Selective pre-operative angiogram showing the feeding and draining vessels to this tumor.

Draining Vein

Lateral Spinal Artery

Figure 2.1. Illustration of an intramedullary spinal cord tumor rotat-ing and distorting the cord, thereby displacing the midline.

Figure 2.2. MRI scan of the cervical spine revealed a large, partially cystic, enhancing intramedullary tumor at C2-C3.

INTRAMEDULLARY SPINAL CORD TUMORClinical Overview

Intramedullary spinal cord tumors comprise 4% of central nervous system tumors and 20% of all intraspinal tumors (Fig. 2.1). Traditionally, two types of tumors occur primarily in this location: ependymomas and astrocytomas. Astrocytomas occur more fre-quently in children, and ependymomas are more commonly found in the adult population.

The vast majority of ependymomas are slow growing, well- demarcated neoplasms. They produce symptoms by compression of adjacent spinal cord tissue, as opposed to infiltration, as occurs in astrocytomas. These tumors occur predominantly in the cervical and cervico-thoracic regions. A group of these ependymal tumors occurs in the filum terminale and conus of the spinal cord. Filum ependymomas are papillary or myxo-papillary and are more benign than the intramedullary and intracranial counterparts.

In terms of clinical presentation, most patients have pain either in the neck or back as the predominant symptom. They can develop any combination of numbness, weakness, gait imbalance, and finally bowel or bladder dysfunction.

Radiologic Evaluation

MRI scanning with and without gadolinium is the preferred study for diagnosis. Ependymomas typically are well circumscribed, located more centrally, enhance with contrast, and may have a rostral and caudal associated cyst. Astrocytomas on the other hand may be irregular, eccentric, and may or may not enhance with gadolinium.

Management

Surgery is the treatment of choice once a definitive diagnosis of intramedullary spinal cord tumor has been made. Biopsy is notoriously unreliable, and observation for evidence of tumor growth is not recom-mended. It is generally accepted that the most important factor in the postoperative outcome after surgery, is the pre-operative neurological status. Functional recovery is greatest in patients presenting with minimal neurological dysfunction, and dramatic improvement should not be expected in patients presenting with significant neurological deficits. Secondly, the shorter the duration of symptoms, the better the outcome after surgery and, thus the recommendation for early surgery versus long-term observation is usually made.


A 35-year-old female presented with a three-month history of intractable neck pain radiating to both shoulders and associated with bilateral upper extremity numbness and tingling. Neurological examination revealed motor weakness in both triceps muscles 3+/5, sensory exam revealed diminution to pin sensation over the tips of all the fingers. Deep tendon reflexes were 3+ brisk in all 4 extremities,

with a positive Hoffman’s reflex, without upgoing toes or clonus. Joint-position sense was preserved in the right hand and both lower extremities, but impaired in the left hand. MRI scans of the cervical spine revealed a large, partially cystic, enhancing intramedullary tumor at C2- C3 (Fig. 2.2). There was cord edema extending up the cervical cord and down into the thoracic spine.

Surgical Resection

The spinal cord was edematous and enlarged, distorting the normal anatomic landmarks. Dorsal column mapping revealed cord rotation with the midline located to the right side. A midline myelotomy was performed after which the pia-arachnoid layers were separated and sutured to the edges of the adjacent dura and, the dissec-tion carried to the tumor by separating the white matter tracts. A gross total removal of the tumor was achieved with the use of the operating microscope and the ultra-sonic aspirator. Continuous monitoring of the motor, somatosensory and “D” waves allowed us to prevent significant neural injury the histology was a tanycytic ependymoma.

Postoperatively, the patient had some weakness in both hands, but maintained good lower extremity strength with preservation of joint position sense. The patient had a month of inpatient rehabilitation prior to being discharged home. At follow-up, the patient had stiffness and some difficulty with fine movements in her hands but returned to her previous occupation working as a jeweler.

10


11


Figure 4.2. Axial MRI images revealed the presence of a chordoma with compression of the spinal cord and involvement of the vertebral body.

