CAJ | 학술논문

目的：探讨脑室外神经细胞瘤的临床病理学特征。方法回顾性分析12例脑室外神经细胞瘤的临床、病理学特征,并复习相关文献。结果肿瘤位置：大脑半球5例,右丘脑2例,小脑蚓部2例,鞍区2例,脊髓1例。瘤组织由大小较一致的圆形细胞构成,核圆形或卵圆形,核周空晕常见,可见神经毡样结构、神经节细胞或神经节样细胞分化、血管周围假菊形团、钙化、微血管壁增厚及透明变性等病理改变。免疫表型：Syn弥漫强阳性,7例瘤组织Ki-67增殖指数≤2%,5例Ki-67增殖指数为3%~8%。3例行染色体1p/19q荧光原位杂交检测显示1p和19q均不缺失。随访4个月~4年,1例术后2年肿瘤复发并播散。结论脑室外神经细胞瘤与中枢神经细胞瘤组织学表现虽相似,但其更复杂,多见神经节细胞或神经节样细胞分化,钙化、血管壁增厚及透明变性常见,神经毡样结构少见。需与少枝胶质细胞瘤、室管膜瘤、胚胎发育不良性神经上皮肿瘤等鉴别,免疫组化标记及染色体1p/19q缺失检测有助于鉴别诊断。治疗以手术全切除为主,对于次全切除、非典型及复发病例术后应辅以放疗。
목적：탐토뇌실외신경세포류적림상병이학특정。방법회고성분석12례뇌실외신경세포류적림상、병이학특정,병복습상관문헌。결과종류위치：대뇌반구5례,우구뇌2례,소뇌인부2례,안구2례,척수1례。류조직유대소교일치적원형세포구성,핵원형혹란원형,핵주공훈상견,가견신경전양결구、신경절세포혹신경절양세포분화、혈관주위가국형단、개화、미혈관벽증후급투명변성등병리개변。면역표형：Syn미만강양성,7례류조직Ki-67증식지수≤2%,5례Ki-67증식지수위3%~8%。3례행염색체1p/19q형광원위잡교검측현시1p화19q균불결실。수방4개월~4년,1례술후2년종류복발병파산。결론뇌실외신경세포류여중추신경세포류조직학표현수상사,단기경복잡,다견신경절세포혹신경절양세포분화,개화、혈관벽증후급투명변성상견,신경전양결구소견。수여소지효질세포류、실관막류、배태발육불량성신경상피종류등감별,면역조화표기급염색체1p/19q결실검측유조우감별진단。치료이수술전절제위주,대우차전절제、비전형급복발병례술후응보이방료。
Purpose To study the clinicopathological features of extraventricular neurocytoma. Methods 12 cases of extraventricular neurocytoma were retrospectively analyzed for their clinical information and histopathological features. Results The distribution of ex-traventricular neurocytoma was as follows: cerebral hemisphere (5 cases), right thalamus (2 cases), cerebellar vermis (2 cases), saddle area (2 cases), and spinal cord (1 case). The histological features were round uniform cells, round or oval nuclei, with peri-nuclear clearing commonly seen. Neuropil-like structure, ganglion cells or ganglion-like cells, perivascular rosettes, calcification, cap-illary wall thickening and hyaline degeneration and other pathological changes were also observed. All cases showed strong immunoreac-tivity for Syn. 7 cases exhibited Ki-67 labeling index of≤2%, and other 5 cases showed 3% ~8%. 3 cases were performed with fluo-rescence in situ hybridization ( FISH) to detect loss of chromosome 1p/19q, but no loss was detected. Postoperative follow-up for 4 months to 4 years showed one case had tumor recurrence and intracranial spread in postoperative 2 years. Conclusions Extraventricu-lar neurocytoma shares similar histological features with central neurocytoma, but often more complex. Ganglion cells or ganglion-like cells, calcification, capillary wall thickening and hyaline degeneration are more frequently seen, but neuropil-like structure is less seen. Extraventricular neurocytoma needs differential diagnoses from oligodendroglioma, ependymoma, dysembryoplastic neuroepitheli-al tumor and others. Immunohistochemistry and chromosome 1p/19q loss can help in the differential diagnosis. The treatment for ex-traventricular neurocytoma is grossly total resection, with post-operative radiotherapy for subtotal resection, atypical or recurrent cases.