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Intradural Extramedullary Tumors

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Intradural Extramedullary Tumors

Comprehensive guide to intradural extramedullary spinal tumors including schwannoma, meningioma, and neurofibroma - diagnosis, MRI features, and surgical management for FRACS exam

complete
Updated: 2025-12-24
High Yield Overview

INTRADURAL EXTRAMEDULLARY TUMORS

Schwannoma | Meningioma | Neurofibroma | Myxopapillary Ependymoma

40%Schwannoma (most common)
25%Meningioma
90%Benign histology
80%GTR achievable

COMMON INTRADURAL EXTRAMEDULLARY TUMORS

Schwannoma
PatternMost common IDEM tumor, nerve root origin
TreatmentGTR curative in most cases
Meningioma
PatternSecond most common, dura origin, thoracic
TreatmentGTR with dural resection
Neurofibroma
PatternAssociated with NF1, nerve root infiltration
TreatmentMay require root sacrifice
Myxopapillary Ependymoma
PatternFilum terminale, young adults
TreatmentGTR preferred, radiosensitive

Critical Must-Knows

  • Schwannoma vs Meningioma: Schwannoma is T2 hyperintense, meningioma is T2 iso/hypointense
  • Dural tail sign: Suggestive of meningioma (contrast-enhancing dura adjacent to tumor)
  • Dumbbell tumors: Neural foraminal extension, most commonly schwannoma (69%)
  • Gross total resection: Curative for most benign IDEM tumors
  • NF2 association: Multiple schwannomas/meningiomas, consider NF2 screening

Examiner's Pearls

  • "
    T2 bright = schwannoma, T2 iso/dark = meningioma
  • "
    Thoracic location + female = think meningioma
  • "
    Dumbbell shape + cervical = think schwannoma
  • "
    NF1 = neurofibromas, NF2 = schwannomas and meningiomas

Clinical Imaging

Imaging Gallery

(a and b) Axial contrast MRI showing small vestibular schwannomas within bilateral acoustic canal (black arrows) and a posterior parafalcine meningioma (white arrow). (c) Coronal MR cervical spine ima
Click to expand
(a and b) Axial contrast MRI showing small vestibular schwannomas within bilateral acoustic canal (black arrows) and a posterior parafalcine meningiomCredit: Aiyappan SK et al. via J Neurosci Rural Pract via Open-i (NIH) (Open Access (CC BY))
Forty-seven-year-old female with intradural extramedullary menigioma with paraparesis. (A) T1-weighted sagittal MR image shows homogenous high signal intensity mass. The tumor is located at the T10-11
Click to expand
Forty-seven-year-old female with intradural extramedullary menigioma with paraparesis. (A) T1-weighted sagittal MR image shows homogenous high signal Credit: Song KW et al. via Clin Orthop Surg via Open-i (NIH) (Open Access (CC BY))
Thirty-five-year-old male with intradural extramedullary schwannoma with both upper & lower extremity weakness and tingling sensation below C3 level. (A) T2-weighted sagittal MR image shows a huge
Click to expand
Thirty-five-year-old male with intradural extramedullary schwannoma with both upper & lower extremity weakness and tingling sensation below C3 levCredit: Song KW et al. via Clin Orthop Surg via Open-i (NIH) (Open Access (CC BY))
A man in his late 40s with a schwannoma at C1–C2. A) Post-contrast sagittal T1-weighted MRI showing the enhancing tumor. B) Axial T2-weighted MRI showing the left-sided tumor, which involved the C1–C2
Click to expand
A man in his late 40s with a schwannoma at C1–C2. A) Post-contrast sagittal T1-weighted MRI showing the enhancing tumor. B) Axial T2-weighted MRI showCredit: Open-i / NIH via Open-i (NIH) (Open Access (CC BY))

Critical IDEM Tumor Exam Points

MRI Differentiation

Schwannoma: T2 hyperintense, may have cystic change, heterogeneous enhancement. Meningioma: T2 iso/hypointense, homogeneous enhancement, dural tail sign. Signal intensity ratio on T2 differentiates reliably.

Location Pattern

Meningioma: 80% thoracic, lateral or posterolateral, 80% female. Schwannoma: Any level, often cervical/lumbar, equal gender. Ependymoma: Lumbar, filum terminale.

