Intramedullary Spinal Cord Tumors Leslie Hutchins, MD Shore Memorial Cancer Conference September 18, 2008

Spinal Cord Anatomy- Meninges Protective Sheath Duramater Arachnoid Piamater Potential Space Subdural Space Located between the Dura & Arachnoid Subarachnoid Space The Cerebrospinal Fluid flows between the Arachnoid & Piamater in this space.

Protective Sheath

Duramater

Arachnoid

Piamater

Potential Space

Subdural Space

Located between the Dura & Arachnoid

Subarachnoid Space

The Cerebrospinal Fluid flows between the Arachnoid & Piamater in this space.

Spinal Cord Anatomy Begins Foramen Magnum as a continuation of the Medulla Oblongata Terminates Conus Medullaris Adult: Lower Border of L1 Young Child: Upper Border of L3 Filum Terminale Prolongation of the piamater Attaches to the spinal cord at the coccyx Dural Sac Ends at the level of the second sacral vertebra

Begins

Foramen Magnum as a continuation of the Medulla Oblongata

Terminates

Conus Medullaris

Adult: Lower Border of L1

Young Child: Upper Border of L3

Filum Terminale

Prolongation of the piamater

Attaches to the spinal cord at the coccyx

Dural Sac

Ends at the level of the second sacral vertebra

Spinal Cord Anatomy Gray Matter Ventral (motor) Horns Dorsal (sensory) Horns White Matter Anterior Columns Lateral Columns Posterior Columns D.E. Haines, Neuroanatomy: An Atlas of Structures, Sections, and Systems, 3rd ed. (1991), Urban & Schwarzenberg Encyclopedia Britannica, Inc.

Gray Matter

Ventral (motor) Horns

Dorsal (sensory) Horns

White Matter

Anterior Columns

Lateral Columns

Posterior Columns

Spinal Cord Anatomy 31 pairs of spinal nerves (Each formed by 2 roots) The spinal nerves exit through the intervebral foramen 8 cervical 1 st exits through the Occipital Bone and C 1 8 th exits between C7 & T 1 1 2 thoracic Distal to T 1 each spinal nerve exits below its corresponding vertebra 5 lumbar 5 sacral 1 coccygeal

31 pairs of spinal nerves (Each formed by 2 roots)

The spinal nerves exit through the intervebral foramen

8 cervical

1 st exits through the Occipital Bone and C 1

8 th exits between C7 & T 1

1 2 thoracic

Distal to T 1 each spinal nerve exits below its corresponding vertebra

5 lumbar

5 sacral

1 coccygeal

Motor impairment related to Spinal Cord Injury

Dermatomes

Spinal Cord Cross Section: Fiber Tracts

Spinal Cord Anatomy: Vasculature Upper Cervical Spine Vertebral Artery: One Anterior Spinal Artery Two Posterior Spinal Arteries

Upper Cervical Spine

Vertebral Artery:

One Anterior Spinal Artery

Two Posterior Spinal Arteries

Spinal Cord Anatomy: Vasculature Lower Third of C-Spine: Anterior Segmental Medullary (Radicular) Arteries Vertebral Artery Ascending Cervical Artery Deep Cervical Artery

Lower Third of C-Spine:

Anterior Segmental Medullary (Radicular) Arteries

Vertebral Artery

Ascending Cervical Artery

Deep Cervical Artery

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 Spinal Cord Anatomy: Vasculature

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

Spinal Cord Anatomy: Vasculature Below C-Spine Continuous anastomoses with the radicular arteries. Aorta Intercostal Arteries Spinal Arteries Anterior & Posterior Radicular Arteries

Below C-Spine

Continuous anastomoses with the radicular arteries.

