Spinal ependymomas are the most common spinal cord tumour overall, seen both in adult and paediatric population.
This article specifically relates to spinal cord ependymomas. For a discussion of intracranial ependymomas and for a general discussion of the pathology refer to the main article: ependymoma. Additionally, due to different imaging features and demographics, myxopapillary ependymomas are also discussed separately.
Spinal ependymomas are the most common intramedullary neoplasm in adults, comprising 60% of all glial spinal cord tumours 7. They are the second most common intramedullary neoplasm in the paediatric population, representing 30% of paediatric intramedullary spinal neoplasms 6.
Peak incidence is in the fourth decade, with 39 years being the mean age at presentation. Males are more commonly affected than females.
There is an increased incidence with neurofibromatosis type 2.
Clinical presentation is similar to that of other intramedullary spinal tumours, with pain, weakness and sensory changes common.
The frequent presentation of sensory symptoms may be explained by the proximity of these centrally located tumours to the spinothalamic tracts. Dominant motor symptoms are commonly associated with very large ependymomas 5.
Ependymomas arise from ependymal cells lining the central canal or cell rests along the filum. Six histological subtypes are recognised:
- cellular (the most common intramedullary type)
- clear cell
- virtually always located along the filum terminale with occasional extension into the conus medullaris
- discussed separately: see myxopapillary ependymomas
- melanotic (the least common type)
On histologic evaluation, uniform moderately hyperchromatic nuclei are typically observed. Perivascular pseudorosettes are the classical finding.
Using the WHO classification, almost all intramedullary ependymomas may be classified as grade II or sometimes III (anaplastic). Malignant types are rare.
This is in contrast to myxopapillary ependymomas which are grade I lesions.
Ependymomas can occur anywhere along the spinal cord, however, the cervical cord is the most common site (44%). An additional 23% occur within the cervical cord and extend into the upper thoracic cord, and 26% occur in the thoracic cord alone 3.
Plain film features that may be seen with a spinal ependymoma include:
CT may demonstrate:
- non-specific canal widening
- iso to slightly hyper-attenuating compared with normal spinal cord
- intense enhancement with iodinated contrast
- large lesions may cause scalloping of the posterior vertebral bodies and neural exit foraminal enlargement
MRI is the modality of choice for evaluating suspected spinal cord tumours. Features include:
- widened spinal cord (as ependymomas arise from ependymal cells lining the central canal, they tend to occupy the central portion of the spinal cord and cause symmetric cord expansion)
- although unencapsulated, they are well-circumscribed
- tumoral cysts are present in 22%. Non-tumoral cysts are present in 62% 5
- syringohydromyelia occurs in 9-50% of cases
- in contrast to intracranial ependymomas, calcification is uncommon
- average length of four vertebral body segments 10
Typical signal characteristics:
- T1: most are isointense to hypointense; mixed signal lesions are seen if cyst formation, tumour necrosis or haemorrhage has occurred
- peritumoural oedema is seen in 60% of cases
- associated haemorrhage leads to the “cap sign” (a hypointense haemosiderin rim on T2 weighted images) in 20-33% of cases 5. The cap sign is suggestive of but not pathognomonic for ependymoma as it may also be seen in haemangioblastomas and paragangliomas
- T1 C+ (Gd): virtually all enhance strongly, somewhat inhomogeneously
Treatment and prognosis
Most ependymomas are slow-growing. They tend to compress adjacent spinal cord tissue rather than infiltrate it, almost always leaving a cleavage plane between tumour and spinal cord tissue.
A complete curative excision may be achieved in approximately 50% of cases. In those patients, the 5-year survival rate is approximately 85%. In patients who are not able to achieve complete resection, the 5-year survival rate is approximately 57%. Recurrence is rare following complete excision 6.
Although metastatic spread is rare, the most common sites for metastases include the retroperitoneum and lymph nodes.
- most common spinal cord tumour in children
- eccentric location in the spinal canal
- haemorrhage is uncommon
- patchy irregular contrast enhancement
- bone changes are infrequent
- involvement of the entire cord diameter and longer cord segments favours an astrocytoma (i.e., an intramedullary neoplasm involves the total spinal cord, it is more likely to be an astrocytoma 9 )
spinal cavernous malformation
- no enhancement
- complete hemosiderin ring
- 1. Brant WE, Helms CA. Fundamentals of Diagnostic Radiology. Lippincott Williams & Wilkins. (2007) ISBN:0781761352. Read it at Google Books - Find it at Amazon
- 2. Osborn AG. Diagnostic neuroradiology. Mosby Inc. (1994) ISBN:0801674867. Read it at Google Books - Find it at Amazon
- 3. Grossman RI, Yousem DM. Neuroradiology, the requisites. Mosby Inc. (2003) ISBN:032300508X. Read it at Google Books - Find it at Amazon
- 4. Davies SG, (BChir.) SG, Chapman S et-al. Aids to Radiological Differential Diagnosis. Saunders Limited. (2009) ISBN:0702029793. Read it at Google Books - Find it at Amazon
- 5. Koeller KK, Rosenblum RS, Morrison AL. Neoplasms of the spinal cord and filum terminale: radiologic-pathologic correlation. Radiographics. 20 (6): 1721-49. Radiographics (citation) - Pubmed citation
- 6. Smith AB, Soderlund KA, Rushing EJ et-al. Radiologic-pathologic correlation of pediatric and adolescent spinal neoplasms: Part 1, Intramedullary spinal neoplasms. AJR Am J Roentgenol. 2012;198 (1): 34-43. doi:10.2214/AJR.10.7311 - Pubmed citation
- 7. Dähnert WF. Radiology Review Manual. (2007) ISBN:0781766206. Read it at Google Books - Find it at Amazon
- 8. Kahan H, Sklar EM, Post MJ et-al. MR characteristics of histopathologic subtypes of spinal ependymoma. AJNR Am J Neuroradiol. 1996;17 (1): 143-50. AJNR Am J Neuroradiol (citation) - Pubmed citation
- 9. Kahan H, Sklar EM, Post MJ et-al. MR characteristics of histopathologic subtypes of spinal ependymoma. AJNR Am J Neuroradiol. 1996;17 (1): 143-50. AJNR Am J Neuroradiol (citation) - Pubmed citation
- 10. Patel U, Pinto RS, Miller DC et-al. MR of spinal cord ganglioglioma. AJNR Am J Neuroradiol. 1998;19 (5): 879-87. AJNR Am J Neuroradiol (citation) - Pubmed citation