Dumbbell Tumors

Neural foraminal extension creates dumbbell shape. Most common cause is schwannoma (69%). Consider stability if large extraforaminal component. May need combined anterior-posterior approach.

NF Association

NF1 (von Recklinghausen): Neurofibromas, plexiform neurofibromas. NF2: Bilateral vestibular schwannomas PLUS spinal schwannomas/meningiomas. Multiple tumors = screen for NF2.

Schwannoma vs Meningioma - Key Differences

FeatureSchwannomaMeningioma
T2 SignalHYPERINTENSE (bright)Iso/HYPOINTENSE (dark)
EnhancementHeterogeneous, may be cysticHomogeneous
Dural tailAbsent/rarePresent (64%)
LocationAny level, cervical common80% thoracic
GenderM = F80% female
Foraminal extensionCommon (29%)Rare (3%)
Cystic changeCommon (96%)Rare (24%)
Nerve root originYes (displaced/encased)No (dural origin)
Mnemonic

IDEM Tumors - By Frequency

S
Schwannoma
40% - most common, nerve sheath origin, T2 bright
M
Meningioma
25% - dural origin, thoracic, female, dural tail
N
Neurofibroma
15% - NF1 associated, infiltrative, root sacrifice may be needed
E
Ependymoma
10% - filum terminale, myxopapillary type, young adults

Memory Hook:SMNE - Schwannoma Most common, Neurofibromas with NF1, Ependymoma at filum

Mnemonic

Schwannoma vs Meningioma MRI

S
Signal T2 high
Schwannoma is T2 hyperintense (bright)
C
Cystic change
Common in schwannoma (96%), rare in meningioma
H
Heterogeneous enhancement
Schwannoma enhances heterogeneously
M
Meningioma dark
T2 iso/hypointense, homogeneous
D
Dural tail
Characteristic of meningioma (64%)

Memory Hook:SCHMD - Schwannoma is bright, Cystic, Heterogeneous; Meningioma is Dark with Dural tail

Mnemonic

NF1 vs NF2 Features

NF1
Neurofibromatosis Type 1
Neurofibromas, cafe-au-lait spots, plexiform, chromosome 17
NF2
Neurofibromatosis Type 2
Bilateral vestibular schwannomas, spinal schwannomas/meningiomas, chromosome 22

Memory Hook:NF1 = Neurofibromas (type 1), NF2 = schwannomas times 2 (bilateral)

Overview and Epidemiology

Intradural extramedullary (IDEM) tumors are located within the dural sac but outside the spinal cord parenchyma. They account for 55-75% of all intradural spinal tumors and are predominantly benign.

Distribution of IDEM Tumors:

Tumor TypeFrequencyPeak AgeGender
Schwannoma40%30-60M = F
Meningioma25%40-70F more than M (4:1)
Neurofibroma15%30-50M = F
Myxopapillary Ependymoma10%20-40M more than F
Others10%VariableVariable

Key Epidemiological Points:

Meningiomas show strong female predominance (80%) and thoracic predilection. Schwannomas affect males and females equally and occur at any spinal level. Ependymomas of the filum terminale (myxopapillary type) are the most common primary tumor of the conus/cauda equina region.

NF2 Screening

If a patient presents with multiple schwannomas or meningiomas, screen for Neurofibromatosis Type 2. NF2 is characterized by bilateral vestibular schwannomas and multiple spinal tumors. Chromosome 22 mutation.

Pathophysiology

Tumor Origins

Nerve Sheath Origin

Schwannomas arise from Schwann cells of spinal nerve roots. They are encapsulated tumors that displace rather than infiltrate nerve fibers.

Histology:

  • Antoni A areas: Cellular, organized palisading (Verocay bodies)
  • Antoni B areas: Loose, myxoid, less cellular
  • S-100 protein positive

Growth Pattern:

  • Eccentric growth from nerve root
  • Nerve fibers displaced around capsule
  • Usually single nerve root involved
  • Nerve can often be preserved at surgery

Malignant transformation is extremely rare in sporadic schwannomas but may occur in NF2.

Dural/Arachnoid Origin

Spinal meningiomas arise from arachnoid cap cells in the spinal meninges, most commonly at the posterolateral aspect of the dura.

