Clınıcal Neurosciences


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Clınıcal Neurosciences

  1. 1. Clınıcal Neurosciences Spine & Spinal Cord Tumors Volume 3 Number 1 Winter 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. 2. 2 Issues in Clinical Neurosciencestm 3 Spine & Spinal Cord Tumors Welcome Avarietyoftumors, both primary and second- ary, involves the spinal column. These tumors mayarisefrombone,nerve roots,meninges,orthespi- nal cord. Tumors of the 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 subsequentstabilization.Asubstantialdegreeofjudgmentandexperi- 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 optimizedwithanadvancedintensivecareandrehabilitativeprogram within our hospital. InourNeurosurgeryDepartment,wearefortunatetobeable toofferourpatientswithspineandspinalcordtumorsstate-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 1000 Tenth Avenue, Suite 5G-80 New York, NY 10019 All rights reserved. Graphic Design: State of the Art. Cover Illustration: iStock Images Online version: 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 theepidemiologyofspinaltumorsarediscussed.Clinicalevaluation, radiologicstudies,andotherdiagnosticproceduresarebroughtinto 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 intramedullaryspinalcordsurgeryarediscussedwithanillustrative 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 A49-year-oldfemalepatientwasdiagnosedwithacervicalheman- 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.
  3. 3. 4 Issues in Clinical Neurosciencestm 5 Spine & Spinal Cord Tumors 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 helpdecideifembolizationmightbeusefultohelpreducetheamount 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 rationaleuponwhichtobasetreatmentandprognosis.Inthisprocess, bothanatomicandhistologicdataareintegrated.Inthecaseofskeletal tumors,thestagingsystemdevisedbyEnnekinghasbeenquiteuseful. 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 areradiographicallymoreaggressiveinappearancebuthistologically 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” whenintracompartmentaland“B”whenextracompartmental.Using thissystem,malignantbonetumorsareclassifiedasIA,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. SundaresanN,DiGiacintoGVD,HughesJEO.Surgicaltreatment 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 Introduction Lesions of the Osseous Spine Themoleculartransformationtomalignantcellsisamultistep process involving the deregulation of normal cellular growth to unbridled proliferation. Factors involved in the deregulation process arestillunderinvestigationandincludelossoftumorsuppressorgenes and the presence of activated oncogenes. Taking into account the multifactorialmolecularbasisofthistransformationfromprecancer- 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 commonsiteformetastasisfromdistantprimarymalignancies.2 These malignanciescanincludelung,breast,prostate,kidney,andGItract. Primary Neoplasms Primary neoplasms of the spine tend to be rare, and several largestudieshaveshownthatskeletaltumorsaroseinonly11%ofall 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,shouldberuledoutinthedifferentialdiagnosisof atypical symptoms often presenting as spondylosis.4 Epidemiology of Spinal Tumors TheAmericanCancerSocietyhasreportedthatapproximately 2000newcasesofbonecancerand6000casesofsofttissuesarcomas are diagnosed each year, with about 5% involving the spine. Certain tumorsariseprimarilyinthevertebralcolumn(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,andthehematopoeiticsystem.Thetendencyof sometumorstometastasizetothespineandskeletalsystemisknown as osteotropism.4 Pathogenesis of Spinal Tumors Embryonicoriginisthemaindifferentiatorintheclassification ofprimarytumorsofthespine.Thisclassificationestablishesthedif- 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 histologicarchitecture.Tumorsthatarederivedfromfibroblasts(e.g., fibrous histiocytoma, fibrosarcoma, fibroma) feature collagen (see Table).Bydefinition,theosteoclastdestroysbone;itistheprogenitor to the giant cell tumor. Inthemajorityofpatientswithcancer,metastasistothespineis generallyaccomplishedviathehematogenousroute.Inthisparadigm, 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- ferentialdiagnosisdifficult.Adistinguishingfactorindifferentiating the pain involved in these two conditions is that tumor pain seldom improveswithrestandmayactuallygetworseatnightwithpatientsin thesupineposition.In80%to100%ofprimaryneoplasms,pain(the cardinal symptom of spinal tumors) can often be the only symptom at the time of initial diagnosis. Alongwithpain,neurologicdeficitsareoftenpathognomonic and frequently develop when a tumor compresses or infiltrates the spinalcord,thenerveroots,orparaspinalnerveplexuses.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 axialandradicularpain(withorwithoutneurologicdeficits)willhave an MRI scan done as the initial imaging study. If a primary or meta- staticbonetumorisnotedonMRI,aCTscanisobtainedtoevaluate thebonyanatomyandbonedestruction.Ifthelesionisthoughttobe metastatic in nature, a metastatic workup should be initiated in the absence of a known primary tumor
