INTRADURAL EXTRAMEDULLARY TUMORS
Schwannoma | Meningioma | Neurofibroma | Myxopapillary Ependymoma
COMMON INTRADURAL EXTRAMEDULLARY TUMORS
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




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
| Feature | Schwannoma | Meningioma |
|---|---|---|
| T2 Signal | HYPERINTENSE (bright) | Iso/HYPOINTENSE (dark) |
| Enhancement | Heterogeneous, may be cystic | Homogeneous |
| Dural tail | Absent/rare | Present (64%) |
| Location | Any level, cervical common | 80% thoracic |
| Gender | M = F | 80% female |
| Foraminal extension | Common (29%) | Rare (3%) |
| Cystic change | Common (96%) | Rare (24%) |
| Nerve root origin | Yes (displaced/encased) | No (dural origin) |
IDEM Tumors - By Frequency
Memory Hook:SMNE - Schwannoma Most common, Neurofibromas with NF1, Ependymoma at filum
Schwannoma vs Meningioma MRI
Memory Hook:SCHMD - Schwannoma is bright, Cystic, Heterogeneous; Meningioma is Dark with Dural tail
NF1 vs NF2 Features
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 Type | Frequency | Peak Age | Gender |
|---|---|---|---|
| Schwannoma | 40% | 30-60 | M = F |
| Meningioma | 25% | 40-70 | F more than M (4:1) |
| Neurofibroma | 15% | 30-50 | M = F |
| Myxopapillary Ependymoma | 10% | 20-40 | M more than F |
| Others | 10% | Variable | Variable |
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.
Classification
Spinal Tumor Compartments
| Compartment | Location | Common Tumors |
|---|---|---|
| Extradural | Outside dura | Metastases, primary bone tumors |
| Intradural Extramedullary | Inside dura, outside cord | Schwannoma, meningioma, neurofibroma |
| Intramedullary | Within spinal cord | Ependymoma, astrocytoma, hemangioblastoma |
IDEM tumors displace the spinal cord rather than infiltrate it. This generally allows preservation of cord function with surgical resection.
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.
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:
- Posterior midline incision
- Laminectomy/laminoplasty
- Durotomy (midline or paramedian)
- Tumor identification
- Microsurgical resection
- Watertight dural closure
- Bone replacement if laminoplasty
Intraoperative neurophysiological monitoring (SSEP, MEP) recommended.
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
| Tumor | GTR Recurrence | STR Recurrence |
|---|---|---|
| Schwannoma | Less than 5% | 30-40% |
| Meningioma | 5-10% | 20-30% |
| Neurofibroma | Variable | Higher with NF1 |
| Myxopapillary Ependymoma | Less than 10% | 50-70% |
Evidence Base
Schwannoma vs Meningioma MRI Differentiation
- 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
Spinal Schwannoma Surgical Outcomes
- 187 patients with spinal schwannoma
- GTR achieved in 93%
- Recurrence rate 3% with GTR
- Nerve root function preserved in majority
Spinal Meningioma Surgery
- GTR achievable in 89-100%
- Functional improvement in 80%+
- Simpson Grade influences recurrence
- Long-term recurrence 5-10%
Myxopapillary Ependymoma Outcomes
- 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
Exam Viva Scenarios
Practice these scenarios to excel in your viva examination
Thoracic IDEM Tumor in Female
"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."
Cervical Dumbbell Tumor
"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."
Filum Terminale Tumor in Young Adult
"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."
Multiple IDEM Tumors
"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."
Management Algorithm

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
- 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.
- 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.
- Gottfried ON, Gluf W, Quinones-Hinojosa A, et al. Spinal meningiomas: surgical management and outcome. Neurosurg Focus. 2003;14(6):e2.
- 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.
- Jinnai T, Koyama T. Clinical characteristics of spinal nerve sheath tumors: analysis of 149 cases. Neurosurgery. 2005;56(3):510-5.