多形性黄色星形细胞瘤色素沉着变体——一种罕见的与长期癫痫相关的肿瘤。

Pigmented variant of pleomorphic xanthoastrocytoma - A rare long-term epilepsy associated neoplasm.

作者信息

Poyuran Rajalakshmi, Moudgil Nirupam, Arimappamagan Arivazhagan, Bharath Rose D, Mahadevan Anita

机构信息

Department of Neuropathology, NIMHANS, Bengaluru, Karnataka, India.

Department of Neurosurgery, NIMHANS, Bengaluru, Karnataka, India.

出版信息

Indian J Pathol Microbiol. 2019 Jul-Sep;62(3):445-447. doi: 10.4103/IJPM.IJPM_723_18.

DOI:10.4103/IJPM.IJPM_723_18

PMID:31361236

Abstract

Pleomorphic xanthoastrocytoma (PXA) is an uncommon, long-term epilepsy associated tumor of young adults. Its pigmented variant is exceedingly rare, with only five previously reported cases on record. We report the sixth case of pigmented PXA in a 24-year-old lady presenting with long-standing seizures. The MRI revealed a solid cystic lesion located in the right medial temporal lobe. Histopathologically, the superficially located tumor showed typical features of PXA with melanin-laden astrocytic component and was negative for V600E-mutant BRAF. The histogenesis is discussed.

摘要

多形性黄色星形细胞瘤（PXA）是一种罕见的、与年轻人长期癫痫相关的肿瘤。其色素沉着变体极为罕见，此前仅有5例报告病例。我们报告了第六例色素沉着型PXA，患者为一名24岁女性，有长期癫痫发作史。MRI显示右侧颞叶内侧有一个实性囊性病变。组织病理学检查显示，位于浅表的肿瘤具有PXA的典型特征，含有黑色素的星形细胞成分，V600E突变型BRAF检测为阴性。本文还讨论了其组织发生。