Multiple ependymomas

Case contributed by Ng Thanh Van Anh
Diagnosis almost certain

Presentation

2 months lower extremity‘s muscle atrophy and mild paralysis

Patient Data

Age: 30 years
Gender: Male
mri

MRI reveals multiple extra-axial lesions

Spinal canal

  • Intradural extramedullary mass displace spinal cord anteriorly
  • The tumor is hypointense in T1W, hyperintense in T2W, show strong and homogenous enhancement

Brain

  • 2 masses, one in 4th ventricle, one extra-axial abutting the cerebellar tentorium. Both are restricted diffusion with ADC values ~ 600  (x10-6 mm2/s), and show strong and homogenous post-contrast like the intradural extramedullary mass. 

Case Discussion

Multiple extra-axial lesions with atypical appearance. 

These intradural extramedullary masses do not show characteristic of common lesions such as meningioma (with ginkgo leaf sign or dural tail sign) or dumbbell sign of nerve sheath tumor. 

The preoperative diagnosis was of multiple lymphoma. 

The patient went on surgery to remove the intradural extramedullary mass. 

Histology revealed ependymoma grade III.

Discussion

Ependymoma is the most common intramedullary spinal cord tumor in adults (60%), but intradural extramedullary is an uncommon location and hard to make the right preoperative diagnosis. 

In this patient, two hypothesizes are considered: 

  1. Primary ependymoma with multiple lesion, involve extra-axial cerebral masses. 
  2. Cerebral ependymoma with drop metastases.

Both are uncommon and atypical, but the latter hypothesis is by far the most likely. 

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