Intramedullary Spinal Cord Tumors

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  • Intramedullary Spinal Cord Tumors

    1. 1. Intramedullary Spinal Cord Tumors Leslie Hutchins, MD Shore Memorial Cancer Conference September 18, 2008
    2. 2. Spinal Cord Anatomy- Meninges <ul><li>Protective Sheath </li></ul><ul><ul><li>Duramater </li></ul></ul><ul><ul><li>Arachnoid </li></ul></ul><ul><ul><li>Piamater </li></ul></ul><ul><li>Potential Space </li></ul><ul><ul><li>Subdural Space </li></ul></ul><ul><ul><ul><li>Located between the Dura & Arachnoid </li></ul></ul></ul><ul><ul><li>Subarachnoid Space </li></ul></ul><ul><ul><ul><li>The Cerebrospinal Fluid flows between the Arachnoid & Piamater in this space. </li></ul></ul></ul>
    3. 3. Spinal Cord Anatomy <ul><li>Begins </li></ul><ul><ul><li>Foramen Magnum as a continuation of the Medulla Oblongata </li></ul></ul><ul><li>Terminates </li></ul><ul><ul><li>Conus Medullaris </li></ul></ul><ul><ul><ul><li>Adult: Lower Border of L1 </li></ul></ul></ul><ul><ul><ul><li>Young Child: Upper Border of L3 </li></ul></ul></ul><ul><li>Filum Terminale </li></ul><ul><ul><li>Prolongation of the piamater </li></ul></ul><ul><ul><li>Attaches to the spinal cord at the coccyx </li></ul></ul><ul><li>Dural Sac </li></ul><ul><ul><li>Ends at the level of the second sacral vertebra </li></ul></ul>
    4. 4. Spinal Cord Anatomy <ul><li>Gray Matter </li></ul><ul><ul><li>Ventral (motor) Horns </li></ul></ul><ul><ul><li>Dorsal (sensory) Horns </li></ul></ul><ul><li>White Matter </li></ul><ul><ul><li>Anterior Columns </li></ul></ul><ul><ul><li>Lateral Columns </li></ul></ul><ul><ul><li>Posterior Columns </li></ul></ul>D.E. Haines, Neuroanatomy: An Atlas of Structures, Sections, and Systems, 3rd ed. (1991), Urban & Schwarzenberg Encyclopedia Britannica, Inc.
    5. 5. Spinal Cord Anatomy <ul><li>31 pairs of spinal nerves (Each formed by 2 roots) </li></ul><ul><li>The spinal nerves exit through the intervebral foramen </li></ul><ul><ul><li>8 cervical </li></ul></ul><ul><ul><ul><li>1 st exits through the Occipital Bone and C 1 </li></ul></ul></ul><ul><ul><ul><li>8 th exits between C7 & T 1 </li></ul></ul></ul><ul><ul><li>1 2 thoracic </li></ul></ul><ul><ul><ul><li>Distal to T 1 each spinal nerve exits below its corresponding vertebra </li></ul></ul></ul><ul><ul><li>5 lumbar </li></ul></ul><ul><ul><li>5 sacral </li></ul></ul><ul><ul><li>1 coccygeal </li></ul></ul>
    6. 6. Motor impairment related to Spinal Cord Injury
    7. 7. Dermatomes
    8. 8. Spinal Cord Cross Section: Fiber Tracts
    9. 9. Spinal Cord Anatomy: Vasculature <ul><li>Upper Cervical Spine </li></ul><ul><ul><li>Vertebral Artery: </li></ul></ul><ul><ul><ul><li>One Anterior Spinal Artery </li></ul></ul></ul><ul><ul><ul><li>Two Posterior Spinal Arteries </li></ul></ul></ul>
    10. 10. Spinal Cord Anatomy: Vasculature <ul><li>Lower Third of C-Spine: </li></ul><ul><ul><li>Anterior Segmental Medullary (Radicular) Arteries </li></ul></ul><ul><ul><ul><li>Vertebral Artery </li></ul></ul></ul><ul><ul><ul><li>Ascending Cervical Artery </li></ul></ul></ul><ul><ul><ul><li>Deep Cervical Artery </li></ul></ul></ul>
    11. 11. <ul><li>1: posterior spinal vein 2: anterior spinal vein 3: posterolateral spinal vein 4: radicular (or segmental medullary) vein 5: posterior spinal arteries 6: anterior spinal artery 7: radicular (or segmental medullary) artery </li></ul>Spinal Cord Anatomy: Vasculature
