| Yayang Chen,Bin Li,Boning Luo,Xiaoying Tian,Zhi Li. Non-specific histological variant of dysembryoplastic neuroepithelial tumor: a diagnostic challenge. Oncol Transl Med, 2016, 2: 104-109. |
| 胚胎发育不良性神经上皮肿瘤之非特殊性亚型：组织学诊断的挑战 |
| Non-specific histological variant of dysembryoplastic neuroepithelial tumor: a diagnostic challenge |
| Received:December 16, 2015 Revised:June 05, 2016 |
| DOI：10.1007/s10330-015-0126-4 |
| 中文关键词: 脑肿瘤；胚胎发育不良性神经上皮肿瘤；皮层发育不良；鉴别诊断 |
| 英文关键词: brain tumor; dysembryoplastic neuroepithelial tumor; cortical dysplasia; differential diagnosis |
| 基金项目: |
| Author Name | Affiliation | E-mail | | Yayang Chen | Department of Pathology, The First Affiliated Hospital, Guangzhou Medical University, Guangzhou 510120, China | lizhi@mail.sysu.edu.cn | | Bin Li | Department of Pathology, The First Affiliated Hospital, Guangzhou Medical University, Guangzhou 510120, China | lizhi@mail.sysu.edu.cn | | Boning Luo | Sun Yat-sen University, Guangzhou 510080, China | lizhi@mail.sysu.edu.cn | | Xiaoying Tian | ptist University. Kowloon Tong, Hong Kong, China | lizhi@mail.sysu.edu.cn | | Zhi Li* | Department of Pathology, The First Affiliated Hospital, Guangzhou Medical University, Guangzhou 510120, China | lizhi@mail.sysu.edu.cn |
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| 中文摘要: |
| 目的：精确诊断胚胎发育不良性神经上皮肿瘤（DNT）的非特殊性亚型往往非常困难，因此亚型缺乏“特征性胶质神经元成分”的组织学标志。本文对2例罕见的非特殊性亚型病变的临床表现、影像学特征和组织学表现加以分析，以期提高临床对此少见亚型的鉴别诊断能力。方法：16岁和23岁患者各一名，均有数年难治性癫痫病史，磁共振检查显示两位患者大脑皮层内均可见境界清楚的囊性病灶，无明显瘤周水肿，增强后病灶强化不明显。病灶均完整切除，行组织学检查和免疫组织化学染色及基因检测分析。结果：两处病变的组织学结构相似，均可见囊状结构和附壁的瘤结节。其中胶质结节主要由少突胶质细胞样成份构成，部分区域形似毛细胞星形细胞瘤样。病灶邻近的脑皮层可见局灶皮层发育不良，I型。免疫组织化学染色显示少突胶质细胞样成份突触素（Syn）和Olig-2弥漫阳性，但CD34、P53和IDH1 R132H均阴性。增殖指数（Ki-67）较低，约为1%。原位荧光杂交检测（FISH）显示两处病灶均未见染色体1p/19q共缺失。两位患者在术后均未给予辅助治疗，随访24个月，未见肿瘤复发且再无癫痫发作。结论：DNT的非特殊性亚型的组织学诊断是对神经病理工作者的挑战，必须考虑与其它相似的低级别胶质瘤相鉴别。仔细全面地观察其影像学和组织学特征，结合患者的临床表现方能得出正确的诊断。 |
| 英文摘要: |
| Objective The accurate diagnosis of the non-specific variant of dysembryoplastic neuroepithelial tumor
(DNT) is very difficult because it is characterized by absence of the histological hallmark of the “specific
glioneuronal element” in lesions. We herein present two cases of the non-specific form of DNT to analyze
the clinical, radiological, and histological features of this unusual subtype of DNT.
Methods A 16-year-old and a 23-year-old patient had been treated for pharmacoresistant epilepsy for
several years before undergoing referral to the hospital for further examination and treatment. Magnetic
resonance imaging (MRI) revealed that both patients had a small, well-demarcated cystic lesion within the
cortex of the brain without obvious contrast enhancement or peritumoral edema. The lesions were totally
resected and routinely examined using histological and immunohistochemical analysis.
Results Both lesions exhibited similar histological appearances with cyst formation and mural nodule
architecture. The glial nodules were mainly composed of oligodendrocyte-like components, and partly of
piloid cells resembling pilocytic astrocytoma. The cortex adjacent to the lesion in both cases was found to
have the histological features of focal cortical dysplasia (FCD) Type I. Immunohistochemically, the oligodendrocyte-like components were diffusely positive for Syn and Olig-2, but staining for CD34, p53, and IDH1
R132H was negative. The Ki-67 (MIB-1) labeling index was low, approximately 1%. There was no 1p/19q
co-deletion in either lesion by fluorescence in situ hybridization (FISH) assay. Neither patient received
postoperative adjuvant treatment, and both underwent regular follow-up for at least 24 months. No signs of
recurrence or epileptic attacks were observed during the follow-up period.
Conclusion The non-specific variant of DNT is a diagnostic challenge for pathologists in clinical practice,
and differentiation from some low-grade gliomas needs to be considered. The careful inspection of radiologic and histopathologic findings, accompanied by analysis of patients’ clinical manifestations, may be
helpful in making an accurate diagnosis. |
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