临床与实验病理学杂志
臨床與實驗病理學雜誌
림상여실험병이학잡지
CHINESE JOURNAL OF CLINICAL AND EXPERIMENTAL PATHOLOGY
2014年
4期
419-422
,共4页
乳头状胶质神经元肿瘤%神经元-胶质混合性肿瘤%临床病理
乳頭狀膠質神經元腫瘤%神經元-膠質混閤性腫瘤%臨床病理
유두상효질신경원종류%신경원-효질혼합성종류%림상병리
papillary glioneuronal tumor%mixed neuronal-glial tumor%clinicopathology
目的：探讨乳头状胶质神经元肿瘤( papillary glioneuronal tumor, PGNT)的临床病理学特征、免疫表型及鉴别诊断。方法分析3例PGNT的临床表现、影像学、病理形态及免疫表型特征,并复习相关文献。结果本组中女性2例,男性1例,临床表现为头晕或慢性难治性癫痫,头部MRI检查显示左侧颞叶或右侧顶叶脑实质内囊性占位。镜下可见肿瘤由星形胶质细胞、少突样胶质细胞和神经节细胞及神经节样细胞构成,呈假乳头状排列,乳头中心为玻璃样变性的血管。免疫表型：星形胶质细胞GFAP阳性,少突样胶质细胞Olig-2阳性,神经节细胞Syn、NeuN阳性。结论 PGNT是一种新增的、少见的神经元-胶质混合性肿瘤,绝大多数呈良性经过,属WHOⅠ级,预后良好。熟悉其病理学特征有助于与其他具有乳头状结构的中枢神经系统肿瘤鉴别诊断。
目的：探討乳頭狀膠質神經元腫瘤( papillary glioneuronal tumor, PGNT)的臨床病理學特徵、免疫錶型及鑒彆診斷。方法分析3例PGNT的臨床錶現、影像學、病理形態及免疫錶型特徵,併複習相關文獻。結果本組中女性2例,男性1例,臨床錶現為頭暈或慢性難治性癲癇,頭部MRI檢查顯示左側顳葉或右側頂葉腦實質內囊性佔位。鏡下可見腫瘤由星形膠質細胞、少突樣膠質細胞和神經節細胞及神經節樣細胞構成,呈假乳頭狀排列,乳頭中心為玻璃樣變性的血管。免疫錶型：星形膠質細胞GFAP暘性,少突樣膠質細胞Olig-2暘性,神經節細胞Syn、NeuN暘性。結論 PGNT是一種新增的、少見的神經元-膠質混閤性腫瘤,絕大多數呈良性經過,屬WHOⅠ級,預後良好。熟悉其病理學特徵有助于與其他具有乳頭狀結構的中樞神經繫統腫瘤鑒彆診斷。
목적：탐토유두상효질신경원종류( papillary glioneuronal tumor, PGNT)적림상병이학특정、면역표형급감별진단。방법분석3례PGNT적림상표현、영상학、병리형태급면역표형특정,병복습상관문헌。결과본조중녀성2례,남성1례,림상표현위두훈혹만성난치성전간,두부MRI검사현시좌측섭협혹우측정협뇌실질내낭성점위。경하가견종류유성형효질세포、소돌양효질세포화신경절세포급신경절양세포구성,정가유두상배렬,유두중심위파리양변성적혈관。면역표형：성형효질세포GFAP양성,소돌양효질세포Olig-2양성,신경절세포Syn、NeuN양성。결론 PGNT시일충신증적、소견적신경원-효질혼합성종류,절대다수정량성경과,속WHOⅠ급,예후량호。숙실기병이학특정유조우여기타구유유두상결구적중추신경계통종류감별진단。
Purpose To investigate clinicopathological features, immunohistochemical findings and differential diagnosis of papillary glioneuronal tumor ( PGNT) . Methods The clinical features, imaging, histological and immunohistochemical findings were analyzed in three cases of PGNT, along with review of the related literatures. Results Two patients were female adults, and the other was male adults. One presented with dizziness, and the other exhibited intractable seizures. Magnetic resonance imaging ( MRI) revealed a cyst-ic space-occupying mass in brain parenchyma of left temporal lobe or right parietal lobe. Microscopically, it was characterized by pseu-dopapillary structures composed of hyalinized vessels rimmed by cuboidal glial cells, oligodendrocyte-like cells and the proliferation of neuronal cells. On immunohistochemical staining, the perivascular cuboidal cells were GFAP-positive, the oligodendrocyte-like cells were Olig-2-positive, and the neuronal cells were synaptophysin-and nuclear protein-positive. Conclusion PGNT is a rare and new variant of mixed neuronal-glial neoplasm, the natural evolution of this tumor is one of low malignant potential and it has a good progno-sis. The distinctive pathologic features help to differentiate this entity from other neoplasm of the central nervous system which has pa-pillary structures.