Aorta

Intercostal Arteries

Spinal Arteries

Anterior & Posterior Radicular Arteries

Spinal Cord Anatomy: Vasculature Thoracic/Lumbar Spine Anterior Segmental Arteries Alternate Sides of Cord Artery of Adamkiewicz Major Blood Supply to Lower Thoracic & Lumbar Spine Arises on the Left ~78% Enters single intervertebral foramen between T8 & L3

Thoracic/Lumbar Spine

Anterior Segmental Arteries Alternate Sides of Cord

Artery of Adamkiewicz

Major Blood Supply to Lower Thoracic & Lumbar Spine

Arises on the Left ~78%

Enters single intervertebral foramen between T8 & L3

Spinal Cord Anatomy: Vasculature Batson’s Plexus: "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." -Batson Multiple anastomoses with: Azygous system IVC Pelvic plexus Prostatic plexus

Batson’s Plexus:

"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." -Batson

Multiple anastomoses with:

Azygous system

IVC

Pelvic plexus

Prostatic plexus

Classification of Spinal Cord Lesions Extradural Outside the thecal sac (including vertebral bone lesions) Intradural/Extramedullary Within thecal sac, but outside the cord Intramedullary Within cord

Extradural

Outside the thecal sac (including vertebral bone lesions)

Intradural/Extramedullary

Within thecal sac, but outside the cord

Intramedullary

Within cord

Classification of Spinal Cord Lesions

Pediatric Intramedullary Spinal Cord Tumors 4-6% of Pediatric CNS Neoplasms Astrocytomas 60% of all Pediatric IMSCTs 10-15% are High Grade Ependymomas Miscellaneous Teratomas Dermoid Epidermoid Cysts Oligodendrogliomas Schwannomas Neurofibromas Subependymomas Neurocytomas Metastases

4-6% of Pediatric CNS Neoplasms

Astrocytomas

60% of all Pediatric IMSCTs

10-15% are High Grade

Ependymomas

Miscellaneous

Teratomas

Dermoid

Epidermoid Cysts

Oligodendrogliomas

Schwannomas

Neurofibromas

Subependymomas

Neurocytomas

Metastases

Presentation Low Grade Asymptomatic Subtle Presentation Symptoms to Diagnosis 9.2 months High Grade Symptoms to Diagnosis 7 weeks to 4 months

Low Grade

Asymptomatic

Subtle Presentation

Symptoms to Diagnosis

9.2 months

High Grade

Symptoms to Diagnosis

7 weeks to 4 months

Clinical Manifestations Back or neck pain Dull Aching Diffuse Referable to spinal axis involved Worse at night Venous congestion & dural distension caused by recumbent position Meningeal Irritation Weakness Paraesthesias Spinal Deformity Slow neurologic deterioration Dysesthesias 20% Usually asymmetrical in glial tumors Sphincter dysfunction Bowel/Bladder dysfunction is rare unless late in clinical course Exception: Conus Medullaris Lesions Motor Delay in Milestones (i.e. Crawling/Walking) Motor Regression Gait abnormality Frequent falls

Back or neck pain

Dull

Aching

Diffuse

Referable to spinal axis involved

Worse at night

Venous congestion & dural distension caused by recumbent position

Meningeal Irritation

Weakness

Paraesthesias

Spinal Deformity

Slow neurologic deterioration

Dysesthesias

20%

Usually asymmetrical in glial tumors

Sphincter dysfunction

Bowel/Bladder dysfunction is rare unless late in clinical course

Exception: Conus Medullaris Lesions

Motor

Delay in Milestones

(i.e. Crawling/Walking)

Motor Regression

Gait abnormality

Frequent falls

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. 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.

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.

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.

Clinical Exam Mild to Moderate Motor Deficit Upper Motor Neuron Signs Spasticity Hyperreflexia Clonus Increase muscle tone

Mild to Moderate Motor Deficit

Upper Motor Neuron Signs

Spasticity

Hyperreflexia

Clonus

Increase muscle tone

Kyphoscoliosis

Torticollis

Hydrocephalus Associated with 15% of children with IMSCTs Substantially higher in patients with malignant IMSCTs (35%) Increase Concentration of Protein in the CSF Arachnoidal fibrosis Subarachnoid dissemination 4 th Ventricle outlet obstruction

