Hemangioblastoma - cervical cord

Case contributed by Assoc Prof Frank Gaillard

Presentation

Upper limb weakness increasing over a number of weeks with increased tone in legs.

Patient Data

Age: 35 years
Gender: Male

An enhancing nodule is present within the cord at the level of C5 with adjacent peritumoural cysts and extensive cord edema. Posterior to the lesion a number of serpentine flow voids are noted. The mass has evidence of blood products (previous hemorrhage) and vivid enhancement. 

Annotated image

A central vividly enhancing mass (green arrows) is surrounded by cystic elements and has a prominent serpiginous flow void posteriorly (red arrow). The entire cord is edematous, and the peritumoural cists are lined by hemosiderin (blue arrow)

 MICROSCOPIC DESCRIPTION:   Paraffin sections show a moderately hypercellular, intensely  vascular tumor.  This is composed of cells with moderately  pleomorphic round oval and angulated hyperchromatic nuclei and a  variable amount of pale and foamy cytoplasm.  These are arranged in  diffuse sheets as well as lobules which are enclosed by thin-walled  capillaries.  Vascular spaces vary from large caliber thin-walled  sinusoidal structures to capillaries.  A very occasional mitotic  figure is identified in tumor cells.  No areas of necrosis are seen.   Tumor is attached to dense collagenous tissue consistent with  meninges.

Immunohistochemistry shows strong staining of vascular  endothelium for CD34. No reactivity for GFAP, CD34 or cytokeratrin  AE1/AE3 is seen in tumor cells. The features are of capillary hemangioblastoma.   

FINAL DIAGNOSIS: Capillary hemangioblastoma.

Case Discussion

This patient went on to have screening of the rest of the neuraxis and of the kidneys and there was no sign of other lesions. Screening for vHL was negative. 

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