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종결 Case No. 640 2022-11-01~2022-11-30
출제자 : 김슬기 Hit : 780
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Period 2022-11-01~2022-11-30
Diagnosis Polymorphous low-grade neuroepithelial tumor of the young (PLNTY)
Gender
  • M
  •  
  • F
Age 26
Clinical information 26세 여자
C.C>Seizure (두차례, 2 months ago, 1 day ago)
진단은?
Discussion 진단: Polymorphous low-grade neuroepithelial tumor of the young (PLNTY)
고찰
• Pediatric type, low grade diffuse glioma in 2021 WHO brain tumor classification
• First described in 2016
• Pathologic and genetic feature
 Oligodendroglioma-like cellular elements, calcification
 Strong CD34 immunopositivity
 Mutation of BRAF V600E gene and FGFR (fibroblast growth factor receptor) 2 and 3
 Abnormal regulation of the MAPK oncogenic pathway
• Radiologic feature
 Arises from cortex with temporal lobe predilection
 CT - hypodense mass with frequently calcification
 MRI -T2/FLAIR heterogeneous SI with salt and pepper (due to calcification and cyst), no diffusion restriction, slight or no enhancement
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Correct Answer

  • 김신영 양산부산대학교병원

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