Associated with 15% of children with IMSCTs

Substantially higher in patients with malignant IMSCTs (35%)

Increase Concentration of Protein in the CSF

Arachnoidal fibrosis

Subarachnoid dissemination

4 th Ventricle outlet obstruction

Work-up MRI (Study of Choice) With and without gadolinium Plain Radiographs Scoliosis Post-op deformity CT Bony involvement is rare Only obtained to follow spinal deformity or if MRI is contraindicated

MRI (Study of Choice)

With and without gadolinium

Plain Radiographs

Scoliosis

Post-op deformity

CT

Bony involvement is rare

Only obtained to follow spinal deformity or if MRI is contraindicated

Ependymomas Subependymoma WHO Grade 1 Slow Growing Detected Incidentally Good Prognosis Myxopapillary Ependymoma WHO Grade 1 Arise in the filum terminale or conus medullaris

Subependymoma

WHO Grade 1

Slow Growing

Detected Incidentally

Good Prognosis

Myxopapillary Ependymoma

WHO Grade 1

Arise in the filum terminale or conus medullaris

Ependymomas Ependymoma WHO Grade II Anaplastic WHO Grade III Are thought to arise from malignant transformation of low-grade ependymomas Glioblastoma Multiforme WHO Grade 4

Ependymoma

WHO Grade II

Anaplastic

WHO Grade III

Are thought to arise from malignant transformation of low-grade ependymomas

Glioblastoma Multiforme

WHO Grade 4

Myxopapillary Ependymomas Abundant Mucin Production, GFAP Immunoreactivity, Rare mitosis, & Cuboidal or elongated morphology

Abundant Mucin Production, GFAP Immunoreactivity,

Rare mitosis, & Cuboidal or elongated morphology

Ependymomas Highly cellular tumors with rare mitotic activity. GFAP Immunoreactivity, Perivascular Psuedorosettes, & Occasional Ependymal Rosettes

Highly cellular tumors with rare mitotic activity.

GFAP Immunoreactivity, Perivascular Psuedorosettes, & Occasional Ependymal Rosettes

Anaplastic Ependymomas Frequent mitosis, Necrosis, Neovascularization, & Increased Cellularity

Frequent mitosis, Necrosis, Neovascularization, & Increased Cellularity

Ependymoma Centrally located 3-4 Vertebral Bodies Defined Borders Enhance brightly & homogenously Cystic components

Centrally located

3-4 Vertebral Bodies

Defined Borders

Enhance brightly & homogenously

Cystic components

Nineteen year-old who presented with finger numbness & “clumsiness”

Ependymoma Kothbauer KF. Neurosurgical Management of Intramedullary Spinal Cord Tumors in Children. Pediatric Neurosurgery. 2007; 43:222-235.

www.stevenscordfoundation.com

Astrocytoma Pathogenesis Unclear Genetic disorders associated with family clustering of IMSCTs Li Fraumeni syndrome Turcot syndrome Tuberous sclerosis Multiple enchondromatosis NF Radiation Induced Astrocytomas

Pathogenesis Unclear

Genetic disorders associated with family clustering of IMSCTs

Li Fraumeni syndrome

Turcot syndrome

Tuberous sclerosis

Multiple enchondromatosis

NF

Radiation Induced Astrocytomas

Astrocytoma Pilocytic Astrocytoma WHO Grade I Most Common Fibrillary Astrocytoma WHO Grade II Most Common Anaplastic Astrocytoma WHO Grade III GBM WHO Grade IV

Pilocytic Astrocytoma

WHO Grade I

Most Common

Fibrillary Astrocytoma

WHO Grade II

Most Common

Anaplastic Astrocytoma

WHO Grade III

GBM

WHO Grade IV

Pilocytic Astrocytoma Elongated bipolar astrocytes, Intracytoplasmic Rosenthal fibers, & Eosinophilic granular bodies

Elongated bipolar astrocytes, Intracytoplasmic Rosenthal fibers,

& Eosinophilic granular bodies

Fibrillary Astrocytomas Infiltrative tumors, Mild nuclear pleomorphism, & Hypercellularity with mitosis

