Abstract
BackgroundTumors that arise in the temporal lobes of infants and spread to the neural system are limited to several diagnoses. Herein, we present an infantile case of a temporal tumor showing neuronal and glial differentiation.Case presentationThe patient was a 9-month-old boy with low body weight due to intrauterine growth retardation. At 9 months after birth, he presented partial seizures. Computed tomography scanning revealed a mass (35 * 40 mm) in the left temporal lobe. Isointensity was noted on magnetic resonance T1-weighted images and fluid attenuation inversion recovery images. The tumor was heterogeneously enhanced with gadolinium. Positron emission tomography showed high methionine uptake in the tumor. During surgery, the tumor, which was elastic and soft and bled easily, was gross totally resected. A moderately clear boundary was noted between the tumor and normal brain parenchyma. Histologically, the tumor mainly comprised a ganglioglioma-like portion and short spindle cells at different densities. The former was immunohistochemically positive for some kinds of neuronal markers including synaptophysin. The spindle cells were positive for glial fibrillary acidic protein, but desmoplasia was not observed.DiscussionThe tumor contained both neuronal and glial elements; the former were the main constituents of the tumor and included several ganglion-like cells. Because neuronal elements gradually transited to glial cells, a mixed neuronal-glial tumor was diagnosed.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2045126100982604
Highlights
Tumors that arise in the temporal lobes of infants and spread to the neural system are limited to several diagnoses
Tumors that arise in the temporal lobes of infants and spread to the neural system include desmoplastic infantile ganglioglioma (DIG), pleomorphic xanthoastrocytoma (PXA), neuroblastoma (NB), and extraventricular neurocytoma (EVN)
Case presentation The patient was a 9-month-old boy who was born at 36 weeks 5 days of gestation with low body weight (2170 g) due to intrauterine growth retardation
Summary
The tumor in the present case was considered to have both neuronal and glial elements, according to the results of immunohistochemical and EM analyses. PXA was a possible diagnosis in the present case These tumors occur most often in the temporal lobes of children or young adults [11,12,13,14], and associated seizures occur in up to 78% of cases [13]. Higher cellularity, dominant neuronal differentiation, and fewer pleomorphic findings of multinucleated giant cells noted in this case were different from the common type of PXA. For these reasons, we disregarded the diagnosis of PXA. We previously reported a case of a 26-year-old woman who presented with atypical EVN, in which glial and neuronal differentiation was demonstrated by immunohistochemical analysis [26].
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