287 lines
26 KiB
Markdown
287 lines
26 KiB
Markdown
---
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title: "Intramedullary Mass"
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docid: "635c114e-6260-4e9d-a36a-368deeb05f05"
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authors:
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- key: "bee1f359-33fb-4cba-9e6b-ed1ca1842439"
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value: "Jeffrey S. Ross, MD"
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- key: "bf7af3a5-468c-48d0-8aea-4f6e808af979"
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value: "Bryson Borg, MD"
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breadcrumbs:
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-
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name: "Spine"
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slug: "spine"
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treeNodeId: "b337a156-914a-4696-a77c-af206720fab5"
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-
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name: "Differential Diagnosis"
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slug: "differential-diagnosis"
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treeNodeId: "ef5fd925-2033-4f3b-aa7c-640fef9aa956"
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-
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name: "Intramedullary"
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slug: "intramedullary"
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treeNodeId: "b72eef28-6a45-4665-8758-eb14b7ac4462"
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-
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name: "Anatomically Based Differentials"
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slug: "anatomically-based-differentials"
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treeNodeId: "3c8e44bc-84bc-455a-b832-a139b0d0a604"
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-
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name: "Intramedullary Mass"
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slug: "intramedullary-mass"
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treeNodeId: null
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category: "Spine"
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cmeTopicId: "6ba91891-8860-49fd-a3a6-ba09453efe41"
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documentVersionId: "e4262d97-22fd-491c-8854-f05e2f9a215d"
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imageCount: 32
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lastUpdated: "02/14/23"
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pageDescription: "Intramedullary Mass"
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pageKeywords: "Spine, Differential Diagnosis, Intramedullary, Anatomically Based Differentials, Intramedullary Mass"
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pageTitle: "Intramedullary Mass | STATdx"
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enhancedTitle: "Intramedullary Mass"
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type: "DDX"
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references: true
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breadcrumbs:
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- "Spine"
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- "Differential Diagnosis"
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- "Intramedullary"
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- "Anatomically Based Differentials"
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- "Intramedullary Mass"
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---
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# ESSENTIAL INFORMATION
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- ## Key Differential Diagnosis Issues
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- MR ± contrast is best tool to evaluate intramedullary processes of cord
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- Discovery of intramedullary cord lesion typically followed by imaging of remainder of neuraxis
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- Infiltrative cord lesion: Image brain to potentially identify characteristic white matter lesion(s) of multiple sclerosis (MS)
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- Discovery of intramedullary tumor typically accompanied by insidious onset of myelopathic symptoms (months or years)
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- Patient with syrinx should be imaged at least once with contrast-enhanced MR to exclude cord neoplasm
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- Hemorrhagic lesion: Think ependymoma, cavernous malformation, contusion
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- ## Helpful Clues for Common Diagnoses
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- **Multiple Sclerosis, Spinal Cord**
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- Typically located eccentrically, not involving entire cord on axial imaging; relatively short in length (< 2 vertebral bodies)
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- Enlargement of cord is unusual (6-14%)
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- Dissemination in time: Some enhance, some do not
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- 5-24% of patients with MS cord plaques may not have supratentorial disease at presentation
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- **Neuromyelitis Optica Spectrum Disorder**
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- Autoimmune astrocytopathic disease targeting water channel proteins (AQP4) with involvement of optic nerves and spinal cord with limited brain parenchymal involvement
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- Longitudinally extensive transverse myelitis (LETM) (> 3 vertebral segments) T2 hyperintensity within cord
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- Enhancement of optic nerves
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- Brain lesions not typical for MS
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- **Acute Disseminated Encephalitis, Spinal Cord**
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- Clinical: Self-limited, monophasic demyelinating illness 5-14 days following viral infection or vaccination
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- Usually indistinguishable from MS on imaging at single time point