Infiltrative tumors, Mild nuclear pleomorphism, & Hypercellularity with mitosis

Astrocytomas Eccentrically located Produce symmetric enlargement Ill-defined borders Span 5-6 vertebral bodies Demonstrate moderate and heterogeneous Location 75% Cervical/Thoracic 20% Distal 5% Filum Terminale

Eccentrically located

Produce symmetric enlargement

Ill-defined borders

Span 5-6 vertebral bodies

Demonstrate moderate and heterogeneous

Location

75% Cervical/Thoracic

20% Distal

5% Filum Terminale

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

Pilocytic Astrocytoma Kothbauer KF. Neurosurgical Management of Intramedullary Spinal Cord Tumors in Children. Pediatric Neurosurgery. 2007; 43:222-235.

Fibrillary Astrocytoma Kothbauer KF. Neurosurgical Management of Intramedullary Spinal Cord Tumors in Children. Pediatric Neurosurgery. 2007; 43:222-235.

Young adult who presents with neck pain, weakness, and numbness in Right Arm.

History 1887 Sir Victor Horsley First resection of an intradural neoplasm 1907 Anton von Eiselsberg First resection of an intramedullary tumor 1911 Charles Elsberg Two-stage operation Myelotomy Tumor Resection 1925 Charles Elsberg “ The majority of tumors within the substance of the cord are infiltrating in character and therefore not removable.” Sir Victor Horsley

1887 Sir Victor Horsley

First resection of an intradural neoplasm

1907 Anton von Eiselsberg

First resection of an intramedullary tumor

1911 Charles Elsberg

Two-stage operation

Myelotomy

Tumor Resection

1925 Charles Elsberg

“ The majority of tumors within the substance of the cord are infiltrating in character and therefore not removable.”

History Surgical Challenge Biopsy Dural Decompression Radiotherapy 1980’s Microscope MRI

Surgical Challenge

Biopsy

Dural Decompression Radiotherapy

1980’s

Microscope

MRI

Treatment Surgery Extent of resection differs for each tumor type Infiltrative: utility is unclear Ependymomas: Complete or near complete >90% resection is associated with long-term tumor control & even cure Clear tumor plane Completely resected with acceptable morbidity Radiation Therapy Reserved for: Incompletely Resected Tumors Experience of Rapid Recurrence High grade astrocytomas Chemotherapy Not well established Has been used to delay radiotherapy in children less than 3

Surgery

Extent of resection differs for each tumor type

Infiltrative: utility is unclear

Ependymomas: Complete or near complete >90% resection is associated with long-term tumor control & even cure

Clear tumor plane

Completely resected with acceptable morbidity

Radiation Therapy

Reserved for:

Incompletely Resected Tumors

Experience of Rapid Recurrence

High grade astrocytomas

Chemotherapy

Not well established

Has been used to delay radiotherapy in children less than 3

Outcome Predictive by: Histologic grade of tumor Patient’s pre-operative neurologic status Children with pre-operative motor deficits are more likely to deteriorate following surgery. Pilocytic > Ependymomas Grade II > Astrocytomas II Low grade neoplasms 5- & 10- survival 88% & 82% respectively. Anaplastic Limited evidence that GTR prolongs disease-free survival. Malignant Despite aggressive therapy 6-12 month median survival 18% 5-year survival

Predictive by:

Histologic grade of tumor

Patient’s pre-operative neurologic status

Children with pre-operative motor deficits are more likely to deteriorate following surgery.

Pilocytic > Ependymomas Grade II > Astrocytomas II

Low grade neoplasms 5- & 10- survival 88% & 82% respectively.

Anaplastic

Limited evidence that GTR prolongs disease-free survival.

