Case 666 -- A 3-year-old girl with altered mental status, gait difficulty, and vomiting

Contributed by Ilya Lim, MD1, Irina Mikolaenko, MD2, Mark Cohen, MD3
    1 Department of Internal Medicine, NYU Langone Medical Center, New York, NY
    2 Department of Neuropathology, NYU Langone Medical Center, New York, NY
    3 Department of Neuropathology, Case Western Reserve University, Cleveland, OH


A 3-year-old girl with a history of mild motor delay, presented with a change in mental status, gait difficulty, nausea and vomiting. The patient was in her usual state of health until 9 months prior to presentation, when she was found to have abnormal adduction of the left eye. Over the next several months, she developed gait difficulties and behavioral changes. On the day of admission, she developed confusion, severe gait difficulties, nausea and vomiting. On physical examination, she had right homonymous hemianopsia, right hemiparesis, and increased deep tendon reflexes of her right Achilles tendon. She subsequently became unresponsive. She was intubated and given mannitol and steroids. An external ventricular drain was placed. Neuroimaging showed a minimally heterogeneous enhancing tumor measuring 8 x 8 x 9 cm involving the left parietal region and, partly filling the left lateral ventricle, with accompanying hydrocephalus, midline shift and uncal herniation (Figures 1, 2, 3). Gross total resection of the tumor was confirmed by post-operative imaging. Staging evaluation revealed no metastasis. The patient was discharged one week later.


Grossly, tumor submitted for neuropathological evaluation consisted of multiple pieces of soft, tan and red tissue ranging in size from 2.0 x 2.0 x 2.0 cm in greatest dimension.

Microscopically (Figures 4, 5, 6, 7, 8), the tumor was comprised of clusters and cords of small to medium sized hyperchromatic cells with round to oval nuclei and indistinct cell borders set in a neuropil-like matrix. Rosettes with well-formed central lumina, some with central granular material were seen (Figure 8). Densely cellular regions with extensive apoptosis were also present. MIB-1 proliferation index was high, focally approaching 70% (Figure 9). The neuropil-like areas were labeled with synaptophysin antibodies (Figure 10). True rosettes were highlighted by vimentin immunostaining (Figure 11), but were negative for GFAP (Figure 12) and neurofilament protein immunostains.


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