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- **Acute Transverse Myelitis, Idiopathic**
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- Clinical: Acute-onset myelopathy, ascending or static loss of sensory and motor function in bilateral and symmetric distribution
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- Infiltrative signal abnormality may extend above level of deficit; variable enhancement
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- Mild fusiform enlargement may be present and simulate appearance of primary cord neoplasm
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- **Ependymoma, Spinal Cord**
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- Circumscribed, enhancing intramedullary mass; located centrally within cord
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- Can show signs of necrosis (heterogeneity, cyst formation) and hemorrhage (hyperintense T1, susceptibility artifact, hemosiderin cap sign)
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- Most common intramedullary neoplasm in adults
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- 10% may show very little enhancement
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- **Astrocytoma, Spinal Cord**
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- Fusiform enlargement, infiltrative margins, long segment of involvement; no or variable enhancement
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- Most commonly located in cervical and upper thoracic cord
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- Uncommon/rare imaging features: Hemorrhage, necrosis, caudal location, exophytic growth, holocord involvement
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- Cannot be reliably differentiated from ependymoma by imaging
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- 2nd most common cord neoplasm in adults; most common cord neoplasm in children (60%)
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- **Syringomyelia**
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- Abnormal cystic cord lesion with surrounding gliosis; variable expansion of cord; focal or extensive; typically longitudinal
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- Secondary to chronic insult/injury (cavitation) or to altered CSF dynamics in central canal of cord (technically termed hydromyelia, such as seen in Chiari 1 malformation)
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- ## Helpful Clues for Less Common Diagnoses
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- **Hemangioblastoma, Spinal Cord**
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- Intensely enhancing, hypervascular tumor(s); usually located dorsally within cord
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- Multiple lesions common (check posterior fossa)
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- May or may not have associated syrinx, which can be disproportionately large relative to size of actual enhancing tumor
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- Often with prominent serpiginous subarachnoid flow voids due to enlarged draining veins
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- 70-90%**not**associated with von Hippel-Lindau
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- **Intramedullary Arteriovenous Malformation**
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- Hyperintense T2 signal in cord
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- Tortuous vessels/flow voids on MR, hypervascularity on CT angiography
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- **Infarction, Spinal Cord**
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- Hyperintensity on T2WI, possibly with mild expansion
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- Conus and variable thoracic cord involvement, cervical ischemia is atypical
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- Most often associated with aortic pathology (dissection, thoracoabdominal aortic surgery), rarely with atherosclerotic disease or embolism
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- Look for associated vertebral body infarction with geographic areas of abnormal increased T2 and decreased enhancement
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- **Cavernous Malformation, Spinal Cord**
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- Variable hyperintensity on T1, heterogeneously hyperintense on T2 with surrounding rim of susceptibility due to prior episodes of hemorrhage that blooms on gradient-echo sequences
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- Rare enhancement; may have some surrounding edema if recent bleed
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- **Metastases, Spinal Cord**
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- Most common primary is lung, followed by breast
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- Melanoma often shows intrinsic T1 hyperintensity
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- **Lymphoma**
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- Relatively low signal on T2WI
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- Homogenous enhancement (like brain)
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- May be extradural, intradural extramedullary or intramedullary
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- ## Helpful Clues for Rare Diagnoses
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- **Sarcoidosis**
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- Mimic of other diseases; diagnosis usually preceded by known pulmonary or systemic involvement
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- ## Other Essential Information
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- Tumor associated syrinx (a.k.a., polar or satellite cysts): Intramedullary fluid collections rostral &/or caudal to tumor with nonenhancing margins
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- More rostral tumor associated with higher likelihood of syrinx formation
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## References
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# Selected References
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1. [Cooper D et al: Sarcoidosis. Emerg Med Clin North Am. 40(1):149-57, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=34782085%5Bpmid%5D)