Malignant

Despite aggressive therapy 6-12 month median survival

18% 5-year survival

Intramedullary Spinal Cord Metastases 3-5% of cases of myelopathy in patients affected by cancer Found 0.9-2% of cancer patients at autopsy 3.4% symptomatic metastic spinal cord lesions Primaries: Small cell lung cancer Breast cancer Malignant melanoma Lymphoma Colon Cancer Rarely present initially with intramedullary spinal metastases

3-5% of cases of myelopathy in patients affected by cancer

Found 0.9-2% of cancer patients at autopsy

3.4% symptomatic metastic spinal cord lesions

Primaries:

Small cell lung cancer

Breast cancer

Malignant melanoma

Lymphoma

Colon Cancer

Rarely present initially with intramedullary spinal metastases

Intramedullary Spinal Cord Metastases Costigan DA, Winkelman MD. Intramedullary spinal cord metastasis. A clinicopathologic study of 13 cases. J Neurosurg 1985;62:227-33.

Intramedullary Spinal Cord Metastases: Clinical Manifestations Early in the Course Local back pain Radicular or Referred pain in a Limb Aggravation of pain with Cough Straining of stool Deep inspiration Straight leg raise Vertebral Percussion Tenderness Dorsal Horn Weakness in one arm or leg Medial portion of the lateral column or in the ventral horn. Later in the Course Transverse Myelopathy Brown-Sequard Syndrome Ascending Myelopathy or Descending Myelopathy Signs of Leptomeningeal Metastases

Early in the Course

Local back pain

Radicular or Referred pain in a Limb

Aggravation of pain with

Cough

Straining of stool

Deep inspiration

Straight leg raise

Vertebral Percussion Tenderness

Dorsal Horn

Weakness in one arm or leg

Medial portion of the lateral column or in the ventral horn.

Later in the Course

Transverse Myelopathy

Brown-Sequard Syndrome

Ascending Myelopathy or Descending Myelopathy

Signs of Leptomeningeal Metastases

Presentation Initial Symptoms 17/40 Sensory Alteration 12/40 Weakness 12/40 Pain 2/40 Gait 1/40 Urinary Incontinence Diagnosis 21/40 Pain 9/21 had local back pain 12/21 radicular pain 37/40 Motor deficits 16 Asymmetrical 15 Bilateral 35/40 Sensory deficits 25/40 Urinary Incontinence 9/40 True Brown Sequard 9/40 Pseudo- Brown Sequard Schiff D, et al. Intramedullary spinal cord metastases: Clinical features and treatment outcome. Neurology. 1996; 906-912.

Initial Symptoms

17/40 Sensory Alteration

12/40 Weakness

12/40 Pain

2/40 Gait

1/40 Urinary Incontinence

Diagnosis

21/40 Pain

9/21 had local back pain

12/21 radicular pain

37/40 Motor deficits

16 Asymmetrical

15 Bilateral

35/40 Sensory deficits

25/40 Urinary Incontinence

9/40 True Brown Sequard

9/40 Pseudo- Brown Sequard

Intramedullary Spinal Cord Metastases (Contributed by Dr. Marc D Winkelman.)

Intramedullary Spinal Cord Metastases (Contributed by Dr. Marc D Winkelman.)

Intramedullary Spinal Cord Metastases (Contributed by Dr. Marc D Winkelman.)

Intramedullary Spinal Cord Metastases (Contributed by Dr. Marc D Winkelman.)

Initial Presentation History and Physical 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. General exam: “Almost negative” Neurological exam: Spastic bilateral hemiparesis Diffusely hyper-reflexic Bilateral Babinski Absence of pain and temperature sense below the clavicle, with preserved sense of discriminatory touch.

History and Physical

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.

General exam: “Almost negative”

Neurological exam:

Spastic bilateral hemiparesis

Diffusely hyper-reflexic

Bilateral Babinski

Absence of pain and temperature sense below the clavicle, with preserved sense of discriminatory touch.

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.

Presentation 9/40 Initial Presentation 4 Lung Cancer, 2 Adenocarcinoma of unknown etiology, One each had melanoma, colon, and renal carcinoma 31/40 Diagnosis of malignancy had been made 2 months to 12 years earlier (Median 17 months) Schiff D, et al. Intramedullary spinal cord metastases: Clinical features and treatment outcome. Neurology. 1996; 906-912.

