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Brain Tumor Pathology

, Volume 35, Issue 4, pp 209–216 | Cite as

Anaplastic changes of diffuse leptomeningeal glioneuronal tumor with polar spongioblastoma pattern

  • Tomohiro Yamasaki
  • Naoto Sakai
  • Kazuya Shinmura
  • Hiroshi Kawaji
  • Shinichiro Koizumi
  • Tetsuro Samashima
  • Hiroki Namba
Case Report

Abstract

Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare glioneuronal neoplasm with oligodendroglioma-like cells confined in the subarachnoid spaces. A great majority of DLGNT are histologically low grade. However, some tumors show features of anaplasia with increased mitotic and proliferative activity. Due to the limited number of patients and inadequate clinical follow-up reported to date, the WHO classification does not yet assign a distinct WHO grade to this entity. Polar spongioblastoma pattern, in which bipolar cells are arranged in parallel with palisading nuclei, remains poorly understood about the pathological process of forming this pattern. We experienced a case of 22-year-old man developing DLGNT with extensively distributed anaplastic changes involving polar spongioblastoma pattern and the secondary tumor invasion to brain parenchyma in 4½ years before the autopsy. Clinical and pathological courses of the patient are presented with radiological, histopathological, and genetic examinations. This is the first report demonstrating the immunohistological and genetic evaluation of a DLGNT with polar spongioblastoma pattern.

Keywords

Diffuse leptomeningeal glioneuronal tumor Polar spongioblastoma pattern Anaplastic change Autopsy 

Notes

Acknowledgements

The authors thank Drs Hideaki Yokoo and Junko Hirato (Japan Brain Tumor Reference Center) for their analysis of KIAA1549-BRAF fusion by FISH.

Compliance with ethical standards

Conflict of interest

The authors report no conflict of interest concerning the findings specified in this paper.

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Copyright information

© The Japan Society of Brain Tumor Pathology 2018

Authors and Affiliations

  1. 1.Department of NeurosurgeryHamamatsu University School of MedicineHamamatsuJapan
  2. 2.Department of Tumor PathologyHamamatsu University School of MedicineHamamatsuJapan

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