Histology:

  • Meningothelial (most common spinal type)
  • Psammomatous (with calcifications)
  • Transitional
  • WHO Grade I (benign) in 90%+ cases

Growth Pattern:

  • Broad dural attachment
  • May calcify (psammoma bodies)
  • Typically well-circumscribed
  • Dural tail on MRI reflects reactive dural thickening

Association with female gender and hormonal factors (progesterone receptors often positive).

Nerve Root Infiltrative

Unlike schwannomas, neurofibromas contain both Schwann cells and fibroblasts, and nerve fibers are incorporated within the tumor.

Histology:

  • Schwann cells + fibroblasts + collagen
  • Nerve fibers pass THROUGH tumor
  • S-100 positive (Schwann cell component)
  • CD34 positive (fibroblast component)

Types:

  • Localized: Single nerve, similar to schwannoma
  • Plexiform: Bag of worms appearance, pathognomonic of NF1
  • Diffuse: Poorly circumscribed

Important difference from schwannoma: nerve root sacrifice usually required for complete resection due to infiltrative nature.

Ependymal Cell Origin

Myxopapillary ependymoma is a distinct subtype arising from ependymal cells of the filum terminale.

Histology:

  • Papillary architecture with myxoid cores
  • GFAP positive
  • WHO Grade II (but can seed CSF if violated)

Characteristics:

  • Arises from filum terminale
  • Well-circumscribed, encapsulated
  • Contains mucin (T2 hyperintense)
  • May have hemorrhagic areas

Important: Despite benign histology, tumor capsule violation at surgery increases risk of CSF seeding and recurrence.

Classification

Spinal Tumor Compartments

CompartmentLocationCommon Tumors
ExtraduralOutside duraMetastases, primary bone tumors
Intradural ExtramedullaryInside dura, outside cordSchwannoma, meningioma, neurofibroma
IntramedullaryWithin spinal cordEpendymoma, astrocytoma, hemangioblastoma

IDEM tumors displace the spinal cord rather than infiltrate it. This generally allows preservation of cord function with surgical resection.

WHO Classification

Schwannoma:

  • WHO Grade I (benign)
  • Cellular schwannoma: Grade I
  • Malignant peripheral nerve sheath tumor (MPNST): Grade III/IV

Meningioma:

  • Grade I (benign): 90%+ of spinal meningiomas
  • Grade II (atypical): 5-10%
  • Grade III (anaplastic): Less than 1%

Neurofibroma:

  • WHO Grade I
  • MPNST transformation risk in NF1 (8-13% lifetime)

Myxopapillary Ependymoma:

  • WHO Grade II
  • Low malignant potential but CSF seeding possible

Spinal meningiomas are almost always benign (Grade I), unlike intracranial meningiomas.

Eden Classification for Dumbbell Tumors

Tumors with both intraspinal and extraspinal components.

TypeLocation
Type IPurely intraspinal
Type IIaForaminal extension
Type IIbParaspinal extension
Type IIIBoth foraminal and paraspinal

Surgical Implications:

  • Type I: Posterior approach alone
  • Type II/III: May need combined anterior-posterior approach
  • Large extraforaminal component: Assess stability
  • Vertebral body involvement: May need reconstruction

Schwannomas account for 69% of dumbbell tumors.

Neurofibromatosis Classification

NF1 (von Recklinghausen Disease):

  • Chromosome 17q11.2 (neurofibromin gene)
  • Diagnostic criteria: 2 or more of: cafe-au-lait spots (6+), neurofibromas (2+), plexiform neurofibroma, axillary/groin freckling, optic glioma, Lisch nodules, bony dysplasia, first-degree relative
  • Spinal tumors: Neurofibromas (plexiform common)

NF2:

  • Chromosome 22q12 (merlin gene)
  • Diagnostic criteria: Bilateral vestibular schwannomas OR family history + unilateral VS + 2 of (meningioma, schwannoma, glioma, neurofibroma, cataract)
  • Spinal tumors: Multiple schwannomas AND meningiomas

If multiple spinal tumors found, always consider NF2 screening.