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1. [Hussain I et al: Surgical management of intramedullary spinal cord tumors. Neurosurg Clin N Am. 31(2):237-49, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32147015%5Bpmid%5D)
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1. [Freund P et al: MRI in traumatic spinal cord injury: from clinical assessment to neuroimaging biomarkers. Lancet Neurol. 18(12):1123-35, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31405713%5Bpmid%5D)
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1. [Prasad S et al: What you need to know about AQP4, MOG, and NMOSD. Semin Neurol. 39(6):718-31, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=31847043%5Bpmid%5D)
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1. [Barnett Y et al: Conventional and advanced imaging in neuromyelitis optica. AJNR Am J Neuroradiol. 35(8):1458-66, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=23764723%5Bpmid%5D)
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1. [Mechtler LL et al: Spinal cord tumors: new views and future directions. Neurol Clin. 31(1):241-68, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23186903%5Bpmid%5D)
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1. [Sato DK et al: Aquaporin-4 antibody-positive cases beyond current diagnostic criteria for NMO spectrum disorders. Neurology. 80(24):2210-6, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=23677744%5Bpmid%5D)
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1. [West TW et al: Acute transverse myelitis: demyelinating, inflammatory, and infectious myelopathies. Semin Neurol. 32(2):97-113, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22961185%5Bpmid%5D)
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1. [Boström A et al: Surgery for spinal cord ependymomas: outcome and prognostic factors. Neurosurgery. 68(2):302-8; discussion 309, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21135741%5Bpmid%5D)
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1. [Rodallec MH et al: Diagnostic imaging of solitary tumors of the spine: what to do and say. Radiographics. 28(4):1019-41, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18635627%5Bpmid%5D)
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## Images
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### Selected Images
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**Multiple Sclerosis, Spinal Cord**
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*Sagittal T2 MR shows focal areas of increased signal <img src='img/arrows/WS.png'/> from the thoracic cord in this patient with multiple sclerosis. Cord expansion may occur in the acute phase but is uncommon.*
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**Multiple Sclerosis, Spinal Cord**
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*Sagittal T2 MR shows focal areas of increased signal <img src='img/arrows/WS.png'/> from the thoracic cord in this patient with multiple sclerosis. Cord expansion may occur in the acute phase but is uncommon.*
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**Neuromyelitis Optica Spectrum Disorder**
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*Sagittal T2 MR shows a longitudinally extensive region of increased signal intensity within the expanded cervical cord. Neuromyelitis optica lesions typically are > 3 vertebral body segments in length.*
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**Ependymoma, Spinal Cord**
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*Sagittal T1 MR shows expansion of the cervical cord with an isointense mass at the C3-C5 level <img src='img/arrows/WS.png'/> with a large linear area of low-signal hemorrhage extending inferiorly within the cord <img src='img/arrows/WO.png'/>.*
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**Ependymoma, Spinal Cord**
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*Sagittal T2 MR shows heterogenous expansion of the cervical cord from the ependymoma nidus <img src='img/arrows/WS.png'/> plus extension of hemorrhage inferiorly <img src='img/arrows/WC.png'/>.*
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**Syringomyelia**
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*Sagittal T1 MR shows well-defined fluid signal intensity from the upper cervical cord in this patient with a syrinx and prior posterior fossa decompression for Chiari 1 malformation.*
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**Contusion-Hematoma, Spinal Cord**
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*Sagittal T2 MR shows a severe flexion injury at C6-C7 with a traumatic disc herniation <img src='img/arrows/WS.png'/> and extensive contusion and hemorrhage within the cord <img src='img/arrows/WO.png'/>. Note the edema extending superiorly and inferiorly from the injury site <img src='img/arrows/WC.png'/>.*
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**Hemangioblastoma, Spinal Cord**
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*Sagittal T1 C+ FS MR shows multiple enhancing masses along and within the thoracic cord <img src='img/arrows/WS.png'/> in this patient with von Hippel-Lindau.*
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**Intramedullary Arteriovenous Malformation**
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*Sagittal T2 MR shows multiple prominent round flow voids <img src='img/arrows/WS.png'/> from nidal aneurysms and venous varices in this patient with a large conus-type arteriovenous malformation (AVM).*
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**Intramedullary Arteriovenous Malformation**
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*Sagittal T1 MR shows a small, nonspecific focus of low signal intensity within the cervical cord <img src='img/arrows/WS.png'/> at the C2-C3 level in this patient with a type II intramedullary AVM.*
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**Intramedullary Arteriovenous Malformation**
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*Sagittal T2 MR in this patient with a type II AVM shows the site of the nidus <img src='img/arrows/WS.png'/> as well as linear areas of hemosiderin deposition with the cord <img src='img/arrows/WO.png'/> from prior hemorrhage.*
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**Infarction, Spinal Cord**
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*Sagittal T2WI FSE MR shows ill-defined hyperintensity and mild enlargement of the conus medullaris <img src='img/arrows/WO.png'/> in this patient with lower extremity paralysis following aortic aneurysm repair.*
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**Cavernous Malformation, Spinal Cord**