Surgery & Management

The optimal treatment for chordomas involving the axial skel-eton is surgery, with an attempt at gross total resection. Conventional radiation and chemotherapy are ineffective in chordomas. Proton-beam, heavy-particle radiation is usually offered after radical debulking of these tumors.

This patient underwent surgical resection of this tumor, using a combined anterolateral approach with anterior stabilization, followed by posterior stabilization (Fig. 4.3). The epidural tumor compressing the spinal cord, together with the involved vertebral bodies, was removed. (Fig. 4.4 & 4.5). Subsequently, a fusion was performed from C2 through C5.

Once stabilization and fusion were achieved, the wound was closed, and the patient was placed in a firm cervical collar. The physi-ologic monitoring remained stable throughout the procedure.

A transverse incision was utilized, from the mastoid tip in a curvilinear fashion over the lateral neck along a neck crease. The height of this incision was planned, based on known anatomical landmarks and pre-operative imaging. A long incision was used to allow adequate craniocaudal exposure. The dissection plane was carried through the platysma and along the anterior border of the sternocleidomastoid muscle (SCM), going between the internal jugular vein and the SCM (Fig. 4. 3).

We then came down upon the transverse processes in order to identify all spinal levels to be treated. The entire neurovascular bundle was individually dissected, including the vagus and hypoglossal nerves, as well as the sympathetic chain. Similarly, the accessory nerve was visualized (and adequately protected), as it turned around the lateral aspect of C1 underneath the internal jugular vein.

The large retropharyngeal component of the tumor was care-fully dissected away from the pharyngeal wall, and the tumor was removed. Special care was taken not to injure the pharyngeal wall and not to leave any tumor behind. The anterior scalenus muscle was identified, making sure that the phrenic nerve was not in the vicinity of the field.

Figure 4.1. Sagittal MRI images showing the chordoma behind bod-ies C2, C3, and C4, extending more to the right side and abutting the vertebral artery on the right. Vertebral body involvement is noted at C3 with no signal change in the C2 or C4 bodies.

Figure 3.4. Postoperative T2-weighted MRI demonstrated complete tumor removal and resolution of edema

Figure 3.5. Postoperative axial T1-weighted image showed a post-resection defect.

Follow-Up & Summary

No further adjuvant therapy was indicated. Spinal hemangio-blastomas are rare intramedullary tumors. These tumors present with similar symptoms as intramedullary astrocytomas and ependymomas.

MRI is helpful for the differential diagnosis. T1-weighted images often reveal homogeneous enhancing tumor with rostral/caudal cyst(s) and associated edema (Fig. 3.1). T2-weighted images typically demonstrate extensive edema, which is not seen in other intramedullary neoplasms (Fig. 3.2).

Angiograms are useful in defining the feeding and draining vessels (Fig. 3.3). Embolization of these feeding vessels is generally not needed prior to surgery. All these tumors are removed via posterior laminectomy. Patient symptoms tend to stabilize or improve after surgery. Adjuvant therapy is not indicated for these neoplasms.

REFERENCES

1. Roonprapunt C, Silvera VM, Setton A, Freed D, Epstein FJ, Jallo GI. Surgical management of isolated hemangioblastomas of the spinal cord. Neurosurgery. 2001; 49(2): 321-327.

2. Jallo GI, Roonprapunt C, Kothbauer K, Freed D, Allen J, Epstein FJ. Spinal solitary fibrous tumors: a series of four patients. Neurosurgery. 2005;57(1): E195.

3. Roonprapunt C, Houten J. Spinal Cord Astrocytomas: presenta-tion, management, and outcome. Neurosurg Clin North America. 2006;17(1): 29-36.

4. Roonprapunt C, Silvera VM, Jallo GI. Hemangioblastoma, spine. eMedicine from WebMD. Updated December 23, 2008. Available at: http://www.emedicine.com/radio/topic776.htm

CERVICAL CHORDOMAClinical Overview

Chordomas are low-grade primary malignant tumors aris-ing from notochordal remnants. They account for 1% to 4% of all malignant bone tumors. These tumors invade the cancellous bone and present as lytic lesions with areas of calcification. The tumor arises most commonly in the clivus (50%), next in frequency is the sacrum (35%), and less frequently in the spine (15%).