Clinical Presentation

Presenting Symptoms

Pain (Most Common Initial Symptom):

  • Localized back pain (70%)
  • Radicular pain following nerve root distribution
  • May precede neurological deficit by months to years
  • Night pain common

Neurological Deficit:

  • Sensory changes (numbness, paresthesias)
  • Motor weakness (progressive)
  • Bladder/bowel dysfunction (late)
  • Gait disturbance

Tumor-Specific Presentations

Schwannoma:

  • Radicular pain in dermatomal distribution
  • May have sensory loss in affected root
  • Motor weakness if motor root involved

Meningioma:

  • Often presents with progressive myelopathy
  • Gait disturbance, spasticity
  • Brown-Sequard syndrome if lateral compression
  • May have minimal radicular symptoms

Myxopapillary Ependymoma:

  • Low back pain
  • Cauda equina symptoms (bowel/bladder, saddle anesthesia)
  • Often long history before diagnosis

Examination Findings

Upper Motor Neuron Signs (cord compression):

  • Spasticity, hyperreflexia below lesion
  • Positive Babinski
  • Clonus

Lower Motor Neuron Signs (root compression):

  • Weakness in myotomal distribution
  • Hyporeflexia at affected level
  • Muscle atrophy

Cauda Equina Syndrome

Tumors of the lumbar spine may cause cauda equina syndrome with saddle anesthesia, urinary retention, and bilateral leg weakness. This is a surgical emergency requiring urgent decompression.

Investigations

MRI - Gold Standard

Standard Sequences

Essential for IDEM tumor assessment.

T1-Weighted:

  • Tumors usually isointense to cord
  • Defines anatomical relationships
  • Pre-contrast baseline

T2-Weighted/STIR:

  • Schwannoma: Hyperintense
  • Meningioma: Iso/hypointense
  • Key differentiating sequence
  • Shows cord edema

Post-Gadolinium T1:

  • All IDEM tumors enhance
  • Schwannoma: Heterogeneous
  • Meningioma: Homogeneous + dural tail

Additional: Whole spine MRI to exclude skip lesions or NF2.

MRI Characteristics

T1: Iso/hypointense to cord

T2: HYPERINTENSE (key feature)

Enhancement: Heterogeneous, may have non-enhancing cystic areas

Other Features:

  • Eccentric to nerve root
  • Foraminal extension (29%)
  • Cystic change common (96%)
  • Bone erosion with dumbbell tumors
  • Round shape

Signal Intensity Ratio: Schwannoma/fat ratio on T2 is significantly higher than meningioma.

MRI Characteristics

T1: Isointense to cord

T2: Iso/HYPOINTENSE (key feature)

Enhancement: Homogeneous, avid

Other Features:

  • Broad dural base
  • Dural tail sign (64%)
  • Calcification may be present (dark on all sequences)
  • Thoracic location (80%)
  • Posterolateral position
  • Ginkgo leaf shape

The dural tail is suggestive but not pathognomonic for meningioma.

Neurofibroma:

  • T2 hyperintense (like schwannoma)
  • Target sign: Central low T2 signal
  • Plexiform: Bag of worms appearance
  • May be multiple in NF1

Myxopapillary Ependymoma:

  • T2 hyperintense
  • Well-circumscribed at filum terminale
  • Homogeneous enhancement
  • May have hemorrhage (T1 bright areas)

Drop Metastases:

  • Multiple nodular lesions
  • Coating of nerve roots
  • Consider primary CNS tumor (medulloblastoma, ependymoma)

Whole spine MRI essential to identify additional lesions.

Additional Investigations

CT Scan:

  • Bone erosion (dumbbell tumors)
  • Calcification (meningioma)
  • Pre-operative planning

Blood Tests:

  • Routine pre-operative workup
  • Consider genetic testing if NF suspected

Management

Surgical Management

Standard Posterior Approach

Most IDEM tumors approached posteriorly.

Laminectomy vs Laminoplasty:

  • Laminectomy: Standard approach
  • Laminoplasty: May reduce post-laminectomy kyphosis
  • Consider instrumented fusion if extensive laminectomy (3+ levels)

Technical Steps:

  1. Posterior midline incision
  2. Laminectomy/laminoplasty
  3. Durotomy (midline or paramedian)
  4. Tumor identification
  5. Microsurgical resection
  6. Watertight dural closure
  7. Bone replacement if laminoplasty

Intraoperative neurophysiological monitoring (SSEP, MEP) recommended.

Surgical Technique

Goal: Gross total resection with nerve preservation if possible.

