Contributed by Anne Shepler, MD and Julia Kofler, MD
Contributed by Anne Shepler, MD and Julia Kofler, MD
CLINICAL HISTORY AND NEUROIMAGING
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The patient is a preadolescent female who presented with nausea, vomiting, blurred vision, diplopia, gait instability and ataxia. An MRI showed a 22 x 21 x 78 mm enhancing tumor with a necrotic center located at the craniocervical junction expanding the medulla oblongata and the proximal aspect of the spinal cord to the level of T3. Additionally, there was a non-enhancing exophytic component of the tumor abutting the left premedullary cistern (Figure 1). The radiologic impression was an infiltrative, likely astrocytic, craniocervical junction tumor with an exophytic component.
INTRAOPERATIVE FINDINGS
The smear preparation showed proteinaceous debris and neutrophils which was interpreted as necrotic tissue.
MICROSCOPIC FINDINGS
Permanent sections showed membranous fibrovascular tissue lined by columnar to pseudostratified and partially ciliated epithelium. Proteinaceous mucinous material was also present with numerous admixed neutrophils. No acute inflammation was seen in the cyst wall.
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WHOLE SLIDE H&E IMAGE:
http://image.upmc.edu:8080/NeuroPathology/Malformations/Malformations4/MAL.37a.svs/view.apml?
IMMUNOHISTOCHEMICAL STAINS
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The epithelial lining was positive for AE1/AE3 (Figure 4) and EMA (Figure 5) and was negative for GFAP (Figure 6). No neuroglial tissue was identified on GFAP, synaptophysin, and Olig2 immunostains. A Ki-67 proliferation index labeled a few inflammatory and epithelial cells. Gram and Grocott stains were negative for bacterial and fungal organisms.