In the spine, the upper cervical spine and the lower lumbar spine are more frequently involved, only rarely involving the thoracic spine. Cervical spine involvement without involvement of the clivus is relatively uncommon and often presents management problems. The optimal management for chordomas of the spine with a contained tumor would be en bloc resection.

Unlike sacral chordomas, in which en bloc resection is the ultimate surgical goal, this may not be feasible in the cervical spine, due to the complex local anatomy. Additionally, by the time these tumors present clinically, they show spread into the epidural and paravertebral locations.


A 35-year-old man noted onset of intermittent neck pain over the course of three years. He described the pain as a mild ache, which periodically radiated into the right jaw. His pain was felt more intensely at night and upon flexion of the neck. Approximately three months after noticing pain, the patient began experiencing some numbness in both hands, accompanied by a bilateral tingling sensation in his fingertips.

Neurological Examination and Imaging Studies

Initial examination revealed a patient who was alert with normal mental status. He had no weakness or difficulty with gait or balance. Deep tendon reflexes were normal and symmetrical. No long-tract signs were detected. Cranial nerve examination was normal.

MRI scans revealed a tumor compressing the spinal cord with involvement of the vertebral body (Figs. 4.1 & 4.2). Transpharyngeal needle biopsy was performed at an outside institution, confirming the diagnosis of chordoma.

12


13


Figure 4.6. Illustration showing the vertebrectomy defect at C3, C4, and the fibular allograft strut with an anterior plate. The vertebral artery has been dissected out of the foramen transversarium posteriorly and the jugular vein anteriorly.

Figure 4.7. CT scan of the sagittal reconstruction showing the fibular allograft between C2 & C5, with the anterior translational plate from C2 to C5.

Figure 4.8. The patient had a posterior stabilization and fusion with lateral mass screws to supplement the anterior stabilization.

Anterior plating with screw fixation into the C2 and C5 bodies provided the necessary tension banding to hold the fibular graft in place (Fig. 4.6).

The patient underwent a posterior stabilization with lateral mass screws and rods from C2 to C5, preserving C1-2 and his rota-tional ability. (Figs. 4.7 & 4.8).

Postoperative Evaluation

The patient tolerated the procedure and returned home. He was able to get back to work as an emergency room physician in one month. Transient swallowing difficulty resolved in two weeks. An immediate postoperative MRI and CT scan were performed, and no residual tumor was noted, compared with the pre-operative scans. The patient received postoperative proton-beam therapy.

Figure 4.4. The vertebral artery is freed from the foramen transver-sarium and mobilized posteriorly to allow resection of the tumor within the vertebral body and in the canal.

Figure 4. 3. Illustration showing the neck dissection for a C3,4 chordoma with epidural tumor. The entire pharynx and esophagus are retracted medially. The carotid artery, jugular vein, and neural structures are retracted medially and anteriorly with wide retractors.

(Illustrations A, B, C, D modified from: Barrenechea IJ, Perin NI, Triana A, Lesser J, Costantino P, Sen C. Surgical management of chordomas of the cervical spine. J Neurosurg Spine. 2007;398-406, reprinted with permission from the American Association of Neurosurgeons).

Figure 4.5. Vertebral endplates are prepared at C2 and C5 following vertebrectomy of C3 and C4, for placement of the fibular strut graft .

Once the entire neurovascular bundle and pharynx were retracted anteriorly with wide retractors, the microscope was brought in for high-magnification dissection (Fig. 4.4). Depending on the number of levels to be treated, the vertebral artery was carefully freed from the foramen transversarium and mobilized posteriorly. During this maneuver, extreme care was taken to identify and preserve the sympathetic chain (Fig. 4.3).