9/40 Initial Presentation

4 Lung Cancer, 2 Adenocarcinoma of unknown etiology, One each had melanoma, colon, and renal carcinoma

31/40

Diagnosis of malignancy had been made 2 months to 12 years earlier (Median 17 months)

Theories Hematogenous dissemination through either arterial supply to the spinal cord or retrograde spread from venous system. Brain receives a third of the cardiac output through large vessels under high pressure Spinal cord receives it arterial supply from small vessels under low pressure.

Hematogenous dissemination through either arterial supply to the spinal cord or retrograde spread from venous system.

Brain receives a third of the cardiac output through large vessels under high pressure

Spinal cord receives it arterial supply from small vessels under low pressure.

Spinal Cord Anatomy: Vasculature Below C-Spine Continuous anastomoses with the radicular arteries. Aorta Intercostal Arteries Spinal Arteries Anterior & Posterior Radicular Arteries

Below C-Spine

Continuous anastomoses with the radicular arteries.

Aorta

Intercostal Arteries

Spinal Arteries

Anterior & Posterior Radicular Arteries

Drop Metastases (Contributed by Dr. Marc D Winkelman.)

Drop Metastases (Contributed by Dr. Marc D Winkelman.)

Intramedullary vs. Epidural The neurologic deficit Intramedullary metastasis Evolves over weeks Epidural cord compression Progresses over days 20 to 30x more common than Intramedullary metastasis

The neurologic deficit

Intramedullary metastasis

Evolves over weeks

Epidural cord compression

Progresses over days

20 to 30x more common than Intramedullary metastasis

Intramedullary Metastasis vs. Radiation Myelopathy Intramedullary Metastases Pain (Early Onset) Onset (Days to Weeks) Abnormal MRI Radiation Myelopathy Pain (Usually Absent) Onset (Months to Years) Increased with: Higher Total Dose Higher Fractional Dose Shorter treatment time Greater Length of Cord Irradiated Delay of at least 4 months Normal MRI

Intramedullary Metastases

Pain (Early Onset)

Onset (Days to Weeks)

Abnormal MRI

Radiation Myelopathy

Pain (Usually Absent)

Onset (Months to Years)

Increased with:

Higher Total Dose

Higher Fractional Dose

Shorter treatment time

Greater Length of Cord Irradiated

Delay of at least 4 months

Normal MRI

Prognosis & Complications Median survival with intramedullary spinal cord metastases 7 months (Range 2 weeks to 2 years) Favorable prognostic factors Breast > Lung Surgery > Radiation Cause of death is usually widespread systemic and CNS metastic disease.

Median survival with intramedullary spinal cord metastases 7 months (Range 2 weeks to 2 years)

Favorable prognostic factors

Breast > Lung

Surgery > Radiation

Cause of death is usually widespread systemic and CNS metastic disease.

Management Radiation therapy Stabilization of spinal cord function Not short-lived Lasts for as long as the patient survives Response to therapy depends on extent & duration of neurological deficit and less on radiosensitivity of the tumor.

Radiation therapy

Stabilization of spinal cord function

Not short-lived

Lasts for as long as the patient survives

Response to therapy depends on extent & duration of neurological deficit and less on radiosensitivity of the tumor.

Paraplegia Complications? Ability to walk is usually preserved following radiation therapy Tumor vs. Necrosis Rapid progression Neuro-oncology emergency

Ability to walk is usually preserved following radiation therapy

Tumor vs. Necrosis

Rapid progression

Neuro-oncology emergency

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.

Intramedullary Spinal Cord Metastases Schiff D, et al. Intramedullary spinal cord metastases: Clinical features and treatment outcome. Neurology. 1996; 906-912.

Multiple Metastases The spinal cord contains more than 1 intramedullary metastatic tumor in at least 12 % to 23% of patients. ? Entire spinal cord radiation To prevent relapse elsewhere Avoid overlapping fields of consecutive courses of radiotherapy. Risk of bone-marrow suppression

The spinal cord contains more than 1 intramedullary metastatic tumor in at least 12 % to 23% of patients.