    12. 12. Spinal Cord Anatomy: Vasculature <ul><li>Below C-Spine </li></ul><ul><ul><li>Continuous anastomoses with the radicular arteries. </li></ul></ul><ul><ul><ul><li>Aorta </li></ul></ul></ul><ul><ul><ul><li>Intercostal Arteries </li></ul></ul></ul><ul><ul><ul><li>Spinal Arteries </li></ul></ul></ul><ul><ul><ul><li>Anterior & Posterior Radicular Arteries </li></ul></ul></ul>
    13. 13. Spinal Cord Anatomy: Vasculature <ul><li>Thoracic/Lumbar Spine </li></ul><ul><ul><li>Anterior Segmental Arteries Alternate Sides of Cord </li></ul></ul><ul><ul><li>Artery of Adamkiewicz </li></ul></ul><ul><ul><ul><li>Major Blood Supply to Lower Thoracic & Lumbar Spine </li></ul></ul></ul><ul><ul><ul><li>Arises on the Left ~78% </li></ul></ul></ul><ul><ul><ul><li>Enters single intervertebral foramen between T8 & L3 </li></ul></ul></ul>
    14. 14. Spinal Cord Anatomy: Vasculature <ul><li>Batson’s Plexus: </li></ul><ul><ul><li>&quot;In brief, the vertebral veins are a valveless plexiform network with a longitudinal pattern. They parallel and communicate with the superior and inferior venae cavae. The plexus extends the entire length of the vertebral column and finds a cranial terminus in the dural sinuses.&quot; -Batson </li></ul></ul><ul><li>Multiple anastomoses with: </li></ul><ul><ul><li>Azygous system </li></ul></ul><ul><ul><li>IVC </li></ul></ul><ul><ul><li>Pelvic plexus </li></ul></ul><ul><ul><li>Prostatic plexus </li></ul></ul>
    15. 15. Classification of Spinal Cord Lesions <ul><li>Extradural </li></ul><ul><ul><li>Outside the thecal sac (including vertebral bone lesions) </li></ul></ul><ul><li>Intradural/Extramedullary </li></ul><ul><ul><li>Within thecal sac, but outside the cord </li></ul></ul><ul><li>Intramedullary </li></ul><ul><ul><li>Within cord </li></ul></ul>
    16. 16. Classification of Spinal Cord Lesions
    17. 17. Pediatric Intramedullary Spinal Cord Tumors <ul><li>4-6% of Pediatric CNS Neoplasms </li></ul><ul><li>Astrocytomas </li></ul><ul><ul><li>60% of all Pediatric IMSCTs </li></ul></ul><ul><ul><li>10-15% are High Grade </li></ul></ul><ul><li>Ependymomas </li></ul><ul><li>Miscellaneous </li></ul><ul><ul><li>Teratomas </li></ul></ul><ul><ul><li>Dermoid </li></ul></ul><ul><ul><li>Epidermoid Cysts </li></ul></ul><ul><ul><li>Oligodendrogliomas </li></ul></ul><ul><ul><li>Schwannomas </li></ul></ul><ul><ul><li>Neurofibromas </li></ul></ul><ul><ul><li>Subependymomas </li></ul></ul><ul><ul><li>Neurocytomas </li></ul></ul><ul><ul><li>Metastases </li></ul></ul>
    18. 18. Presentation <ul><li>Low Grade </li></ul><ul><ul><li>Asymptomatic </li></ul></ul><ul><ul><li>Subtle Presentation </li></ul></ul><ul><ul><li>Symptoms to Diagnosis </li></ul></ul><ul><ul><ul><li>9.2 months </li></ul></ul></ul><ul><li>High Grade </li></ul><ul><ul><li>Symptoms to Diagnosis </li></ul></ul><ul><ul><ul><li>7 weeks to 4 months </li></ul></ul></ul>
    19. 19. Clinical Manifestations <ul><li>Back or neck pain </li></ul><ul><ul><li>Dull </li></ul></ul><ul><ul><li>Aching </li></ul></ul><ul><ul><li>Diffuse </li></ul></ul><ul><ul><li>Referable to spinal axis involved </li></ul></ul><ul><ul><li>Worse at night </li></ul></ul><ul><ul><ul><li>Venous congestion & dural distension caused by recumbent position </li></ul></ul></ul><ul><ul><ul><li>Meningeal Irritation </li></ul></ul></ul><ul><li>Weakness </li></ul><ul><li>Paraesthesias </li></ul><ul><li>Spinal Deformity </li></ul><ul><li>Slow neurologic deterioration </li></ul><ul><li>Dysesthesias </li></ul><ul><ul><li>20% </li></ul></ul><ul><ul><li>Usually asymmetrical in glial tumors </li></ul></ul><ul><li>Sphincter dysfunction </li></ul><ul><ul><li>Bowel/Bladder dysfunction is rare unless late in clinical course </li></ul></ul><ul><ul><li>Exception: Conus Medullaris Lesions </li></ul></ul><ul><li>Motor </li></ul><ul><ul><li>Delay in Milestones </li></ul></ul><ul><ul><ul><li>(i.e. Crawling/Walking) </li></ul></ul></ul><ul><ul><li>Motor Regression </li></ul></ul><ul><ul><li>Gait abnormality </li></ul></ul><ul><ul><li>Frequent falls </li></ul></ul>
