Central nervous system neurocytoma and neuroblastoma in adults-report of eight cases

Abstract

The clinical features, pathologic findings and treatment courses of eight adults with central nervous system small-cell neuronal tumors were reviewed. Five patients had central neurocytomas, two patients central nervous system neuroblastomas, and one patient a neurocytoma-like spinal cord tumor. The neurocytomas were intraventricular, moderately cellular tumors with bland nuclei and perinuclear halos. Patients with neurocytoma were treated with surgery, radiation therapy, and/or chemotherapy, and have followed favorable clinical courses. The neuroblastomas were intraparenchymal, hypercellular tumors with necrosis and frequent mitoses. Patients with neuroblastomas were treated with surgery, radiation therapy and chemotherapy, with some clinical response, but overall poor survival. One of the two patients developed extracranial metastasis. The spinal cord tumor had histologic features of neurocytoma, and responded well to biopsy and radiation therapy. The cases are compared with the varieties of small-celled neuronal tumors described in the literature, and pathologic, histogenetic and treatment implications are discussed.

Central neurocytoma: A clinical, radiological and pathological study of nine cases

“…Extension into the third ventricle occurs in 26% of central neurocytomas. Isolated third or fourth ventricular occurrence and extraventricular sites are rarely reported [9][10][11][12][13][14]. There are also rare cases of central neurocytomas with craniospinal dissemination [15].…”

MR Appearance of Central Neurocytoma

“…Central neurocytoma exhibits immunoreactivity for neuron-specific enolase and synaptophysin, indicating neuronal differentiation. Ultrastructurally, the tumor contains synaptic vesicles, neuritic processes and neurosecretory granules (1,8,(12)(13)(14)18). The tumors are found almost exclusively in the anterior portion of the lateral ventricle, invariably producing an asymmetric hydrocephalus caused by obstruction of the foramen of Monro.…”

“…Common presenting symptoms and signs are those related to increased intracranial pressure including headache, nausea, vomiting, blurred vision, dizziness, memory loss, papillo-edema, and rarely paresis (1,13,14,16). Although 2 cases of malignant variants characterized by an increased mitotic activity, vascular endothelial proliferation, and tumor necrosis, and 3 cases of local tumor recurrences have been recently reported (25), most central neurocytomas have been regarded as benign lesions with a favorable prognosis (1.…”

“…Although 2 cases of malignant variants characterized by an increased mitotic activity, vascular endothelial proliferation, and tumor necrosis, and 3 cases of local tumor recurrences have been recently reported (25), most central neurocytomas have been regarded as benign lesions with a favorable prognosis (1. 6,13,14,17,25). In one study, 5 of 6 patients with central neurocytoma were disease-free 15 to 227 months after total or partial tumor resection (14).…”

“…Central neurocytoma is a recently described, rare primary brain tumor of neuronal origin, which is characterized by predominant occurrence in the young adults, exclusive lateral ventricular location, and oligodendroglioma-like histology (1,8,(12)(13)(14)17). Without ultrastructural and immunohistochemical studies, it is difficult to make the correct diagnosis of central neurocytoma, because light microscopic findings of the neurocytoma are quite similar to those of oligodendroglioma or ependymoma (1,8,12,14,18).…”