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;

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Keywords：

胚胎发育不良性神经上皮肿瘤; 临床; 病理; 诊断

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目的:探讨胚胎发育不良性神经上皮肿瘤(DNT)的临床、影像及病理学特征、诊断及鉴别诊断.方法:回顾性分析8例胚胎发育不良性神经上皮肿瘤患者的临床和影像学资料,进行光镜和免疫组织化学染色观察,并获得6例的随访资料.结果:胚胎发育不良性神经上皮肿瘤男性7例,女性1例,年龄为5～19岁,平均年龄13岁,5例以癫痫小发作为主要临床表现,病变均位于幕上,以皮层为主,影像学检查均无明显的占位效应及瘤周水肿。肿瘤细胞主要由少突胶质样细胞(OLC)、神经元和星形细胞组成,4例伴有皮质发育不良。免疫组织化学结果为神经元及部分少突胶质样细胞呈嗜铬素A、突触素及S-100阳性表达;少突胶质样细胞呈胶质纤维酸性蛋白(GFAP)阴性表达,而星形细胞呈GFAP阳性表达;Ki-67抗原标记指数 lt;1%。结论: 胚胎发育不良性神经上皮肿瘤为WHOⅠ级良性肿瘤,可结合临床、影像及病理学表现明确诊断,预后良好,无需放疗和化疗。

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(回允中主译). 阿克曼外科病理学[M]. 第8 版, 沈阳:辽宁教育出版社,1999:2308.
8.	李南云,周晓军,金行藻,等.胚胎发育不良性神经上皮瘤临床病理观察[J].中华病理学杂志.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(回允中主译). 阿克曼外科病理学[M]. 第8 版, 沈阳:辽宁教育出版社,1999:2308.
8. 李南云,周晓军,金行藻,等.胚胎发育不良性神经上皮瘤临床病理观察[J].中华病理学杂志.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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