Central neurocytoma: morphological, flow cytometric, polymerase chain reaction, fluorescence in situ hybridization, and karyotypic analyses

Case report

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✓ The results of cytogenetic and molecular genetic analysis of a central neurocytoma are presented. Central neurocytomas are intriguing neoplasms that exhibit primarily neuronal, but also glial characteristics, which indicate an origin from a pluripotential neuroglial precursor. The authors describe an intraventricular neurocytoma in an 11-year-old boy that showed anaplastic features with widespread necrosis and mitoses, as well as extensive calcification and foci that exhibited marked neuronal differentiation with clusters of ganglion cells. Immunohistochemical examination showed prominent synaptophysin and neurofilament positivity and focal glial fibrillary acidic protein positivity. Electron microscopy revealed abundant neuritic processes with microtubules and dense core granules as well as mature ganglion cells. Flow cytometry studies revealed increased S (7.8%) and G2M (9.7%) phase components. Molecular and cytogenetic studies were undertaken to assess whether there were similarities to two other tumor types that exhibit neuronal differentiation, the neuroblastoma and medulloblastoma. Polymerase chain reaction and fluorescence in situ hybridization (FISH) analysis revealed no evidence of amplification of the MYCN oncogene or chromosome 1p deletion, which are common in neuroblastomas. Chromosomal analysis by G banding revealed a complex karyotype, with counts in the near-diploidy range (45–48). Two chromosomes 1 appeared normal on G banding and FISH analysis, with p58 signals present on the distal p arm of both chromosomes 1; however, three additional copies of distal 1q were present in rearrangements with 4 and 7. Although the histological findings indicate a kinship to the neuroblastoma and medulloblastoma, the central neurocytoma appears to have a different karyotypic profile, although more cases need to be assessed using molecular genetic analysis.

Article Information

Address reprint requests to: Venita Jay, M.D., Division of Pathology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada. email: vjay@sickkids.on.ca.

© AANS, except where prohibited by US copyright law.

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Figures

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    Left: Axial unenhanced CT scan revealing evidence of a previous right-sided craniotomy for epidural hematoma. In addition, a focus of calcification is noted in the high right parietal area (arrow). Interestingly, it was from this site that 1 year later the patient was noted to have developed a large intraaxial tumor. Center: Contrast-enhanced axial CT scan obtained 1 year after removal of the right parietal epidural hematoma, revealing a large tumor that is predominantly intraventricular in location. The tumor enhances irregularly with contrast administration. There is shift of midline structures from right to left, and the ventricles are moderately dilated. Right: Gadolinium-enhanced axial magnetic resonance image demonstrating irregularly enhancing tumor mass filling the right lateral ventricle, with shift of the septum pellucidum across the midline.

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    Photomicrographs of tumor specimens. Original magnifications × 225. Upper: Tumor cells exhibit clear perinuclear haloes (arrowheads). There is individual cell tumor necrosis with karyorrhectic nuclear debris (arrow). H & E. Center: Focus of prominent neuronal differentiation with mature neuropil-like background and a binucleated ganglion cell (arrowhead). There is also extensive calcification (arrows). H & E. Lower: The tumor exhibits a high MIB-1 labeling index on immunohistochemical studies. Immunoperoxidase stain.

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    Chromosomal analysis showing representative G-banded karyotype from the tumor: the derivative chromosomes 4, 7, 16, and 18 are placed to the right of the normal homologs; the derivative chromosome 8 is placed to the right of the two normal homologs of chromosome 8. The origin of mar 1 and mar 2 were not determined. There is a random loss of chromosome 2 in this metaphase spread.

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