  4. 4. 6 Issues in Clinical Neurosciencestm 7 Spine Spinal Cord Tumors 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 #1 Chief 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.Neurologicalexaminationshowedanantalgicgait,aspastic 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 intramedullarytumoratT7-T8enlargingthespinalcord.Thetumor wasisointenseonT1,hyperintenseonT2,withoutenhancementwith 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 midlinemacroscopically.Byusingthestripelectrodefordorsalcolumn mapping (Fig. 1.2), the neurophysiology team was able to locate the anatomicmidlineaslyingbetweenelectrodes6and7.Themyelotomy was placed at the selected site, and a gross total removal of the tumor was achieved. The final pathology was gangliocytoma. Postoperatively,thepatienthadtransientincreasedweaknessin therightlowerextremity,butgoodstrengthintheleft.Jointposition sensewaspreservedontheleftandabsentontheright,andthepatient wasstablefromherpre-operativeexam.Atfollow-up,herrightlower extremity strength continued to improve with physical therapy. REFERENCES 1. Costa P, Bruno A, Bonzanino M, Massaro F, Caruso L, Vincenzo I,CiaramitaroP,MontalentiE.Somatosensory-andmotor-evoked potential monitoring during spine and spinal cord surgery. Spinal Cord. 2007;45(1):86-91. 2. Deletis V, De Camargo BA. Interventional neurophysiological mappingduringspinalcordprocedures.StereotactFunctNeurosurg. 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 microsurgicaltreatmentforintramedullaryspinalcordependymo- 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 intramedullaryspinalcordtumors:theroleofintraoperative(neu- rophysiological) monitoring. Eur Spine J. 2007;16 Suppl 2:S130-9. 10. Shrivastava RK, Epstein FJ, Perin NI, Post KD, Jallo GI. Intramedullaryspinalcordtumorsinpatientsolderthan50years 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 SURGERY Clinical Overview TheNeurosciencesGroupatRooseveltHospitalworksclosely withtheprestigiousgroupofneurophysiologistsledbyVedrenDeletis, MD, PhD. Dr. Deletis is one of the pioneers in the development of motor-evokedpotentialmonitoringinspinalcordsurgery.Hegained extensiveexperienceworkingwiththelateDr.FredEpsteinonmany hundreds of spinal cord tumor surgeries ay NYU Medical Center, thenatBeth-IsraelNorth,andnowattheRooseveltMedicalCenter. Wecollaboratecloselywiththeneurophysiologyteamandhave developed many techniques to monitor neural tracts to enable us to performspinalsurgerymorepreciselyandsafely.Morerecently,using dorsal column mapping to identify the physiological and functional midline of the spinal cord for myelotomy, this technique has enabled ustominimizeinjurytothedorsalcolumns.Allpatientsundergoing intramedullary surgery have motor-evoked potential monitoring, somatosensory-evokedpotentialmonitoring,dorsalcolumnmapping, 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,whereasependymomasaremorefrequentlyencountered intheadultpopulation.Thesetumorsareveryslowgrowingandcan reachsignificantproportionswithinthespinalcordbeforebecoming symptomatic.Theytendtoexpandthespinalcordandcandistortthe surface anatomy.4,5 Surgicalresectionisthedefinitivetreatmentforintramedullary spinalcordtumors.4,5,6,7 Resectionoflargecentrallylocatedintramed- ullary spinal cord tumors is achieved via a midline myelotomy. The midlineinanormalcordisthedorsalmediansulcus,locatedbetween 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,thisanatomyisfrequentlydistortedincasesoftumor, due to edema, neovascularization, or scar formation. The distortion canbeacombinationofrotationofthecordandasymmetricenlarge- ment,makingidentificationofthesurfaceanatomyextremelydifficult (Fig.1.1).Inadvertentdissectionthroughthedorsalcolumnswillcause 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 postoperativemorbidityfollowingmyelotomyforspinalcordtumors, reportedin43.6%ofpatientsinoneseries.7 Manyauthorsbelievethat 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 themidlineviastandardanatomicallandmarkswithoutanyobjective neurophysiologicaldata.Injurytothedorsalcolumnsduringthisdis- section can result in dysfunction manifesting as numbness, tingling, painful dysesthesias, or ataxic gait.8 This can be significantly incapacitating on the patient’s functionalstatusandcanaffecttheabilitytorehabilitate.