    20. 20. <ul><li>Steven is 17 and the second of five boys in our family. He has always been a great guy with drive and ambition, honesty, and straight A's. Most importantly, he has a great big, kind heart. Steven was 16 in the spring of 2007. </li></ul><ul><li>He was enjoying a workout with his friends from DeSmet Jesuit High School when he noticed his hands and lower arms going numb. Steven also had pain every night while he was trying to sleep and it was increasing.  Amazingly, these were his only symptoms. </li></ul>
    21. 21. Clinical Exam <ul><li>Mild to Moderate Motor Deficit </li></ul><ul><li>Upper Motor Neuron Signs </li></ul><ul><ul><li>Spasticity </li></ul></ul><ul><ul><li>Hyperreflexia </li></ul></ul><ul><ul><li>Clonus </li></ul></ul><ul><ul><li>Increase muscle tone </li></ul></ul>
    22. 22. Kyphoscoliosis
    23. 23. Torticollis
    24. 24. Hydrocephalus <ul><li>Associated with 15% of children with IMSCTs </li></ul><ul><ul><li>Substantially higher in patients with malignant IMSCTs (35%) </li></ul></ul><ul><ul><ul><li>Increase Concentration of Protein in the CSF </li></ul></ul></ul><ul><ul><ul><li>Arachnoidal fibrosis </li></ul></ul></ul><ul><ul><ul><li>Subarachnoid dissemination </li></ul></ul></ul><ul><ul><ul><li>4 th Ventricle outlet obstruction </li></ul></ul></ul>
    25. 25. Work-up <ul><li>MRI (Study of Choice) </li></ul><ul><ul><li>With and without gadolinium </li></ul></ul><ul><li>Plain Radiographs </li></ul><ul><ul><li>Scoliosis </li></ul></ul><ul><ul><li>Post-op deformity </li></ul></ul><ul><li>CT </li></ul><ul><ul><li>Bony involvement is rare </li></ul></ul><ul><ul><li>Only obtained to follow spinal deformity or if MRI is contraindicated </li></ul></ul>
    26. 26. Ependymomas <ul><li>Subependymoma </li></ul><ul><ul><li>WHO Grade 1 </li></ul></ul><ul><ul><li>Slow Growing </li></ul></ul><ul><ul><li>Detected Incidentally </li></ul></ul><ul><ul><li>Good Prognosis </li></ul></ul><ul><li>Myxopapillary Ependymoma </li></ul><ul><ul><li>WHO Grade 1 </li></ul></ul><ul><ul><li>Arise in the filum terminale or conus medullaris </li></ul></ul>
    27. 27. Ependymomas <ul><li>Ependymoma </li></ul><ul><ul><li>WHO Grade II </li></ul></ul><ul><li>Anaplastic </li></ul><ul><ul><li>WHO Grade III </li></ul></ul><ul><ul><li>Are thought to arise from malignant transformation of low-grade ependymomas </li></ul></ul><ul><li>Glioblastoma Multiforme </li></ul><ul><ul><li>WHO Grade 4 </li></ul></ul>
    28. 28. Myxopapillary Ependymomas <ul><li>Abundant Mucin Production, GFAP Immunoreactivity, </li></ul><ul><li>Rare mitosis, & Cuboidal or elongated morphology </li></ul>
    29. 29. Ependymomas <ul><li>Highly cellular tumors with rare mitotic activity. </li></ul><ul><li>GFAP Immunoreactivity, Perivascular Psuedorosettes, & Occasional Ependymal Rosettes </li></ul>
    30. 30. Anaplastic Ependymomas <ul><li>Frequent mitosis, Necrosis, Neovascularization, & Increased Cellularity </li></ul>
    31. 31. Ependymoma <ul><li>Centrally located </li></ul><ul><li>3-4 Vertebral Bodies </li></ul><ul><li>Defined Borders </li></ul><ul><li>Enhance brightly & homogenously </li></ul><ul><li>Cystic components </li></ul>
    32. 32. Nineteen year-old who presented with finger numbness & “clumsiness”
    33. 33. Ependymoma Kothbauer KF. Neurosurgical Management of Intramedullary Spinal Cord Tumors in Children. Pediatric Neurosurgery. 2007; 43:222-235.