Once the exposure was complete, the tumor was then removed in a piecemeal fashion down to the dura. We then continued in a cephalic and caudal direction and completely removed the involved vertebral bodies all the way across to the opposite vertebral artery.

This tumor involved all of the C3 vertebra allowing us to spare the C2 vertebra superiorly and the C5 vertebra inferiorly.

Reconstruction with Stabilization and Fusion

After completion of the anterior tumor resection, a bed-to-seat bone graft had to be established. The inferior end plate of C2 and the superior endplate of C5 were prepared. A fibular allograft was fashioned and placed between the endplates of C2 and C5 (Fig. 4.5).

14 15

Affiliated Departmental Professionals

Editorial: Spine & Spinal Cord Tumors

During the past few years, important changes in approaches to tumors of the spine have been made. These advances have generated improvements in the various surgical interventions applied to the entire vertebral column. Technical advances, including endoscopy and minimally invasive procedures, have made it routinely possible to resect spinal tumors successfully at all levels.

Advances in biomechanical engineering have also resulted in a vast array of technologically superb instrumentation, which allows for the reconstruction of the spine following resections of both benign and malignant tumors.

Similarly, advances in imaging technologies have also widened the ability of spinal surgeons to diagnose tumors and to visualize them in all their aspects. These new imaging techniques permit spinal surgeons to plan more precise approaches to tumor resection and to better assess the results of their interventions.

For many patients with primary malignancies of the spine, and for others with a solitary spinal metastasis, the most important goal of surgical intervention should be an attempt at local surgical “cure” by total resection. However, tumors often involve more than one area of the spine, and this situation may require a combined anterior-posterior approach or a staged procedure.

Following complicated surgery in some cases, adjuvant therapy (e.g., local irradiation) may be required to maximize the potential for a total cure. Systemic chemotherapy may also be advisable to eradicate an obvious or occult metastasis.

The spinal surgeon, then, is often the lead member of a multi-disciplinary team that may include medical and radiation oncologists and physical rehabilitation specialists. Such an expert team can help to assure the highest potential cure and survival rates for our patients.

Roosevelt Hospital provides expertise in all areas of surgical intervention, adjuvant therapies, and rehabilitation medicine. In the treatment of spine and spinal tumors, we are leading the way to the future.

Sincerely,

Charles J. Ippolito, MD

Charles J. IppolIto, MDEditor-In-Chief Issues in Clinical NeurosciencesDepartment of Neurosurgery

1000 Tenth Avenue , Suite 5G-80 New York, NY 10019 212-523-6097 [email protected]

Affiliates, Residents, Fellows & editorial

Daniel Yanni, MD Fellow, Minimally Invasive Spinal Surgery

Markus Chwajol, MD Chief: PGY-7

Sid Chandela, MD Chief: PGY-7

Adesh Tandon, MD PGY-6

Gaurav Jain, MD PGY-5

Chris Lenart, MD PGY-5

Lawrence Daniels, MD PGY-4

David Altschul, MD PGY-3

Alex Scheer, MD PGY-3

Residents & Fellows

NEUROLOGY

Joel Delfiner, MD 212-636-3236 Chairman

Eugene Pak, MD 212-636-3236

Carolyn Brockington, MD 212-636-3236

Virginia Moreno, MD 212-523-6521

CRANIAL BASE SURGERY & ENT

Yosef Krespi, MD Chairman 212-523-7791

DIAGNOSTIC NEURORADIOLOGY

Daniel Lefton, MD Attending, Neuroradiology 212-870-8421

HEAD AND NECK RADIOLOGY

Deborah Shatzkes. MD 212-523-7043

REHABILITATION

Malcolm Reid, MD 212-523-6595 Chairman

Sal Girardi, MD 212-523-6598 Assistant Chairman

NEURO-INTENSIVE CARE

Rup Swarup, MD Graciano Riviera, MD Armando Aloran, MD Daniel Dinescu, MD

NEURO-ANESTHESIA

Jonathan lesser, MDDirector, Anesthesiology

1000 Tenth Avenue, Suite 5C-03 New York, NY 10019 212-523-6121 [email protected]

NEURO-OPHTHALMOLOGY

Mark kupersMIth, MDDirector, Neuro-Ophthalmology

1000 Tenth Avenue, Suite 10G New York, NY 10019 212-636-3200 [email protected]

NEUROSURGERY

ChanDranath sen, MDChairman, Department of Neurosurgery, Co-Director, Center for Cranial Base Surgery

