Rare-20. pediatric tectal plate gliomas with pilomyxoid histologic features. report of three cases

Pediatric tectal plate gliomas (TPG) are generally considered indolent lesions that uncommonly progress. The majority of these patients require cerebrospinal fluid (CSF) diversion via ventriculostomy or shunting, but oftentimes no tissue is retrieved for pathologic diagnosis. The few reports of TPG histology describe mainly low grade astrocytomas, with few reports of ependymoma, malignant glioma, or pilocytic astrocytoma. We have encountered three pediatric TPGs with pilomyxoid histologic features. This cohort includes two females (ages 3.5 and 7 years) and one male (age 3.8 years). All of these children presented with symptoms indicating increased intracranial pressure. Magnetic resonance imagining (MRI) studies confirmed hydrocephalus, and though the dimensions of their individual tectal tumors were variable, all were T1 hypointense, T2 hyperintense, and showed patchy enhancement post contrast administration. A separate fourth ventricular lesion was detected in the male; it had similar MRI signal characteristics to his tectal tumor. All three patients underwent CSF diverting procedures. Both females additionally underwent tectal tumor biopsies, while the male underwent subtotal resection of the fourth ventricular lesion. The entirety of tissue examined from the youngest patient’s tumor consisted of pilomyxoid astrocytoma, typified by a monomorphous population of bland glial cells with long delicate processes, perivascular rosetting, and myxoid backgroun...
Source: Neuro-Oncology - Category: Cancer & Oncology Authors: Tags: RARE TUMORS Source Type: research