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 Togetherwiththestandardpre-operativeradiographicstudies and intra-operative ultrasound to identify the exact location of the tumorwithinthespinalcord,weutilizedanintra-operativefunctional technique of mapping the dorsal columns to help locate the midline forthemyelotomy.6,8 Thisisaccomplishedbydefiningtheamplitude gradientofconductedsomatosensory-evokedpotentials(SEPs)usinga miniaturemulti-electrodegrid(Fig.1.2).Thesesignalsareinterpreted intra-operativelybytheneurophysiologyteamcorrelatingthesurgical anatomywiththefunctionalanatomy.Wehavefoundthistechnique particularly useful in patients with large intramedullary spinal cord tumors and syringomyelia.9,10,11
  5. 5. 8 Issues in Clinical Neurosciencestm 9 Spine Spinal Cord Tumors This sensory deficit improved when compared to her pre- operativestatus.Postoperativeimagingrevealedgrosstotalresection with significant reduction in intramedullary edema formation (Figs. 3.4 3.5). No further adjuvant therapy was indicated. Discussion Hemangioblastomasarevasculartumors,whichcanbefound throughouttheneuraxis.Theymayoccurassporadicisolatedlesions or multiple lesions in the cerebellum and retina and are often associ- atedwiththedominantlyinheritedfamilialcancersyndromeknown as von Hippel Lindau disease. Although considered histologically benign, hemangioblasto- masmaycausesignificantneurologicaldeficits,especiallywhenthey presentinthespinalcord,wheretheycompriseapproximately3%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 thedifferentialdiagnosis.T1-weightedimagesoftenrevealhomoge- 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 Hemangioblastoma Chief Complaint, Presentation, and History of Present Illness A 49-year-old female presented to her primary care physician withaseveral-monthhistoryofneckpainandtinglinginherfingers. Hersymptomswereinitiallyattributedtocarpaltunnelsyndrome.An MRIscanwasperformedwhenherdysesthesiasprogressedproximally 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 hypervasculartumorreceiveditsbloodsupplyfromthelateralspinal arteries, and drainage through posterior surface veins. Surgical Approach and Results AC1-3laminectomywasperformed,withgrosstotalresection of the neoplasm. Intra-operative somatosensory-evoked poten- tials and motor-evoked potentials were utilized during resection. Postoperatively,shehadatransientincreaseinhersensorydeficit,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 TUMOR Clinical 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. Intermsofclinicalpresentation,mostpatientshavepaineither 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, locatedmorecentrally,enhancewithcontrast,andmayhavearostral and caudal associated cyst. Astrocytomas on the other hand may be irregular, eccentric, and may or may not enhance with gadolinium. Management Surgeryisthetreatmentofchoiceonceadefinitivediagnosisof intramedullaryspinalcordtumorhasbeenmade.Biopsyisnotoriously unreliable,andobservationforevidenceoftumorgrowthisnotrecom- mended.Itisgenerallyacceptedthatthemostimportantfactorinthe postoperativeoutcomeaftersurgery,isthepre-operativeneurological status. Functional recovery is greatest in patients presenting with minimalneurologicaldysfunction,anddramaticimprovementshould 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. CASE PRESENTATION #2 Chief Complaint, Presentation, and History of Present Illness 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, sensoryexamrevealeddiminutiontopinsensationoverthetipsofall 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 myelotomywasperformedafterwhichthepia-arachnoidlayerswere separatedandsuturedtotheedgesoftheadjacentduraand,thedissec- tioncarriedtothetumorbyseparatingthewhitemattertracts.Agross totalremovalofthetumorwasachievedwiththeuseoftheoperating 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,thepatienthadsomeweaknessinbothhands, but maintained good lower extremity strength with preservation of jointpositionsense.Thepatienthadamonthofinpatientrehabilitation priortobeingdischargedhome.Atfollow-up,thepatienthadstiffness andsomedifficultywithfinemovementsinherhandsbutreturnedto her previous occupation working as a jeweler.
