Infiltrative brainstem and cerebellar neurocytoma

Case report

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Neurocytomas are typically intraventricular in location, and extraventricular neurocytomas are uncommon. The authors report the unique case of a 15-year-old girl who was found to have a low-grade neurocytoma infiltrating the brainstem and cerebellum and spreading along the CSF pathways to the lateral and third ventricles. The patient underwent endoscopic third ventriculostomy to treat associated hydrocephalus, and biopsy specimens from intraventricular tumor nodules were obtained. Because of the low-grade pathology, the fact that the lesion was not amenable to resection, and the extensive radiation field required for radiation therapy, she has been treated conservatively with close follow-up. Over the course of almost 4 years since diagnosis, no additional treatment has been required. Neurocytoma with widespread infiltration of the brainstem and cerebellum has not been previously reported.

Abbreviation used in this paper:ETV = endoscopic third ventriculostomy.

Article Information

Address correspondence to: David I. Sandberg, M.D., University of Texas at Houston, 6431 Fannin Street, Suite 6.264, Houston, Texas 77030. email: David.I.Sandberg@uth.tmc.edu.

Please include this information when citing this paper: published online August 31, 2012; DOI: 10.3171/2012.8.PEDS08286.

© AANS, except where prohibited by US copyright law.

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Figures

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    Noncontrast CT scan demonstrating infiltrative brainstem and cerebellar lesion with obstructive hydrocephalus.

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    A: Axial FLAIR MRI scan demonstrating widespread brainstem and cerebellar involvement. B: Axial FLAIR MRI scan revealing intraventricular tumor nodules. C: Sagittal Gd-enhanced T1-weighted MRI scan demonstrating nonenhancing tumor in the brainstem and cerebellum, as well as tumor nodules involving the floor of the third ventricle.

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    Intraoperative photograph from the endoscopic procedure demonstrating multiple tumor nodules involving the floor of the third ventricle.

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    Photomicrographs. A: Smear preparation demonstrating monomorphous population of cells with round nuclei and small nucleoli. B: H & E section demonstrating oligodendrocyte-like clear cells in a delicate neuropil-like matrix. C: CD56-stained specimen exhibiting diffuse and strong synaptophysin expression within the tumor. Original magnification × 200.

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    Left: Coronal Gd-enhanced T1-weighted MRI scan obtained 21 months postoperatively demonstrating small focus (0.3 cm in maximal diameter) of enhancement in the superior vermis. Right: Coronal Gd-enhanced T1-weighted MRI obtained 27 months postoperatively demonstrating increased size of focus of enhancement in the vermis, which now measures 0.8 cm in maximal diameter. Three MRI scans during the subsequent 18 months have demonstrated no changes to this focus of enhancement and no change in overall tumor burden.

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