Intraventricular neurocytoma: a clinical and pathological study of three cases and review of the literature

Abstract

Three patients with a recently described tumor of neuronal origin, intraventricular neurocytoma, are presented. These tumors occur as intraventricular lesions in young patients, and the prognosis after surgical treatment is favorable. The initial pathological diagnosis of intraventricular neurocytoma may be difficult because of the striking resemblance of these tumors to oligodendroglioma and, to a lesser extent, ependymoma on light microscopic examination. Despite the use of wide-ranging panels of monoclonal antibodies, previous authors have not found any characteristic immunohistochemical staining patterns, but in our three patients, the use of synaptophysin, glial fibrillary acidic protein, and Leu-7 demonstrated staining patterns that may be useful in the diagnosis of this tumor. The monoclonal antibody Ki-67 was used to stain one tumor and showed a low cell proliferation rate. We have reviewed the clinical, radiological, and pathological features of these 3 patients and 17 previously described patients in an attempt to determine the important diagnostic features of intraventricular neurocytoma. Intraventricular neurocytoma should be considered in any young patient with symptoms of raised intracranial pressure and radiological evidence of an intraventricular lesion. Pathological diagnosis requires the use of electron microscopy to show features of neuronal differentiation; however, immunohistochemical demonstration of a neuronal phenotype is also a useful adjunct to diagnosis. Failure to use specialized techniques for pathological diagnosis will lead to misdiagnosis of these lesions as oligodendrogliomas, as was the initial diagnosis in 2 of our patients before review.