  6. 6. 10 Issues in Clinical Neurosciencestm 11 Spine Spinal Cord Tumors 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 Theoptimaltreatmentforchordomasinvolvingtheaxialskel- etonissurgery,withanattemptatgrosstotalresection.Conventional radiation and chemotherapy are ineffective in chordomas. Proton- beam, heavy-particle radiation is usually offered after radical debulking of these tumors. Thispatientunderwentsurgicalresectionofthistumor,usinga combinedanterolateralapproachwithanteriorstabilization,followed byposteriorstabilization(Fig.4.3).Theepiduraltumorcompressing the spinal cord, together with the involved vertebral bodies, was removed.(Fig.4.44.5).Subsequently,afusionwasperformedfrom C2 through C5. Once stabilization and fusion were achieved, the wound was closed,andthepatientwasplacedinafirmcervicalcollar.Thephysi- 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 sternocleidomastoidmuscle(SCM),goingbetweentheinternaljugular vein and the SCM (Fig. 4. 3). Wethencamedownuponthetransverseprocessesinorderto identifyallspinallevelstobetreated.Theentireneurovascularbundle wasindividuallydissected,includingthevagusandhypoglossalnerves, 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. Thelargeretropharyngealcomponentofthetumorwascare- 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- blastomasarerareintramedullarytumors.Thesetumorspresentwith similarsymptomsasintramedullaryastrocytomasandependymomas. 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).Embolizationofthesefeedingvesselsisgenerallynot 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. eMedicinefromWebMD.UpdatedDecember23,2008.Available at: CERVICAL CHORDOMA Clinical 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 andpresentaslyticlesionswithareasofcalcification.Thetumorarises 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 spinearemorefrequentlyinvolved,onlyrarelyinvolvingthethoracic spine.Cervicalspineinvolvementwithoutinvolvementoftheclivusis relativelyuncommonandoftenpresentsmanagementproblems.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. CASE PRESENTATION #4 Chief Complaint, Presentation, and History of Present Illness A 35-year-old man noted onset of intermittent neck pain over thecourseofthreeyears.Hedescribedthepainasamildache,which periodicallyradiatedintotherightjaw.Hispainwasfeltmoreintensely 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-tractsignsweredetected.Cranialnerveexaminationwasnormal. MRIscansrevealedatumorcompressingthespinalcordwith involvementofthevertebralbody(Figs.4.14.2).Transpharyngeal needlebiopsywasperformedatanoutsideinstitution,confirmingthe diagnosis of chordoma.
  7. 7. 12 Issues in Clinical Neurosciencestm 13 Spine Spinal Cord Tumors 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. AnteriorplatingwithscrewfixationintotheC2andC5bodies 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 immediatepostoperativeMRIandCTscanwereperformed,andno residualtumorwasnoted,comparedwiththepre-operativescans.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 retractedanteriorlywithwideretractors,themicroscopewasbrought in for high-magnification dissection (Fig. 4.4). Depending on the numberoflevelstobetreated,thevertebralarterywascarefullyfreed from the foramen transversarium and mobilized posteriorly. During this maneuver, extreme care was taken to identify and preserve the sympathetic chain (Fig. 4.3). Oncetheexposurewascomplete,thetumorwasthenremoved 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. ThistumorinvolvedalloftheC3vertebraallowingustospare the C2 vertebra superiorly and the C5 vertebra inferiorly. Reconstruction with Stabilization and Fusion Aftercompletionoftheanteriortumorresection,abed-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).