Key Points:

  • Tumor usually displaces nerve fibers
  • Identify functioning fascicles with stimulation
  • Peel tumor from nerve capsule
  • Sacrifice parent root only if necessary (sensory root often tolerated)
  • GTR achievable in 90%+

Dumbbell Tumors:

  • May need combined anterior-posterior approach
  • Large extraforaminal component: Stage surgery
  • Vascular consideration (vertebral artery in cervical region)

Long-term control excellent with GTR (less than 5% recurrence).

Surgical Technique

Goal: Gross total resection with dural excision.

Key Points:

  • Identify dural attachment
  • Circumferential dissection from cord
  • Resect involved dura (Simpson Grade II)
  • Primary dural closure or graft
  • Coagulate dural margin if excision not possible

Simpson Grading (for recurrence prediction):

  • Grade I: GTR + dural excision + bone removal
  • Grade II: GTR + coagulation of dural attachment
  • Grade III: GTR without dural treatment
  • Grade IV: Subtotal resection
  • Grade V: Decompression only

GTR achievable in 85-95% of spinal meningiomas.

Surgical Technique

Goal: En bloc resection without capsule violation.

Critical Points:

  • Myxopapillary ependymoma has excellent prognosis with GTR
  • Capsule violation increases CSF seeding risk
  • En bloc resection preferred over piecemeal
  • Preserve nerve roots where possible

Post-operative:

  • If GTR achieved: Observation
  • If STR or capsule violated: Radiation therapy
  • Follow-up MRI annually

Prognosis:

  • GTR with intact capsule: more than 90% 10-year survival
  • STR or capsule violation: 50-70% recurrence

En bloc resection is critical for long-term disease control.

Non-Surgical Management

Observation:

  • Small, asymptomatic tumors
  • Elderly or medically unfit patients
  • Slow-growing tumors (serial MRI monitoring)

Radiation:

  • Rarely used for benign IDEM tumors
  • Consider for recurrent/incompletely resected meningioma
  • SRS (stereotactic radiosurgery) for small recurrences
  • Primary treatment if patient not surgical candidate

Nerve Root Sacrifice

For schwannomas, the parent nerve root can often be preserved because the tumor displaces rather than infiltrates nerve fibers. For neurofibromas, nerve fibers pass through the tumor, and root sacrifice is often necessary for complete resection.

Complications

Surgical Complications

Early:

  • CSF leak (most common, 5-10%)
  • Wound infection
  • New neurological deficit (2-5%)
  • Hematoma

Late:

  • Post-laminectomy kyphosis
  • Tumor recurrence
  • Chronic pain
  • Arachnoiditis

CSF Leak Prevention

Intraoperative:

  • Meticulous dural closure
  • Dural grafting if primary closure not possible
  • Fibrin sealant augmentation
  • Watertight closure in layers

If CSF Leak Occurs:

  • Wound care
  • Lumbar drain (3-5 days)
  • Re-exploration if conservative measures fail

Recurrence Rates

TumorGTR RecurrenceSTR Recurrence
SchwannomaLess than 5%30-40%
Meningioma5-10%20-30%
NeurofibromaVariableHigher with NF1
Myxopapillary EpendymomaLess than 10%50-70%

Evidence Base

Schwannoma vs Meningioma MRI Differentiation

III
Maki S et al. • Br J Radiol (2019)
Key Findings:
  • Signal intensity ratio on T2 differentiates schwannoma from meningioma
  • Schwannoma: Higher T2/fat signal ratio
  • Cystic change: 96% schwannoma vs 24% meningioma
  • Dural tail: 64% meningioma vs 1% schwannoma
Clinical Implication: T2 signal intensity and morphological features on MRI can reliably differentiate schwannoma from meningioma preoperatively

Spinal Schwannoma Surgical Outcomes

IV
Seppala MT et al. • J Neurosurg (1995)
Key Findings:
  • 187 patients with spinal schwannoma
  • GTR achieved in 93%
  • Recurrence rate 3% with GTR
  • Nerve root function preserved in majority
Clinical Implication: Gross total resection of spinal schwannomas provides excellent long-term tumor control with low recurrence rates

