Dysembryoplastic Neuroepithelial Tumor: A Clinicopathologic Analysis of 8 Cases_West China Medical Journal

Authors：

1Department of Pathology, West China Hospital, Sichuan University, Sichuan Chengdu 610041; 2 Department of Pathology, Afffilicated Taihe Hospital of Yunyang Medical College, Hubei Shiyan 442000,China;

Corresponding?author：

Keywords：

胚胎發育不良性神經上皮腫瘤; 臨床; 病理; 診斷

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目的:探討胚胎發育不良性神經上皮腫瘤(DNT)的臨床、影像及病理學特征、診斷及鑒別診斷.方法:回顧性分析8例胚胎發育不良性神經上皮腫瘤患者的臨床和影像學資料,進行光鏡和免疫組織化學染色觀察,并獲得6例的隨訪資料.結果:胚胎發育不良性神經上皮腫瘤男性7例,女性1例,年齡為5～19歲,平均年齡13歲,5例以癲癇小發作為主要臨床表現,病變均位于幕上,以皮層為主,影像學檢查均無明顯的占位效應及瘤周水腫。腫瘤細胞主要由少突膠質樣細胞(OLC)、神經元和星形細胞組成,4例伴有皮質發育不良。免疫組織化學結果為神經元及部分少突膠質樣細胞呈嗜鉻素A、突觸素及S-100陽性表達;少突膠質樣細胞呈膠質纖維酸性蛋白(GFAP)陰性表達,而星形細胞呈GFAP陽性表達;Ki-67抗原標記指數 lt;1%。結論: 胚胎發育不良性神經上皮腫瘤為WHOⅠ級良性腫瘤,可結合臨床、影像及病理學表現明確診斷,預后良好,無需放療和化療。

Citation： YUAN Lingling,XU Xia,ZHANG Shangfu,et al.. Dysembryoplastic Neuroepithelial Tumor: A Clinicopathologic Analysis of 8 Cases. West China Medical Journal, 2009, 24(9): 2347-2351. doi: Copy

1.	DAUMASDUPORT C, SCHEITHAUERBW, CHODKIEWICZ JP,et al. Dysembryoplastic neuroepithelial tumor: A surgically curable tumor of young patients with intractable partial seizures. Report of 39 cases[J]. Neurosurgery, 1988, 23:545-556.
2.	DAUMASDUPORT C, VARLET P, BACHA S,et al. Dysembryoplastic neuroepithelial tumors: Nonspecific histological formsa study of 40 cases[J]. J NeuroOncology, 1999, 41:267.
3.	KLEIHUES P, CAVENEE WK. Pathology and genetics of tumors of The nervous system: World Health Organization classification of Tumor[M].Lyon: IARC Press, 2000:103-106.
4.	ROSEMBERG S, VIEIRA GS. Dysembryoplastic neuroepithelial tumor. An epidemiological study from a single Insitution[J]. Arq Neuropsiquiatr, 1998,56(2):232.
5.	TARATUTO AL, POMATA H, SEVLEVER G, et al. Dysembryoplastic neuropithelial tumor: morphological, immunocytochemical, and deoxyribonucleic analyses in a pediatric series[J]. Neurosurgery, 1995, 36(3):474.
6.	BAISDEN BL, BRAT DJ, MELHEM ER, et al.Dysembryoplastic neuroepithelial tumorlike neoplasm of the septum pellucidum: a lesion often misdiagnosed as glioma: report of 10 cases[J]. Am J SurgPathol, 2001,25(4):494-9.
7.	ROSAI J 著(回允中主譯). 阿克曼外科病理學[M]. 第8 版, 沈陽:遼寧教育出版社,1999:2308.
8.	李南云,周曉軍,金行藻,等.胚胎發育不良性神經上皮瘤臨床病理觀察[J].中華病理學雜志.2005,34(9):561-564.
9.	MORENO A, DE FELIPE J, GARCIA SOLA R,et al. Neuronal and mixed neuronal glial tumors associated to epilepsy: A heterogeneous and related group of tumours[J]. Histol Histopathol, 2001, 16(2):613-622.
10.	FERNANDEZ C, GIRARD N, PAZ PAREDES A, et al. The usefulness of MR imaging in the diagnosis of dysembryoplastic neuroepithelial tumors in children:a study of 14 cases[J]. AJNR Am J Neuroradiol,2003,24(5):829-834.
11.	KORDEK R, BIERNAT W, ZAKRZEWSKI K, et al. Dysembryoplastic neuroepithelial tumor (DNT): an ultrastructural study of six cases[J]. Folia Neuropathol, 1999, 37 (3):167-170.
12.	HIROSE T, SCHEITHAUER BW, LOPES MB, et al. Dysembryoplastic neuroepithelial tumor(DNT): an immunohistochemical and ultrastructural study[J]. J Neuropathol Exp Neurol, 1994, 53(2):184-195.
13.	HONAVAR M, JANOTA I, POLKEY CE. Histological heterogeneity of dysembryoplastic neuroepithelial tumor: Identification and differential diagnosis in a series of 74 cases[J]. Histopathology, 1999, 34:342.
14.	PRAYSON RA, CASTILLA EA, HARTKE M, et al.Chromosome 1p allelic loss by fluorescence in situ hybridization is not observed in dysembryoplastic neuroepithelial tumors[J]. Am J Clin Pathol,2002, 118: 512-517.
15.	FUJISAWA H, MARUKAWA K, HASEGAWA M, et al. Genetic differences between neurocytoma and dysembryop lastic neuroep ithelial tumor and oligodendroglial tumors[J]. J Neurosurg, 2002, 97: 1350-1353.
16.	FERNANDEZ C, GIRARD N, PAZ PAREDESA, et al.The usefulness of MR imaging in the diagnosis of dysembryoplastic neuroepithelial tumor in children: a study of 14 cases[J]. AJNR Am J Neuroradiol, 2003, 24:829-834.
17.	NOLAN MA, SAKUTA R, CHUANG N, et al. Dysembryoplatic neuroepithelial tumors in childhood: longterm outcome and p rognostic features[J]. Neurology, 2004, 62: 2270-2276.
18.	DAUMASDUPORT C,LANTOS PL.Dysembryoplastic neuroepithelial tumours. In:Kleihues P, Cavenee WK, eds. Pathology and genetics tumours of the nervous system[M]. Lyon: International Agencyfor Researchon Cancer 1997:73-76.