  8. 8. 14 15 Affiliated Departmental Professionals Editorial: Spine Spinal Cord Tumors Duringthepastfewyears,importantchangesinapproachesto 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. Advancesinbiomechanicalengineeringhavealsoresultedina vastarrayoftechnologicallysuperbinstrumentation,whichallowsfor the reconstruction of the spine following resections of both benign and malignant tumors. Similarly,advancesinimagingtechnologieshavealsowidened theabilityofspinalsurgeonstodiagnosetumorsandtovisualizethem 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. Formanypatientswithprimarymalignanciesofthespine,and forotherswithasolitaryspinalmetastasis,themostimportantgoalof surgical intervention should be an attempt at local surgical “cure” by totalresection.However,tumorsofteninvolvemorethanoneareaof thespine,andthissituationmayrequireacombinedanterior-posterior approach or a staged procedure. Followingcomplicatedsurgeryinsomecases,adjuvanttherapy (e.g.,localirradiation)mayberequiredtomaximizethepotentialfora totalcure.Systemicchemotherapymayalsobeadvisabletoeradicate an obvious or occult metastasis. Thespinalsurgeon,then,isoftentheleadmemberofamulti- disciplinaryteamthatmayincludemedicalandradiationoncologists and physical rehabilitation specialists. Such an expert team can help toassurethehighestpotentialcureandsurvivalratesforourpatients. 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, MD Editor-In-Chief Issues in Clinical Neurosciences Department of Neurosurgery 1000 Tenth Avenue , Suite 5G-80 New York, NY 10019 212-523-6097 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, MD Director, Anesthesiology 1000 Tenth Avenue, Suite 5C-03 New York, NY 10019 212-523-6121 NEURO-OPHTHALMOLOGY Mark Kupersmith, MD Director, Neuro-Ophthalmology 1000 Tenth Avenue, Suite 10G New York, NY 10019 212-636-3200 Neurosurgery Chandranath Sen, MD Chairman, Department of Neurosurgery, Co-Director, Center for Cranial Base Surgery 1000 Tenth Avenue, Suite 5G-80 New York, NY 10019 212-523-6720 George V. DiGiacinto, MD General Neurosurgery 425 W. 59th Street, Suite 4E New York, NY 10019 212-523-8500 Douglas S. Cohen, MD General Neurosurgery, Stereotactic Neurosurgery, Epilepsy, Spine Conditions 425 W. 59th Street, Suite 4E New York, NY 212-523-8502 David J. Langer, MD Cerebrovascular Neurosurgery, Arteriovenous Malformations (Avm), Aneurysms, Benign Brain Tumors, Complex Spinal Surgery 1000 Tenth Avenue, Suite 5G-49 New York, NY 10019 212-636-3204 Eric H. Elowitz, MD Spine Neurosurgery, Co-Director, Center for Minimally Invasive and Endoscopic Spine Surgery 1000 Tenth Avenue, Suite 5G-44 New York, NY 10019 212-636-3660 Noel I. Perin, MD Spine Neurosurgery, Director, Center for Minimally Invasive and Endoscopic Spine Surgery, Spine Tumors, Spinal Cord Tumors 1000 Tenth Avenue, Suite 5G-80 New York, NY 10019 212-523-6720 Raj K. Shrivastava, MD Brain Tumors, Skull Base Surgery. Pituitary Tumors, Trigeminal Neuralgia, Spinal Cord Tumors, Epilepsy Surgery, Endoscopic Surgery 1000 Tenth Avenue, Suite 5G-48 New York, NY 10019 212-636-3119 Allen H. Maniker, MD Chief Neurosurgery, Beth Israel Medical Center Director, Peripheral Nerve Institute Beth Israel Medical Center 212-844-8383 Arthur Williams, MD General Neurosurgery, Neuro-Oncology 1000 Tenth Avenue, Suite 5G-45 New York, NY 10019 212-636-3119 Chan Roonprapunt, MD, PhD Attending Neurosurgeon Division of Spine Minimally Invasive Surgery 1000 Tenth Avenue, Suite 5G-80 New York, NY 10019 212-523-6720 Vedran Deletis, MD, PhD Monitoring Neurophysiologist Associate Professor, Albert Einstein College of Medicine 1000 Tenth Avenue, Suite 5G-45 New York, NY 10019 212-870-9686 CRANIAL BASE SURGERY ENT Peter Costantino, MD Vice-Chairman, Department of Otolaryngology, Head and Neck Surgery, Craniofacial Tumor Reconstructive Surgery, Facial Nerve Paralysis 1000 Tenth Avenue, Suite 5G-80 New York, NY 10019 212-262-4444 ENDOVASCULAR SURGERY Alejandro Berenstein, MD Chief, Interventional Neuroradiology, Arteriovascular Malformations (avm) 1000 Tenth Avenue, Suite 10G New York, NY 10019 212-636-3400 Yasunari Niimi, MD Endovascular Surgery, Aneurysms 1000 Tenth Avenue, Suite 10G New York, NY 10019 212-636-3400 Staff
  9. 9. Issues in Clinical Neurosciencestm © 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 code Recipient Name Recipient Title Institution Name Address 1 Address2 City, State, Zip Online versions of all journal issues are available at: Nonprofit Org. U.S. Postage PAID Permit #8048 New York, NY Leading the way to the future... To reserve your place at this important meeting, register early now at: 2009 NEUROSYMPOSIUM Cerebral 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 major international neurosurgery and interventional neuroradiology event