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Myxopapillary ependymoma (hemorrhagic)

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

Increasing thigh and buttock pain and straight leg raise test positive.

Patient Data

Age: 20 years
Gender: Female

There is a well defined  intradural ovoid lesion that is centered within the distal lumbosacral canal. Lesion fills the canal space from the L5-S2 levels. The lesion is confined to the canal without extension into the adjacent exit foramen. The lesion measures 5.5 x 1.5 x 2.2 cm in vertical, AP and transverse dimensions respectively.  It is difficult to determine if the mass arises from a specific nerve root.  The lesion has heterogeneous signal characteristics. The lesion predominately low signal on T2 weighted images with areas of moderate signal within. The lesion is isointense to CSF on T1 weighted images and enhances fairly homogeneously post contrast. On STIR weighted imaging the lesion is predominantly moderate signal intensity with areas of low signal within which likely represent blood products.

Histology

MICROSCOPIC DESCRIPTION: Paraffin sections show fragments of a moderately hypercellular  tumor composed of cells with uniform round and oval nuclei with  finely granular chromatin and delicate processes. These are arranged  in diffuse sheets and perivascular pseudo-rosettes. No mitotic figures  are seen and there is no vascular endothelial cell hyperplasia and no  necrosis. Myxoid-mucinous material is noted in some of the pseudo-rosettes interposed between the central blood vessel and the  surrounding corona of tumor cells. Tumor cells show strong  immunostaining for GFAP. Weak staining for epithelial membrane antigen  (EMA) is noted in some pseudo-rosettes. The features are of  myxopapillary ependymoma. The Ki-67/MIB-1 labeling index is <1%.  4. The section shows a dense collagenous membrane covered on one  surface by tumor with features as described in specimens 1-3. No  nerve tissue is identified.   

FINAL DIAGNOSIS: Myxopapillary ependymoma.(WHO Grade I) 

Annotated image

The intradural mass ( * ) demonstrates mixed low and high T2 signal, with signal loss (blue arrows) best seen on STIR imaging suggesting blood products. The mass demonstrates enhancement. 

Case Discussion

This case illustrates slightly atypical, but still characteristic, appearances of a myxopapillary ependymoma with evidence of some intratumoral hemorrhage. 

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