Spinal Meningioma Surgery

IV
Gottfried ON et al. • J Neurosurg Spine (2003)
Key Findings:
  • GTR achievable in 89-100%
  • Functional improvement in 80%+
  • Simpson Grade influences recurrence
  • Long-term recurrence 5-10%
Clinical Implication: Spinal meningiomas have excellent surgical outcomes with low recurrence when gross total resection is achieved

Myxopapillary Ependymoma Outcomes

III
Weber DC et al. • Neuro Oncol (2015)
Key Findings:
  • En bloc resection associated with better outcomes
  • Capsule violation increases recurrence risk
  • GTR alone: 92% 10-year PFS
  • STR with radiation: 75% 10-year PFS
Clinical Implication: En bloc gross total resection without capsule violation is critical for long-term control of myxopapillary ependymoma

Exam Viva Scenarios

Practice these scenarios to excel in your viva examination

VIVA SCENARIOStandard

Thoracic IDEM Tumor in Female

EXAMINER

"A 55-year-old woman presents with 6 months of progressive gait difficulty and band-like thoracic pain. MRI shows a T6-T7 intradural extramedullary mass that is isointense on T2, enhances homogeneously, and has a dural tail sign."

EXCEPTIONAL ANSWER
**Most Likely Diagnosis:** Spinal meningioma - based on: - T2 isointense (not hyperintense like schwannoma) - Homogeneous enhancement - Dural tail sign (64% specific for meningioma) - Thoracic location (80% of spinal meningiomas) - Female gender (4:1 female predominance) **Pre-operative Assessment:** 1. Full neurological examination (document ASIA score) 2. Medical fitness for surgery 3. MRI whole spine (rule out additional tumors/NF2) 4. CT if concern about calcification or bone involvement **Surgical Management:** 1. Position: Prone 2. Approach: Posterior midline 3. T5-T8 laminectomy or laminoplasty 4. Midline durotomy 5. Microsurgical dissection from cord 6. Identify dural attachment 7. Resect with dural margin (Simpson Grade II) 8. Primary dural closure or graft 9. Neurophysiological monitoring throughout **Post-operative:** - Early mobilization - MRI at 3 months for baseline - Annual MRI surveillance - GTR = 5-10% recurrence at 10 years **Key Point:** T2 isointense with dural tail = meningioma until proven otherwise.
KEY POINTS TO SCORE
T2 iso/hypointense + dural tail = meningioma
80% of spinal meningiomas are thoracic
4:1 female predominance
Simpson Grade II (GTR + dural coagulation) is goal
Excellent prognosis with GTR
COMMON TRAPS
✗Calling it schwannoma (T2 bright, no dural tail)
✗Forgetting to image whole spine for NF2
✗Not achieving dural margin excision/coagulation
VIVA SCENARIOChallenging

Cervical Dumbbell Tumor

EXAMINER

"A 45-year-old man presents with right-sided neck pain and C6 radiculopathy. MRI shows a C5-C6 intradural extramedullary tumor that is T2 hyperintense with heterogeneous enhancement. There is foraminal extension with an extraspinal component."

EXCEPTIONAL ANSWER
**Diagnosis:** Dumbbell schwannoma - based on: - T2 hyperintense (characteristic of schwannoma) - Heterogeneous enhancement - Neural foraminal extension (29% of schwannomas vs 3% meningiomas) - Radicular symptoms - Male patient (meningioma rare in males) **Classification:** Eden Type II or III dumbbell tumor (foraminal and paraspinal extension). **Pre-operative Planning:** 1. CT angiography - relationship to vertebral artery 2. Assess size of extraforaminal component 3. Evaluate for spinal instability 4. Consider embolization if highly vascular **Surgical Approach Options:** **Option 1: Posterior Alone (if small extraforaminal)** - Laminectomy C5-C6 - Extended foraminotomy - Intraspinal tumor resection - Follow tumor into foramen - May need partial facetectomy **Option 2: Combined Anterior-Posterior (if large extraforaminal)** - Stage 1: Posterior laminectomy + intraspinal resection - Stage 2: Anterior approach for extraspinal component - Consider single-stage combined if experienced team **Intraoperative Considerations:** - Neurophysiological monitoring - Identify C6 nerve root - Attempt nerve preservation (stimulate fascicles) - Vertebral artery protection - May need C5-C6 instrumented fusion if extensive facetectomy **Outcomes:** - GTR achievable in 80-90% of dumbbell tumors - Nerve root sacrifice may be necessary - Sensory root sacrifice usually well tolerated
KEY POINTS TO SCORE
T2 hyperintense + foraminal extension = schwannoma
69% of dumbbell tumors are schwannomas
Combined approach may be needed for large extraspinal component
Vertebral artery at risk in cervical region
Nerve preservation often possible (tumor displaces fibers)
COMMON TRAPS
✗Attempting posterior alone for large extraforaminal tumor
✗Forgetting CT angiography for vertebral artery
✗Not considering instability after extended facetectomy
VIVA SCENARIOStandard

