Sunday 8 September 2013

Spinal Ependymoma

Ependymoma is the commonest primary intramedullary spinal cord tumor, seen both in adults and children. The common locations are cervico-thoracic  and conus.
The most common age group involved is middle adult years. Men and women are equally affected.

 Ependymomas arise from ependymal cells lining the central canal or cell rests along the filum.

6 histological subtypes are recognised:
  1. cellular (the most common intramedullary type)
  2. papillary
  3. clear cell
  4. tanycytic ( fibrillar)
  5. myxopapillary
    •  located along the filum terminale with occasional extension into the conus medullaris
  6. melanotic (the least common type)
On histologic evaluation, uniform moderately hyperchromatic nuceli are typically observed. Perivascular pseudorosettes are the classical finding. Almost all are histologically benign. Although unencaspulated, these glial tumors are usually well circumscribed and do not infiltrate the surrounding spinal cord.
The conus ependymoa is usually myxopapillary type whch is WHO grade 1 and benign and is common in pediatric age group.
Filum terminale ependymoma may become enormous within the capacious thecal sac before they are diagnosed. These uncapsulated tumor can insinuate among the nerve roots and within the arachnoid sheaths of the cauda equine. CSF dissemination is common.
Investigation
Gadolinium enhanced MRI of the spine is the investigation of choice.
T1 weighted image shows expansion of the cord on sagittal image as ependymomas  are usually isointense or hypointense to the surrounding spinal cord which may show symmetrical expansion of the cord. So contrast enhancement will demonstrate the tumor.
Treatment
Surgical resection of the tumor is the treatment of choice.
Per operative frozen section biopsy may demonstrate ependymal rosettes and can differentiate these tumors from astrocytomas.
Surgeon should look for the anatomical plane and try to achieve total or subtotal resection.Post operative radiotherapy is delayed if piecemeal total or nearly total removal has been accomplished.In such cases, tumor recurrences can be treated with repeat surgery followed by radiotherapy.


Sources:
YoumansNeurological Surgery , 6th Ed. Elsevier saunders. .Richard Winn. Chapter 309: Spinal cord tumors in adults. Alfred T. Ogden, Theodore H.Schwartz, Paul C. Mc Cormick

Radiopaedia.org/article/spinal-ependymoma Dr.Andrew Dixon and Dr. Sara Wein et al

 

1 comment:

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