Contributed by Malgorzata Kolodziej1, Ludwig Benes1, Christopher Nimsky1, Hans-Eckart Schaefer3, Axel Pagenstecher2
1Departments of Neurosurgery and 2Neuropathology, University Hospital of Marburg
3Department of Pathology, University Hospital of Freiburg, Germany
CLINICAL HISTORY AND IMAGING STUDY
A 54-year-old woman with no significant medical history complained about nausea and vomiting. Her family reported personality changes over the past few weeks. Neurological investigation revealed paresis of the abducens and hypoglossal nerves on the left side, a right sided reduction of the visual field, as well as dysarthria and ataxia. Cranial MRI demonstrated an extensive circular meningeal lesion (2 cm x 3 cm) of the foramen magnum with contrast enhancement (Figures 1 and 2, arrows). The process compressed the medulla oblongata and ensheathed the spinal cord. Hydrocephalus of all ventricles was noted. The thoracic X-ray and a thyroid scintigraphy were normal. Ophthalmologic investigation revealed an edema of the left papilla. Serological tests for p- and c-ANCA, ANA, DNA, ENA and tumor markers were negative. The CSF showed signs of a chronic lymphocytic meningitis and was negative for Borrelia or mycobacteria. Because of circular growth, the tumor was resected only partially.
Neuropathological examination revealed fibrous tissue (dura mater) with cellular infiltrates (Figure 3) of plasma cells (Figure 4, VS38c), granulocytes (Figure 5, chloracetate esterase) and lymphocytes (Figure 6, LCA). Collections consisting of histiocytes and to a lesser degree of epithelioid cells and multinuclear giant cells were abundant (Figure 7, H&E, 8, CD68). Focal necroses in areas with abundant neutrophil granulocytes indicated immune complex reactions (Figure 5 and 9). Obliterated arterial and venous vessels with inflammatory alterations were scattered throughout the lesion. Several inflamed vessels showed disruption of the walls (Figure 10, EvG). Auramine rhodamine staining was negative and there were no other bacteria or fungi detected. Microbiological examination including PCR for bacteria and fungi, respectively, revealed no pathological microorganisms.