? Entire spinal cord radiation

To prevent relapse elsewhere

Avoid overlapping fields of consecutive courses of radiotherapy.

Risk of bone-marrow suppression

Surgery Effectiveness unknown Recommended for patients not known for systemic disease Neurological deficit progresses during or after radiotherapy Considered in a patient with no other CNS metastases, with well-controlled systemic disease Radioresistant tumor type

Effectiveness unknown

Recommended for patients not known for systemic disease

Neurological deficit progresses during or after radiotherapy

Considered in a patient with no other CNS metastases, with well-controlled systemic disease

Radioresistant tumor type

Adult Primary Intramedullary Spinal Cord Tumors 4% of CNS tumors 20% Intraspinal tumors Astrocytoma: Nonmalignant (30%) Ependymoma (30%) Miscellaneous (30%) Malignant Glioblastoma Dermoid Epidermoid Teratoma Hemangioblastoma Hemangioma Neuroma Extremely Rare

4% of CNS tumors

20% Intraspinal tumors

Astrocytoma: Nonmalignant (30%)

Ependymoma (30%)

Miscellaneous (30%)

Malignant Glioblastoma

Dermoid

Epidermoid

Teratoma

Hemangioblastoma

Hemangioma

Neuroma

Extremely Rare

Pre-operative Presentation of Adult Intramedullary Spinal Cord Tumors 53.9% Sensory Loss 23. 1 % Gait Difficulty 20.5% Motor Weakness 20.5% Pain 30.8% Increase in Urinary Frequency Incontinence Constipation Sexual dysfunction 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.

53.9% Sensory Loss

23. 1 % Gait Difficulty

20.5% Motor Weakness

20.5% Pain

30.8%

Increase in Urinary Frequency

Incontinence

Constipation

Sexual dysfunction

Adult Primary Intramedullary Spinal Cord Tumors Ependymoma Most common glioma of the lower cord, conus and filum. Over 50% in filum Next most common location is cervical Slight male predominance Peak 3 rd to 6 th decade Symptoms present > 1 year prior to diagnosis in 82% of cases Astrocytoma Peak 3 rd to 5 th decade Male to Female = 1.5:1 Benign to Malignant = 3:1 Occurs at all levels Thoracic most common Followed by cervical

Ependymoma

Most common glioma of the lower cord, conus and filum.

Over 50% in filum

Next most common location is cervical

Slight male predominance

Peak 3 rd to 6 th decade

Symptoms present > 1 year prior to diagnosis in 82% of cases

Astrocytoma

Peak 3 rd to 5 th decade

Male to Female = 1.5:1

Benign to Malignant = 3:1

Occurs at all levels

Thoracic most common

Followed by cervical

Hemangioblastoma Occurs in a third of patients with Von Hippel-Lindau Disease 80% Symptomatic by 5 th decade Bright, homogenous, usually non-infiltrating No more than one vertebral body in length

Occurs in a third of patients with Von Hippel-Lindau Disease

80% Symptomatic by 5 th decade

Bright, homogenous, usually non-infiltrating

No more than one vertebral body in length

Hemangioblastoma Kothbauer KF. Neurosurgical Management of Intramedullary Spinal Cord Tumors in Children. Pediatric Neurosurgery. 2007; 43:222-235.

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.

Prognosis: Adult Primary Intramedullary Spinal Cord Tumors Ependymomas Survival is independent of extent of excision Best functional outcome: Modest initial deficits Symptoms <2 years duration Total removal Astrocytomas Radial removal rarely possible 50% recurrence rate at 5 years Long term functional results are poorer than ependymomas

Ependymomas

Survival is independent of extent of excision

Best functional outcome:

Modest initial deficits

Symptoms <2 years duration

Total removal

Astrocytomas

Radial removal rarely possible

50% recurrence rate at 5 years

Long term functional results are poorer than ependymomas

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

Questions?