Filum Terminale Tumor in Young Adult

EXAMINER

"A 28-year-old man presents with 18 months of low back pain and recent onset of urinary hesitancy. MRI shows a well-circumscribed L3-L4 intradural tumor arising from the filum terminale. It is T2 hyperintense with homogeneous enhancement."

EXCEPTIONAL ANSWER
**Diagnosis:** Myxopapillary ependymoma - based on: - Young adult male (typical demographics) - Filum terminale origin (pathognomonic location) - Well-circumscribed - T2 hyperintense - Homogeneous enhancement **WHO Grade II** - but CSF seeding risk if capsule violated. **Pre-operative Considerations:** 1. MRI whole spine and brain - exclude drop metastases 2. Document baseline neurological function (ASIA, bladder/bowel) 3. Counsel regarding surgical risks (bladder, sexual function) **Critical Surgical Principles:** **1. EN BLOC RESECTION ESSENTIAL** - Do NOT violate tumor capsule - Capsule rupture = CSF seeding risk - Piecemeal resection = 50-70% recurrence - En bloc GTR = less than 10% recurrence **2. Technical Approach:** - L2-L5 laminectomy - Midline durotomy - Identify tumor attachment to filum - Divide filum above and below tumor - Remove en bloc - Examine for any CSF seeding **3. Nerve Root Protection:** - Use stimulation to identify motor roots - Cauda equina roots draped over tumor - Meticulous microsurgical dissection **Post-operative:** - If GTR with intact capsule: Observation - If STR or capsule violated: Adjuvant radiation - MRI surveillance (brain and spine) annually **Prognosis:** - GTR intact capsule: more than 90% 10-year survival - STR: 50% 10-year PFS (better with radiation)
KEY POINTS TO SCORE
Filum terminale origin = myxopapillary ependymoma
EN BLOC resection is CRITICAL
Capsule violation = CSF seeding and recurrence
Piecemeal resection has 50-70% recurrence
Radiation for STR or capsule violation
COMMON TRAPS
✗Performing piecemeal resection
✗Violating tumor capsule
✗Not imaging entire neuraxis
✗Not offering radiation for incomplete resection
VIVA SCENARIOChallenging

Multiple IDEM Tumors

EXAMINER

"A 32-year-old woman presents with progressive gait ataxia. MRI shows three intradural extramedullary tumors at C3, T6, and L2. She also reports hearing loss for 2 years."

EXCEPTIONAL ANSWER
**Suspected Diagnosis:** Neurofibromatosis Type 2 (NF2) - based on: - Multiple spinal intradural tumors - Young female - Hearing loss (suggests vestibular schwannoma) **NF2 Diagnostic Criteria:** 1. Bilateral vestibular schwannomas, OR 2. First-degree relative with NF2 + unilateral VS + 2 of: meningioma, schwannoma, glioma, neurofibroma, cataract **Investigation:** 1. MRI internal auditory meatus - bilateral vestibular schwannomas? 2. MRI whole spine - extent of spinal disease 3. MRI brain - intracranial meningiomas? 4. Audiometry - document hearing 5. Ophthalmology - cataracts, retinal hamartomas 6. Genetic testing - NF2 gene (chromosome 22q12) **If NF2 Confirmed:** 1. Family screening (autosomal dominant) 2. Genetic counseling 3. Multidisciplinary team (neurosurgery, ENT, ophthalmology, genetics) **Surgical Strategy in NF2:** - Prioritize symptomatic tumors - Hearing preservation for VS (when possible) - May need multiple staged surgeries - Conservative approach for slow-growing asymptomatic tumors - Bevacizumab (anti-VEGF) may slow tumor growth **This Patient:** - Gait ataxia suggests T6 tumor may be causing myelopathy - Prioritize symptomatic T6 lesion - C3 and L2 may be observed if asymptomatic - Address hearing loss/VS separately **Prognosis:** - Life expectancy reduced in NF2 - Multiple surgeries typically required - Hearing loss common - Quality of life focus important
KEY POINTS TO SCORE
Multiple spinal tumors + hearing loss = NF2 until proven otherwise
NF2 = chromosome 22 (merlin gene)
Bilateral vestibular schwannomas are hallmark
Screen first-degree relatives
Prioritize symptomatic tumors for surgery
COMMON TRAPS
✗Not recognizing pattern of NF2
✗Operating on all tumors simultaneously
✗Forgetting to image brain and IAMs
✗Not offering genetic testing/family screening

