Wegener Granuloma in the Fourth Ventricle
Ansgar Berlisa,
Franziska Petschnerb,
Ingolf Carl Bötefürc and
Joachim Spreera
a Department of Neuroradiology, University of Freiburg, Freiburg, Germany
b Department of Rheumatology and Clinical Immunology, University of Freiburg, Freiburg, Germany
c Department of Neuropathology, University of Freiburg, Freiburg, Germany
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FIG 1. Initial contrast-enhanced T1-weighted MR images obtained in three planes and coronal view diffusion-weighted image (lower right) show inhomogeneous contrast enhancement of a mass at the floor of the fourth ventricle. Compression and infiltration of the left dorsolateral medulla oblongata and the vermis can be seen, with extrusion through the left foramen of Luschka. Concomitant hydrocephalus is revealed, and high signal intensity on the diffusion-weighted image implies dense cellularity of the tumor. Note the thickening of the mucosa with contrast enhancement at the paranasal sinuses, whereas CT scans revealed intact bony structures.
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FIG 2. Follow-up MR images obtained 4 weeks after initial imaging, during immunosuppressive therapy. Approximately 50% tumor regression is revealed. Remaining high signal intensity in the lesion can be seen on the diffusion-weighted image.
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FIG 3. Follow-up MR images obtained 6 weeks after initial imaging show slight additional regression of the tumor but evident reduction of the signal intensity on the diffusion-weighted image and remaining nonocclusive hydrocephalus.
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FIG 4. Image of the cerebellum and medulla oblongata obtained at nearly the same level as the transverse T1-weighted images shown in Figures 1 through 3. Note the poor demarcation of the granuloma (arrows) from surrounding brain tissue (1, foramen of Magendie).
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FIG 5. Histologic specimen (glial fibrillary acidic protein stained) with necrotizing mass (N) shows diffuse infiltration of the cerebellum (C).
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FIG 6. Histologic specimen (periodic acid-Schiff stain) shows typical Wegener granulomatosis patterns with necrotizing vasculitis (1), multinucleated giant cell (arrow), and necrotizing lesions (N). Calcifications (2) were detectable with surrounding scarred connective tissue.
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