    34. 34. www.stevenscordfoundation.com
    35. 35. Astrocytoma <ul><li>Pathogenesis Unclear </li></ul><ul><ul><li>Genetic disorders associated with family clustering of IMSCTs </li></ul></ul><ul><ul><ul><li>Li Fraumeni syndrome </li></ul></ul></ul><ul><ul><ul><li>Turcot syndrome </li></ul></ul></ul><ul><ul><ul><li>Tuberous sclerosis </li></ul></ul></ul><ul><ul><ul><li>Multiple enchondromatosis </li></ul></ul></ul><ul><ul><ul><li>NF </li></ul></ul></ul><ul><ul><li>Radiation Induced Astrocytomas </li></ul></ul>
    36. 36. Astrocytoma <ul><li>Pilocytic Astrocytoma </li></ul><ul><ul><li>WHO Grade I </li></ul></ul><ul><ul><li>Most Common </li></ul></ul><ul><li>Fibrillary Astrocytoma </li></ul><ul><ul><li>WHO Grade II </li></ul></ul><ul><ul><li>Most Common </li></ul></ul><ul><li>Anaplastic Astrocytoma </li></ul><ul><ul><li>WHO Grade III </li></ul></ul><ul><li>GBM </li></ul><ul><ul><li>WHO Grade IV </li></ul></ul>
    37. 37. Pilocytic Astrocytoma <ul><li>Elongated bipolar astrocytes, Intracytoplasmic Rosenthal fibers, </li></ul><ul><li>& Eosinophilic granular bodies </li></ul>
    38. 38. Fibrillary Astrocytomas <ul><li>Infiltrative tumors, Mild nuclear pleomorphism, & Hypercellularity with mitosis </li></ul>
    39. 39. Astrocytomas <ul><li>Eccentrically located </li></ul><ul><li>Produce symmetric enlargement </li></ul><ul><li>Ill-defined borders </li></ul><ul><li>Span 5-6 vertebral bodies </li></ul><ul><li>Demonstrate moderate and heterogeneous </li></ul><ul><li>Location </li></ul><ul><ul><li>75% Cervical/Thoracic </li></ul></ul><ul><ul><li>20% Distal </li></ul></ul><ul><ul><li>5% Filum Terminale </li></ul></ul>
    40. 40. Thoracic Intramedullary Pilocytic Astrocytoma Eleven year-old girl presented with 3 month history of low back pain Binning, M. Spinal Tumors in Children. Neurosurg Clin N Am. 2007; 18: 631-658
    41. 41. Pilocytic Astrocytoma Kothbauer KF. Neurosurgical Management of Intramedullary Spinal Cord Tumors in Children. Pediatric Neurosurgery. 2007; 43:222-235.
    42. 42. Fibrillary Astrocytoma Kothbauer KF. Neurosurgical Management of Intramedullary Spinal Cord Tumors in Children. Pediatric Neurosurgery. 2007; 43:222-235.
    43. 43. Young adult who presents with neck pain, weakness, and numbness in Right Arm.