Management Algorithm

📊 Management Algorithm
Management algorithm for Intradural Extramedullary Tumors
Click to expand
Management algorithm for Intradural Extramedullary TumorsCredit: OrthoVellum

INTRADURAL EXTRAMEDULLARY TUMORS

High-Yield Exam Summary

Schwannoma vs Meningioma

  • •Schwannoma: T2 BRIGHT, heterogeneous, cystic, foraminal extension
  • •Meningioma: T2 ISO/DARK, homogeneous, dural tail, thoracic
  • •Signal intensity ratio on T2 reliably differentiates
  • •Schwannoma = any level, Meningioma = 80% thoracic

Tumor Frequencies

  • •Schwannoma: 40% (most common)
  • •Meningioma: 25% (80% female)
  • •Neurofibroma: 15% (NF1 associated)
  • •Myxopapillary ependymoma: 10% (filum terminale)

Surgical Goals

  • •GTR is curative for most IDEM tumors
  • •Schwannoma: Nerve preservation often possible
  • •Meningioma: Simpson Grade II (GTR + dural excision)
  • •Ependymoma: EN BLOC essential (capsule violation = seeding)

Dumbbell Tumors

  • •69% are schwannomas
  • •Eden Classification: I-III based on extension
  • •Combined approach may be needed
  • •Assess stability if facetectomy required

NF Association

  • •NF1: Neurofibromas, plexiform, cafe-au-lait, chr 17
  • •NF2: Schwannomas + meningiomas, bilateral VS, chr 22
  • •Multiple spinal tumors = screen for NF2
  • •NF2 requires multidisciplinary approach

Complications

  • •CSF leak most common (5-10%)
  • •Post-laminectomy kyphosis (consider fusion if 3+ levels)
  • •Recurrence: GTR less than 5-10%, STR 30-40%
  • •Neurological deficit rare with microsurgical technique

Australian Context

Intradural extramedullary spinal tumors are managed by neurosurgeons and spinal surgeons across Australian tertiary centers. Comprehensive neurosurgical units with intraoperative neurophysiological monitoring capability are recommended for optimal outcomes.

For patients with suspected Neurofibromatosis Type 2, referral to specialist NF clinics (available at major children's hospitals and some adult centers) provides coordinated multidisciplinary care including genetic counseling, hearing assessment, and surgical planning.

Access to microsurgical techniques and intraoperative monitoring has significantly improved outcomes for IDEM tumor surgery in Australia, with gross total resection rates comparable to international centers of excellence.

References

  1. Maki S, Yamamoto K, Nakagawa Y, et al. Differentiating spinal intradural-extramedullary schwannoma from meningioma using MRI T2 weighted images. Br J Radiol. 2019;92(1093):20180262.
  2. Seppala MT, Haltia MJ, Sankila RJ, et al. Long-term outcome after removal of spinal schwannoma: a clinicopathological study of 187 cases. J Neurosurg. 1995;83(4):621-6.
  3. Gottfried ON, Gluf W, Quinones-Hinojosa A, et al. Spinal meningiomas: surgical management and outcome. Neurosurg Focus. 2003;14(6):e2.
  4. Weber DC, Wang Y, Miller R, et al. Long-term outcome of patients with spinal myxopapillary ependymoma: treatment results from the MD Anderson Cancer Center and institutions from the Rare Cancer Network. Neuro Oncol. 2015;17(4):588-95.
  5. Jinnai T, Koyama T. Clinical characteristics of spinal nerve sheath tumors: analysis of 149 cases. Neurosurgery. 2005;56(3):510-5.
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