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Long-term epilepsy-associated tumor in the amygdala of a 16-year-old boy: report of a rare case having intranuclear filaments

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Abstract

The term “long-term epilepsy-associated tumor (LEAT)” encompasses brain lesions associated with drug-resistant epilepsy over a long duration (≥2 years). Notably, some LEATs do not fit into any of the classifications of the World Health Organization (WHO). Herein, we report a LEAT that occurred in the left amygdala of a 16-year-old patient with intractable epilepsy. Histological examination of the resected amygdala revealed diffusely infiltrating tumor cells in the cortex. Perineuronal satellitosis and perivascular aggregation of tumor cells were apparent, along with mild nuclear enlargement and cytologic atypia. Tumor cells were positive for oligodendrocyte transcription factor 2 and neuronal markers including NeuN, neurofilaments, and synaptophysin, but were negative for CD34 and nestin. The most intriguing finding was intranuclear filaments, which appeared as rod- or needle-like shapes under high-power view. Ancillary ultrastructural analysis revealed thin filamentous intranuclear structures in tumor cells. Based on the glioneuronal nature of these cells as well as the infiltrative growth pattern, a diagnosis of LEAT was rendered that was deemed WHO grade I to II; however, the clinicopathological implications of the intranuclear inclusions remain unknown. The patient is currently alive and well without seizures.

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Authors and Affiliations

  1. Department of Pathology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea

    Yoon Jin Cha

  2. Department of Pediatric Neurosurgery, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, South Korea

    Dong-Seok Kim

  3. Department of Radiology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea

    Seung-Koo Lee

  4. Division of Pediatric Neurology, Department of Pediatrics, Epilepsy Research Institute, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, South Korea

    Hoon-Chul Kang

  5. Department of Pathology, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, South Korea

    Se Hoon Kim

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  1. Yoon Jin Cha
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  3. Seung-Koo Lee
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Correspondence to Se Hoon Kim.

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Cha, Y.J., Kim, DS., Lee, SK. et al. Long-term epilepsy-associated tumor in the amygdala of a 16-year-old boy: report of a rare case having intranuclear filaments. Brain Tumor Pathol 34, 172–178 (2017). https://doi.org/10.1007/s10014-017-0294-8

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  • DOI: https://doi.org/10.1007/s10014-017-0294-8

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