    44. 44. History <ul><li>1887 Sir Victor Horsley </li></ul><ul><ul><li>First resection of an intradural neoplasm </li></ul></ul><ul><li>1907 Anton von Eiselsberg </li></ul><ul><ul><li>First resection of an intramedullary tumor </li></ul></ul><ul><li>1911 Charles Elsberg </li></ul><ul><ul><li>Two-stage operation </li></ul></ul><ul><ul><ul><li>Myelotomy </li></ul></ul></ul><ul><ul><ul><li>Tumor Resection </li></ul></ul></ul><ul><li>1925 Charles Elsberg </li></ul><ul><ul><li>“ The majority of tumors within the substance of the cord are infiltrating in character and therefore not removable.” </li></ul></ul>Sir Victor Horsley
    45. 45. History <ul><li>Surgical Challenge </li></ul><ul><ul><li>Biopsy </li></ul></ul><ul><ul><li>Dural Decompression Radiotherapy </li></ul></ul><ul><li>1980’s </li></ul><ul><ul><li>Microscope </li></ul></ul><ul><ul><li>MRI </li></ul></ul>
    46. 46. Treatment <ul><li>Surgery </li></ul><ul><ul><li>Extent of resection differs for each tumor type </li></ul></ul><ul><ul><ul><li>Infiltrative: utility is unclear </li></ul></ul></ul><ul><ul><ul><li>Ependymomas: Complete or near complete >90% resection is associated with long-term tumor control & even cure </li></ul></ul></ul><ul><ul><li>Clear tumor plane </li></ul></ul><ul><ul><li>Completely resected with acceptable morbidity </li></ul></ul><ul><li>Radiation Therapy </li></ul><ul><ul><li>Reserved for: </li></ul></ul><ul><ul><ul><li>Incompletely Resected Tumors </li></ul></ul></ul><ul><ul><ul><li>Experience of Rapid Recurrence </li></ul></ul></ul><ul><ul><ul><li>High grade astrocytomas </li></ul></ul></ul><ul><li>Chemotherapy </li></ul><ul><ul><li>Not well established </li></ul></ul><ul><ul><li>Has been used to delay radiotherapy in children less than 3 </li></ul></ul>
    47. 47. Outcome <ul><li>Predictive by: </li></ul><ul><ul><li>Histologic grade of tumor </li></ul></ul><ul><ul><li>Patient’s pre-operative neurologic status </li></ul></ul><ul><ul><ul><li>Children with pre-operative motor deficits are more likely to deteriorate following surgery. </li></ul></ul></ul><ul><ul><li>Pilocytic > Ependymomas Grade II > Astrocytomas II </li></ul></ul><ul><ul><ul><li>Low grade neoplasms 5- & 10- survival 88% & 82% respectively. </li></ul></ul></ul><ul><ul><li>Anaplastic </li></ul></ul><ul><ul><ul><li>Limited evidence that GTR prolongs disease-free survival. </li></ul></ul></ul><ul><ul><li>Malignant </li></ul></ul><ul><ul><ul><li>Despite aggressive therapy 6-12 month median survival </li></ul></ul></ul><ul><ul><ul><li>18% 5-year survival </li></ul></ul></ul>
    48. 48. Intramedullary Spinal Cord Metastases <ul><li>3-5% of cases of myelopathy in patients affected by cancer </li></ul><ul><li>Found 0.9-2% of cancer patients at autopsy </li></ul><ul><li>3.4% symptomatic metastic spinal cord lesions </li></ul><ul><li>Primaries: </li></ul><ul><ul><li>Small cell lung cancer </li></ul></ul><ul><ul><li>Breast cancer </li></ul></ul><ul><ul><li>Malignant melanoma </li></ul></ul><ul><ul><li>Lymphoma </li></ul></ul><ul><ul><li>Colon Cancer </li></ul></ul><ul><ul><li>Rarely present initially with intramedullary spinal metastases </li></ul></ul>
    49. 49. Intramedullary Spinal Cord Metastases Costigan DA, Winkelman MD. Intramedullary spinal cord metastasis. A clinicopathologic study of 13 cases. J Neurosurg 1985;62:227-33.
