Ependymoma

Steven H. Lin and Ori Shokek

 Background

In children and adults, what % of brain tumors are ependymomas?

1. 
   

   Children: 10%

   
   
2. 
   

   Adults: 5%

What is the median age of Dx for ependymomas?

Bimodal peak distribution, with peaks at 5 yrs and 35 yrs

What % of ependymomas arise intracranially, and how does this differ in children vs. adults? What are the most common locations?

1. 
   

   Children: 90% intracranial (10% cord). If intracranial, the posterior fossa is the most common site (60% infratentorial [floor of 4^th ventricle], 40% supratentorial [lat ventricle]).

   
   
2. 
   

   Adults: ~67% are supratentorial, and ~33% are infratentorial.

What is the cell of origin for ependymomas?

Ependymomas arise from the ependymal cells lining the ventricles.

What genetic syndrome is associated with spinal cord ependymoma?

Spinal cord ependymoma is associated with NF-2.

What % of ependymoma pts present with CSF seeding? What features predispose to seeding?

10%–15%; infratentorial location, high-grade tumors, and LF predispose to CSF seeding.

Which histologic subtypes of ependymoma are considered grades 1, 2, 3, and 4?

1. 
   

   Grade 1: myxopapillary and subependymoma

   
   
2. 
   

   Grade 2: classic ependymoma

   
   
3. 
   

   Grade 3: anaplastic

   
   
4. 
   

   Grade 4: ependymoblastoma

Where do grade 4 ependymomas generally arise?

Grade 4 ependymomas usually arise in the supratentorium.

What is the classical pathologic feature of ependymomas?

Perivascular pseudorosettes are a classical pathologic feature of ependymomas.

What defines malignant ependymomas on pathology?

Greater number of mitoses, cellular atypia, and more necrosis

Which type of ependymomas commonly arise in the conus/filum region of the spinal cord?

Myxopapillary ependymomas usually arise in the conus/filum region of the spinal cord.

What is the typical presentation of ependymomas?

Depends on location. If infratentorial: CN deficits, ↑ICP; if supratentorial: seizures, focal deficits

With what neurologic deficits are spinal cord ependymoma pts likely to present?

Sensory deficits (vs. cord astrocytomas, which present with pain/motor deficits)

 Workup/Staging

What is the workup for ependymoma?

Ependymoma workup: H&P, basic labs, CSF cytology/sampling, and MRI brain/spinal cord

When is LP contraindicated?

LP is contraindicated with a posterior fossa mass.

When should spinal MRI or CSF cytology be obtained after resection?

2 wks (14 days) postop to avoid postsurgical artifacts

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