Primary leptomeningeal gliomatosis is rare, and the diffuse form is even rarer with only three cases reported in the literature. A fourth case is described in this report. Computerized tomography (CT) findings showed hydrocephalus with enhancement of the cerebral cisterns, and analysis of cerebrospinal fluid obtained by lumbar puncture showed many atypical cells. Based on these findings, a diagnosis of leptomeningeal tumor was made. There was some improvement in neurological and CT findings following radiotherapy and chemotherapy.
We report here a case of peripheral neuroepithelioma arising in the uterine cervix. A 44-year-old female had complained of irregular genital bleeding for several months and was diagnosed as extraosseous Ewing's sarcoma (EOE) from biopsy specimens initially. The tumor cells, which were intensely stained by periodic acid-Schiff (PAS) and digested with diastase, were uniformly small and round, had hyperchromatic nuclei and scant, indistinct cytoplasm. The surgically-removed tumor cells were immunohistochemically stained with antisera against neuro-specific enolase (NSE), tyrosine hydroxylase. In ultrastructural study, neurosecretory granules were observed. Finally we diagnosed this case as peripheral neuroepithelioma (peripheral primitive neuroectodermal tumor, PNET).
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