    50. 50. Intramedullary Spinal Cord Metastases: Clinical Manifestations <ul><li>Early in the Course </li></ul><ul><ul><li>Local back pain </li></ul></ul><ul><ul><li>Radicular or Referred pain in a Limb </li></ul></ul><ul><ul><li>Aggravation of pain with </li></ul></ul><ul><ul><ul><li>Cough </li></ul></ul></ul><ul><ul><ul><li>Straining of stool </li></ul></ul></ul><ul><ul><ul><li>Deep inspiration </li></ul></ul></ul><ul><ul><ul><li>Straight leg raise </li></ul></ul></ul><ul><ul><li>Vertebral Percussion Tenderness </li></ul></ul><ul><ul><ul><li>Dorsal Horn </li></ul></ul></ul><ul><ul><li>Weakness in one arm or leg </li></ul></ul><ul><ul><ul><li>Medial portion of the lateral column or in the ventral horn. </li></ul></ul></ul><ul><li>Later in the Course </li></ul><ul><li>Transverse Myelopathy </li></ul><ul><li>Brown-Sequard Syndrome </li></ul><ul><li>Ascending Myelopathy or Descending Myelopathy </li></ul><ul><li>Signs of Leptomeningeal Metastases </li></ul>
    51. 51. Presentation <ul><li>Initial Symptoms </li></ul><ul><ul><li>17/40 Sensory Alteration </li></ul></ul><ul><ul><li>12/40 Weakness </li></ul></ul><ul><ul><li>12/40 Pain </li></ul></ul><ul><ul><li>2/40 Gait </li></ul></ul><ul><ul><li>1/40 Urinary Incontinence </li></ul></ul><ul><li>Diagnosis </li></ul><ul><ul><li>21/40 Pain </li></ul></ul><ul><ul><ul><li>9/21 had local back pain </li></ul></ul></ul><ul><ul><ul><li>12/21 radicular pain </li></ul></ul></ul><ul><ul><li>37/40 Motor deficits </li></ul></ul><ul><ul><ul><li>16 Asymmetrical </li></ul></ul></ul><ul><ul><ul><li>15 Bilateral </li></ul></ul></ul><ul><ul><li>35/40 Sensory deficits </li></ul></ul><ul><ul><li>25/40 Urinary Incontinence </li></ul></ul><ul><ul><li>9/40 True Brown Sequard </li></ul></ul><ul><ul><li>9/40 Pseudo- Brown Sequard </li></ul></ul>Schiff D, et al. Intramedullary spinal cord metastases: Clinical features and treatment outcome. Neurology. 1996; 906-912.
    52. 52. Intramedullary Spinal Cord Metastases (Contributed by Dr. Marc D Winkelman.)
    53. 53. Intramedullary Spinal Cord Metastases (Contributed by Dr. Marc D Winkelman.)
    54. 54. Intramedullary Spinal Cord Metastases (Contributed by Dr. Marc D Winkelman.)
    55. 55. Intramedullary Spinal Cord Metastases (Contributed by Dr. Marc D Winkelman.)
    56. 56. Initial Presentation <ul><li>History and Physical </li></ul><ul><li>This 61-year-old woman had the insidious onset of numbness of the entire right hemibody below the clavicle and weakness of the upper and lower extremities, without pain or sphincter dysfunction. The patient denied pain, fever, weight loss, visual symptoms, or a family history of malignancy. </li></ul><ul><li>General exam: “Almost negative” </li></ul><ul><li>Neurological exam: </li></ul><ul><ul><li>Spastic bilateral hemiparesis </li></ul></ul><ul><ul><li>Diffusely hyper-reflexic </li></ul></ul><ul><ul><li>Bilateral Babinski </li></ul></ul><ul><ul><li>Absence of pain and temperature sense below the clavicle, with preserved sense of discriminatory touch. </li></ul></ul>
    57. 57. Adenocarcinoma of the Colon Grasso G. Intramedullary spinal cord tumor presenting as the initial manifestation of metastatic colon cancer: case report and review of the literature. Spinal Cord. 2007; 45: 793-6.
    58. 58. Presentation <ul><li>9/40 Initial Presentation </li></ul><ul><li>4 Lung Cancer, 2 Adenocarcinoma of unknown etiology, One each had melanoma, colon, and renal carcinoma </li></ul><ul><li>31/40 </li></ul><ul><ul><li>Diagnosis of malignancy had been made 2 months to 12 years earlier (Median 17 months) </li></ul></ul>Schiff D, et al. Intramedullary spinal cord metastases: Clinical features and treatment outcome. Neurology. 1996; 906-912.
    59. 59. Theories <ul><li>Hematogenous dissemination through either arterial supply to the spinal cord or retrograde spread from venous system. </li></ul><ul><ul><li>Brain receives a third of the cardiac output through large vessels under high pressure </li></ul></ul><ul><ul><li>Spinal cord receives it arterial supply from small vessels under low pressure. </li></ul></ul>
    60. 60. Spinal Cord Anatomy: Vasculature <ul><li>Below C-Spine </li></ul><ul><ul><li>Continuous anastomoses with the radicular arteries. </li></ul></ul><ul><ul><ul><li>Aorta </li></ul></ul></ul><ul><ul><ul><li>Intercostal Arteries </li></ul></ul></ul><ul><ul><ul><li>Spinal Arteries </li></ul></ul></ul><ul><ul><ul><li>Anterior & Posterior Radicular Arteries </li></ul></ul></ul>
    61. 61. Drop Metastases (Contributed by Dr. Marc D Winkelman.)