1000 Tenth Avenue, Suite 5G-80 New York, NY 10019 212-523-6720 [email protected]

GeorGe V. DIGIaCInto, MDGeneral Neurosurgery

425 W. 59th Street, Suite 4E New York, NY 10019 212-523-8500 [email protected]

DouGlas s. Cohen, MDGeneral Neurosurgery, Stereotactic Neurosurgery, Epilepsy, Spine Conditions

425 W. 59th Street, Suite 4E New York, NY 212-523-8502 [email protected]

DaVID J. lanGer, MDCerebrovascular Neurosurgery, Arteriovenous Malformations (Avm), Aneurysms, Benign Brain Tumors, Complex Spinal Surgery


erIC h. elowItz, MDSpine Neurosurgery, Co-Director, Center for Minimally Invasive and Endoscopic Spine Surgery

1000 Tenth Avenue, Suite 5G-44 New York, NY 10019 212-636-3660 [email protected] www.nycneurosurgery.com

noel I. perIn, MDSpine Neurosurgery, Director, Center for Minimally Invasive and Endoscopic Spine Surgery, Spine Tumors, Spinal Cord Tumors


raJ k. shrIVastaVa, MDBrain Tumors, Skull Base Surgery. Pituitary Tumors, Trigeminal Neuralgia, Spinal Cord Tumors, Epilepsy Surgery, Endoscopic Surgery


allen h. ManIker, MDChief Neurosurgery, Beth Israel Medical CenterDirector, Peripheral Nerve Institute

Beth Israel Medical Center 212-844-8383 [email protected]

arthur wIllIaMs, MDGeneral Neurosurgery,Neuro-Oncology


Chan roonprapunt, MD, phDAttending Neurosurgeon Division of Spine & Minimally Invasive Surgery


VeDran DeletIs, MD, phDMonitoring Neurophysiologist Associate Professor, Albert Einstein College of Medicine


CRANIAL BASE SURGERY & ENT

peter CostantIno, MDVice-Chairman, Department of Otolaryngology, Head and Neck Surgery, Craniofacial Tumor & Reconstructive Surgery, Facial Nerve Paralysis


ENDOVASCULAR SURGERY

aleJanDro BerensteIn, MDChief, Interventional Neuroradiology, Arteriovascular Malformations (avm)


YasunarI nIIMI, MDEndovascular Surgery, Aneurysms


staff


© 2009. Department of Neurosurgery. Roosevelt Hospital, Suite 5G-80, 1000 Tenth Avenue, New York, NY 10019

Code Number: 09-002 Printed: 03-08-2009

Bar codeRecipient NameRecipient TitleInstitution NameAddress 1Address2City, State, Zip

Online versions of all journal issues are available at: www.roosevelt-neurosurgery.com/publications/

Nonprofit Org.U.S. Postage

PAIDPermit #8048New York, NY

Leading the way to the future...

To reserve your place at this important meeting, register early now at: www.neurosymposium.com

2009 NEUROSYMPOSIUMCerebral Revascularization NYC

Thursday 27 August (8am to 4pm)Friday 28 August (8am to 6pm)

Saturday 29 August (by-invitation-only satellite ELANA user meeting)

Roosevelt Hospital 1000 Tenth Avenue New York, NY 10019

2nd Floor Conference Room

Announcing a ma jor international neurosurgery and interventional neuroradiology event

clınıcal neurosciences

Documents

spinal tumors

s s u e s i n

t r o o s e v e

t h o s p i t

r t e r

primary tumors

spinal column

inthecaseofskeletal