1. DAUMASDUPORT C, SCHEITHAUERBW, CHODKIEWICZ JP,et al. Dysembryoplastic neuroepithelial tumor: A surgically curable tumor of young patients with intractable partial seizures. Report of 39 cases[J]. Neurosurgery, 1988, 23:545-556.
2. DAUMASDUPORT C, VARLET P, BACHA S,et al. Dysembryoplastic neuroepithelial tumors: Nonspecific histological formsa study of 40 cases[J]. J NeuroOncology, 1999, 41:267.
3. KLEIHUES P, CAVENEE WK. Pathology and genetics of tumors of The nervous system: World Health Organization classification of Tumor[M].Lyon: IARC Press, 2000:103-106.
4. ROSEMBERG S, VIEIRA GS. Dysembryoplastic neuroepithelial tumor. An epidemiological study from a single Insitution[J]. Arq Neuropsiquiatr, 1998,56(2):232.
5. TARATUTO AL, POMATA H, SEVLEVER G, et al. Dysembryoplastic neuropithelial tumor: morphological, immunocytochemical, and deoxyribonucleic analyses in a pediatric series[J]. Neurosurgery, 1995, 36(3):474.
6. BAISDEN BL, BRAT DJ, MELHEM ER, et al.Dysembryoplastic neuroepithelial tumorlike neoplasm of the septum pellucidum: a lesion often misdiagnosed as glioma: report of 10 cases[J]. Am J SurgPathol, 2001,25(4):494-9.
7. ROSAI J 著(回允中主譯). 阿克曼外科病理學[M]. 第8 版, 沈陽:遼寧教育出版社,1999:2308.
8. 李南云,周曉軍,金行藻,等.胚胎發育不良性神經上皮瘤臨床病理觀察[J].中華病理學雜志.2005,34(9):561-564.
9. MORENO A, DE FELIPE J, GARCIA SOLA R,et al. Neuronal and mixed neuronal glial tumors associated to epilepsy: A heterogeneous and related group of tumours[J]. Histol Histopathol, 2001, 16(2):613-622.
10. FERNANDEZ C, GIRARD N, PAZ PAREDES A, et al. The usefulness of MR imaging in the diagnosis of dysembryoplastic neuroepithelial tumors in children:a study of 14 cases[J]. AJNR Am J Neuroradiol,2003,24(5):829-834.
11. KORDEK R, BIERNAT W, ZAKRZEWSKI K, et al. Dysembryoplastic neuroepithelial tumor (DNT): an ultrastructural study of six cases[J]. Folia Neuropathol, 1999, 37 (3):167-170.
12. HIROSE T, SCHEITHAUER BW, LOPES MB, et al. Dysembryoplastic neuroepithelial tumor(DNT): an immunohistochemical and ultrastructural study[J]. J Neuropathol Exp Neurol, 1994, 53(2):184-195.
13. HONAVAR M, JANOTA I, POLKEY CE. Histological heterogeneity of dysembryoplastic neuroepithelial tumor: Identification and differential diagnosis in a series of 74 cases[J]. Histopathology, 1999, 34:342.
14. PRAYSON RA, CASTILLA EA, HARTKE M, et al.Chromosome 1p allelic loss by fluorescence in situ hybridization is not observed in dysembryoplastic neuroepithelial tumors[J]. Am J Clin Pathol,2002, 118: 512-517.
15. FUJISAWA H, MARUKAWA K, HASEGAWA M, et al. Genetic differences between neurocytoma and dysembryop lastic neuroep ithelial tumor and oligodendroglial tumors[J]. J Neurosurg, 2002, 97: 1350-1353.
16. FERNANDEZ C, GIRARD N, PAZ PAREDESA, et al.The usefulness of MR imaging in the diagnosis of dysembryoplastic neuroepithelial tumor in children: a study of 14 cases[J]. AJNR Am J Neuroradiol, 2003, 24:829-834.
17. NOLAN MA, SAKUTA R, CHUANG N, et al. Dysembryoplatic neuroepithelial tumors in childhood: longterm outcome and p rognostic features[J]. Neurology, 2004, 62: 2270-2276.
18. DAUMASDUPORT C,LANTOS PL.Dysembryoplastic neuroepithelial tumours. In:Kleihues P, Cavenee WK, eds. Pathology and genetics tumours of the nervous system[M]. Lyon: International Agencyfor Researchon Cancer 1997:73-76.

West China Medical Journal

Dysembryoplastic Neuroepithelial Tumor: A Clinicopathologic Analysis of 8 Cases

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