    62. 62. Drop Metastases (Contributed by Dr. Marc D Winkelman.)
    63. 63. Intramedullary vs. Epidural <ul><li>The neurologic deficit </li></ul><ul><ul><li>Intramedullary metastasis </li></ul></ul><ul><ul><ul><li>Evolves over weeks </li></ul></ul></ul><ul><ul><li>Epidural cord compression </li></ul></ul><ul><ul><ul><li>Progresses over days </li></ul></ul></ul><ul><ul><ul><li>20 to 30x more common than Intramedullary metastasis </li></ul></ul></ul>
    64. 64. Intramedullary Metastasis vs. Radiation Myelopathy <ul><li>Intramedullary Metastases </li></ul><ul><ul><li>Pain (Early Onset) </li></ul></ul><ul><ul><li>Onset (Days to Weeks) </li></ul></ul><ul><ul><li>Abnormal MRI </li></ul></ul><ul><li>Radiation Myelopathy </li></ul><ul><ul><li>Pain (Usually Absent) </li></ul></ul><ul><ul><li>Onset (Months to Years) </li></ul></ul><ul><ul><li>Increased with: </li></ul></ul><ul><ul><ul><li>Higher Total Dose </li></ul></ul></ul><ul><ul><ul><li>Higher Fractional Dose </li></ul></ul></ul><ul><ul><ul><li>Shorter treatment time </li></ul></ul></ul><ul><ul><ul><li>Greater Length of Cord Irradiated </li></ul></ul></ul><ul><ul><ul><li>Delay of at least 4 months </li></ul></ul></ul><ul><ul><li>Normal MRI </li></ul></ul>
    65. 65. Prognosis & Complications <ul><li>Median survival with intramedullary spinal cord metastases 7 months (Range 2 weeks to 2 years) </li></ul><ul><li>Favorable prognostic factors </li></ul><ul><ul><li>Breast > Lung </li></ul></ul><ul><ul><li>Surgery > Radiation </li></ul></ul><ul><li>Cause of death is usually widespread systemic and CNS metastic disease. </li></ul>
    66. 66. Management <ul><li>Radiation therapy </li></ul><ul><ul><li>Stabilization of spinal cord function </li></ul></ul><ul><ul><li>Not short-lived </li></ul></ul><ul><ul><ul><li>Lasts for as long as the patient survives </li></ul></ul></ul><ul><li>Response to therapy depends on extent & duration of neurological deficit and less on radiosensitivity of the tumor. </li></ul>
    67. 67. Paraplegia Complications? <ul><li>Ability to walk is usually preserved following radiation therapy </li></ul><ul><li>Tumor vs. Necrosis </li></ul><ul><li>Rapid progression </li></ul><ul><ul><li>Neuro-oncology emergency </li></ul></ul>
    68. 68. Intramedullary Spinal Cord Metastases 2 months following therapy Schiff D, et al. Intramedullary spinal cord metastases: Clinical features and treatment outcome. Neurology. 1996; 906-912.
    69. 69. Intramedullary Spinal Cord Metastases Schiff D, et al. Intramedullary spinal cord metastases: Clinical features and treatment outcome. Neurology. 1996; 906-912.
