Primary leptomeningeal meningiomatosis with widespread whorl formation

We report a 10-year-old girl with a primary leptomeningeal tumor. She presented with a 5-week history of increased intracranial pressure, progressive cranial nerve deficits, and spinal compression signs. She had previously had a granulosa cell tumor, a benign estrogen-producing ovarian tumor, which was resected 6 months before the initial neurological symptoms developed. At autopsy, the brain and spinal cord showed diffuse neoplastic involvement of the leptomeninges. The tumor was composed of small cells with a high nucleus/cytoplasm ratio, which were immunoreactive for vimentin but not for epithelial membrane antigen or cytokeratin. In addition, the tumor contained many small cellular whorls with desmosome-like junctional complexes between the cells, suggesting that the tumor was a meningioma, basically of the meningothelial type. The term "meningeal meningiomatosis" has been used synonymously with "primary meningeal sarcomatosis." The present tumor was considered to be a rare example of "meningeal meningiomatosis" of true meningothelial cell origin.