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Polymorphous Low-Grade Neuroepithelial Tumor of the Young: A Case Report with Genomic Findings
- Source :
- World neurosurgery. 132
- Publication Year :
- 2019
-
Abstract
- Background Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a recently recognized epileptogenic neuroepithelial tumor. Despite its distinctiveness, its polymorphous histology and the nature of its oligodendrocyte-like cells remain unclear. Case Description A 30-year-old, right-handed man was diagnosed with intractable epilepsy since 22 years of age. Magnetic resonance imaging revealed T2 signal hyperintensity and corresponding T1 signal hypointensity within the subcortical white matter of the right middle temporal gyrus. Positron emission tomography scan demonstrated hypometabolism in the right anterior temporal region. Electroencephalography and stereo-electroencephalography monitoring localized seizures to the right temporal lobe, allowing the patient to undergo right temporal lobectomy. Histologic sections demonstrated cortical dysplasia, white matter heterotopia, and hippocampal reactive gliosis without neuronal loss. Interestingly, an approximately 6-mm subcortical neoplasm was identified in the temporal lobectomy. It was composed of well-differentiated oligodendroglial-like cells but exhibited mild-to-moderate nuclear variability and pleomorphism, and mild infiltration into the overlying cortex without perineuronal satellitosis. No mitotic activity, microvascular proliferation, or necrosis was identified, and Ki-67 labeling index was less than 1%. The tumor was diffusely CD34 positive with moderate glial fibrillary acidic protein and retained ATRX staining, and demonstrated the presence of the BRAF V600E mutation. The tumor was negative for reticulin condensation, synaptophysin, SMI31, neuronal nuclei immunostains, and both the IDH1 mutation and 1p19q codeletion. Overall histologic findings were most consistent with PLNTY. Conclusions The correct diagnosis of PLNTY and its distinction from closely resembling low-grade neuroepithelial tumors is important. We hope our proposed diagnostic features will aid in its proper diagnosis and management.
- Subjects :
- Adult
Male
Pathology
medicine.medical_specialty
Drug Resistant Epilepsy
White matter
Diagnosis, Differential
03 medical and health sciences
0302 clinical medicine
medicine
Biomarkers, Tumor
Humans
ATRX
biology
business.industry
Brain Neoplasms
Electroencephalography
Cortical dysplasia
medicine.disease
Anterior Temporal Lobectomy
Immunohistochemistry
Magnetic Resonance Imaging
Neoplasms, Neuroepithelial
Hyperintensity
Neuroepithelial cell
medicine.anatomical_structure
Heterotopia (medicine)
Pleomorphism (cytology)
030220 oncology & carcinogenesis
Positron-Emission Tomography
Synaptophysin
biology.protein
Surgery
Neurology (clinical)
business
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 18788769
- Volume :
- 132
- Database :
- OpenAIRE
- Journal :
- World neurosurgery
- Accession number :
- edsair.doi.dedup.....3468fe133e630e93c2bd7c529cfa39d8