    70. 70. Multiple Metastases <ul><li>The spinal cord contains more than 1 intramedullary metastatic tumor in at least 12 % to 23% of patients. </li></ul><ul><ul><li>? Entire spinal cord radiation </li></ul></ul><ul><ul><ul><li>To prevent relapse elsewhere </li></ul></ul></ul><ul><ul><ul><li>Avoid overlapping fields of consecutive courses of radiotherapy. </li></ul></ul></ul><ul><ul><li>Risk of bone-marrow suppression </li></ul></ul>
    71. 71. Surgery <ul><li>Effectiveness unknown </li></ul><ul><ul><li>Recommended for patients not known for systemic disease </li></ul></ul><ul><ul><li>Neurological deficit progresses during or after radiotherapy </li></ul></ul><ul><ul><li>Considered in a patient with no other CNS metastases, with well-controlled systemic disease </li></ul></ul><ul><ul><li>Radioresistant tumor type </li></ul></ul>
    72. 72. Adult Primary Intramedullary Spinal Cord Tumors <ul><li>4% of CNS tumors </li></ul><ul><li>20% Intraspinal tumors </li></ul><ul><li>Astrocytoma: Nonmalignant (30%) </li></ul><ul><li>Ependymoma (30%) </li></ul><ul><li>Miscellaneous (30%) </li></ul><ul><ul><li>Malignant Glioblastoma </li></ul></ul><ul><ul><li>Dermoid </li></ul></ul><ul><ul><li>Epidermoid </li></ul></ul><ul><ul><li>Teratoma </li></ul></ul><ul><ul><li>Hemangioblastoma </li></ul></ul><ul><ul><li>Hemangioma </li></ul></ul><ul><ul><li>Neuroma </li></ul></ul><ul><ul><li>Extremely Rare </li></ul></ul>
    73. 73. Pre-operative Presentation of Adult Intramedullary Spinal Cord Tumors <ul><li>53.9% Sensory Loss </li></ul><ul><li>23. 1 % Gait Difficulty </li></ul><ul><li>20.5% Motor Weakness </li></ul><ul><li>20.5% Pain </li></ul><ul><li>30.8% </li></ul><ul><ul><li>Increase in Urinary Frequency </li></ul></ul><ul><ul><li>Incontinence </li></ul></ul><ul><ul><li>Constipation </li></ul></ul><ul><ul><li>Sexual dysfunction </li></ul></ul>Manzano G, et al. Contemporary management of adult intramedullary spinal tumors- pathology and neurologic outcomes related to surgical resection. Spinal Cord. 2008; 46:540-6.
    74. 74. Adult Primary Intramedullary Spinal Cord Tumors <ul><li>Ependymoma </li></ul><ul><ul><li>Most common glioma of the lower cord, conus and filum. </li></ul></ul><ul><ul><ul><li>Over 50% in filum </li></ul></ul></ul><ul><ul><ul><li>Next most common location is cervical </li></ul></ul></ul><ul><ul><li>Slight male predominance </li></ul></ul><ul><ul><li>Peak 3 rd to 6 th decade </li></ul></ul><ul><ul><li>Symptoms present > 1 year prior to diagnosis in 82% of cases </li></ul></ul><ul><li>Astrocytoma </li></ul><ul><ul><li>Peak 3 rd to 5 th decade </li></ul></ul><ul><ul><li>Male to Female = 1.5:1 </li></ul></ul><ul><ul><li>Benign to Malignant = 3:1 </li></ul></ul><ul><ul><li>Occurs at all levels </li></ul></ul><ul><ul><ul><li>Thoracic most common </li></ul></ul></ul><ul><ul><ul><li>Followed by cervical </li></ul></ul></ul>
    75. 75. Hemangioblastoma <ul><li>Occurs in a third of patients with Von Hippel-Lindau Disease </li></ul><ul><li>80% Symptomatic by 5 th decade </li></ul><ul><li>Bright, homogenous, usually non-infiltrating </li></ul><ul><li>No more than one vertebral body in length </li></ul>
    76. 76. Hemangioblastoma Kothbauer KF. Neurosurgical Management of Intramedullary Spinal Cord Tumors in Children. Pediatric Neurosurgery. 2007; 43:222-235.
    77. 77. Hemangioblastoma Francavilla, Thomas. Intramedullary Spinal Cord Tumors. http://www.emedicine.com/med/topic2995.htm Accessed September 13, 2008 . Conus medullaris lesion in a 45-year-old man who presented with midline back pain.
    78. 78. Prognosis: Adult Primary Intramedullary Spinal Cord Tumors <ul><li>Ependymomas </li></ul><ul><ul><li>Survival is independent of extent of excision </li></ul></ul><ul><ul><li>Best functional outcome: </li></ul></ul><ul><ul><ul><li>Modest initial deficits </li></ul></ul></ul><ul><ul><ul><li>Symptoms <2 years duration </li></ul></ul></ul><ul><ul><ul><li>Total removal </li></ul></ul></ul><ul><li>Astrocytomas </li></ul><ul><ul><li>Radial removal rarely possible </li></ul></ul><ul><ul><li>50% recurrence rate at 5 years </li></ul></ul><ul><ul><li>Long term functional results are poorer than ependymomas </li></ul></ul>
    79. 79. Astrocytoma Francavilla, Thomas. Intramedullary Spinal Cord Tumors. http://www.emedicine.com/med/topic2995.htm Accessed September 13, 2008. This 18-year-old woman presented with poor balance and a staggering gait progressing over 1 year. 4-year follow-up
    80. 80. Questions?

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