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*Sagittal T2 MR shows slightly expansile intramedullary lesion at the C2-C3 level <img src='img/arrows/BO.png'/> with a classic heterogeneous hyperintense (popcorn) lesion surrounded by a hypointense hemosiderin rim.*
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**Metastases, Spinal Cord**
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*Sagittal T2WI STIR MR shows nonspecific expansion and hyperintensity within the cervical cord in this patient with metastatic disease.*
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**Metastases, Spinal Cord**
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*Sagittal T1 C+ MR shows a focal mass enhancing within the cervical cord in this patient with cord metastases <img src='img/arrows/WS.png'/>.*
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**Lymphoma**
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*Sagittal T1 C+ MR shows nonspecific enlargement and enhancement of the cervical cord. The differential would include tumors (astrocytoma) and granulomatous disease. This patient has path-proven lymphoma.*
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**Sarcoidosis**
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*Sagittal T1 C+ MR shows an ovoid focus of irregular enhancement within the cervical cord at the C6 and C7 levels <img src='img/arrows/WS.png'/>. This lesion was eventually diagnosed as sarcoidosis.*
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### Additional Images
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**Demyelinating Disease**
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*Sagittal T1 C+ MR shows 3 foci of pathologic enhancement <img src='img/arrows/WS.png'/> due to acute disseminated encephalomyelitis within the cervical cord in this patient with an acute illness following viral prodrome.*
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**Demyelinating Disease**
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*Sagittal T1 C+ MR shows patchy enhancement <img src='img/arrows/BS.png'/> and mild enlargement of cervical cord due to multiple sclerosis (Devic subtype).*
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**Ependymoma, Spinal Cord**
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*Sagittal T1 C+ MR shows the typical appearance of ependymoma. Note a circumscribed, enhancing mass in the central cord with tumor syrinx.*
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**Ependymoma, Spinal Cord**
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*Sagittal T1 C+ MR shows an enhancing intraaxial mass <img src='img/arrows/BO.png'/> with rostral and caudal syrinx and a satellite lesion <img src='img/arrows/WC.png'/> enlarging the cervical cord. Note severe syringobulbia <img src='img/arrows/BS.png'/>.*
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**Ependymoma, Spinal Cord**
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*Sagittal T2 MR shows a complex cervical cord mass containing foci of susceptibility <img src='img/arrows/WS.png'/>, demonstrating prior hemorrhage within an ependymoma.*
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**Astrocytoma, Spinal Cord**
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*Sagittal T2 MR shows an infiltrative lesion expanding the midcervical cord. C3-C5 decompressive laminectomy has been performed.*
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**Astrocytoma, Spinal Cord**
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*Sagittal T1 C+ MR shows an astrocytoma enlarging the cervical cord <img src='img/arrows/WS.png'/> with patchy enhancement and infiltrative margins.*
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**Syringomyelia**
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*Sagittal T2 MR shows haustrated CSF signal mass <img src='img/arrows/WS.png'/> in the central cord in a patient with a Chiari 1 malformation. Hypointense foci <img src='img/arrows/WC.png'/> are due to flow artifact.*
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**Hemangioblastoma, Spinal Cord**
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*Sagittal T1 C+ MR shows holocord syrinx <img src='img/arrows/WO.png'/> and 2 small dorsal enhancing lesions <img src='img/arrows/WS.png'/> in this patient with multiple hemangioblastomas.*
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**Hemangioblastoma, Spinal Cord**
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*Sagittal T2 MR shows a nodular mass dorsally at the C4 level with a peripheral cyst <img src='img/arrows/WS.png'/>. Syrinx enlarges the cord. The ring of hypointensity <img src='img/arrows/BS.png'/> suggests sequelae of hemorrhage.*
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**Intramedullary Arteriovenous Malformation**
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*Sagittal T2 MR shows patchy hyperintensity within the cord <img src='img/arrows/BO.png'/> due to intramedullary AVM. Note dorsal flow voids due to tortuous, dilatated vascularity <img src='img/arrows/BS.png'/>.*
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**Metastases, Spinal Cord**
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*Sagittal T1 C+ FS MR shows focal intramedullary breast carcinoma metastasis enlarging the distal cord <img src='img/arrows/WS.png'/>. Note vertebral metastasis <img src='img/arrows/WO.png'/> with a mild compression fracture.*
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**Sarcoidosis**
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*Sagittal T1 C+ MR demonstrates extensive cord enlargement and intramedullary enhancement due to sarcoidosis.*
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**Cysticercosis**
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*Sagittal T2WI MR shows cystic thoracic cord mass in a patient with cysticercosis.*
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**Lipoma, Spinal**
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*Sagittal T1 MR shows a fusiform mass <img src='img/arrows/WO.png'/> contacting the dorsal pial surface of the cord. The mass was isointense to subcutaneous fat on all pulse sequences.*
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**Glioblastoma, Spinal Cord**
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*Sagittal T1 C+ MR shows a large enhancing mass <img src='img/arrows/BS.png'/> involving the upper thoracic cord with fusiform cord expansion.*
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