Journal of Peking University(Health Sciences) ›› 2017, Vol. 49 ›› Issue (5): 904-909. doi: 10.3969/j.issn.1671-167X.2017.05.029

• Article • Previous Articles     Next Articles

Clinicopathologic features of infant dysembryoplastic neuroepithelial tumor: a case report and literature review

WANG Hui1, 2, YE Jin-tang3, YAO Hong-xin4, LI Dong1, DONG Ying1   

  1. 1. Department of Pathology, 2. Laboratory of Electron Microscopy,3. Department of Imaging,4. Department of Pediatric Surgery, Peking University First Hospital, Beijing 100034, China
  • Received:2016-10-13 Online:2017-10-18 Published:2017-10-18

Abstract: SUMMARY Dysembryoplastic neuroepithelial tumor (DNT) has traditionally been viewed as rare benign tumors that present with seizure widely considered curable with surgery alone. Most DNTs occur in childhood and young adults. However, rare reported cases occur in infants. This paper reported an infant case of DNT and its diagnosis, differential diagnosis, treatment, molecular features and prognosis based on the review of current literatures. The age onset of this patient was only 11 months old. The clinical manifestations were partial seizures and the imaging data untypical;CT and MRI were all supportive of astrocytoma. Typical glioneuronal element histologic features could be seen, which contained oligodendrocyte-like cells attached to bundles of axons and neurons floating in a myxoid interstitial fluid. Meanwhile, some atypical regions could also be seen. These atypical regions showed a mixture of oligodendrocyte-like cells and neurons without a myxoid interstitial fluid, which were easily misdiagnosed. The BRAFV600E mutation was not detected. This patient had a good response to drug therapy. Totally surgical resection of the tumor was conducted. The patient had been seizures free for 6 months. In conclusion, DNT is a rare and well prognostic tumor (WHO grade Ⅰ), which most often arise in children in the setting of medically refractory epilepsy. The most common tumor location was temporal. Because clinical symptoms, imaging and histological features of DNT and other low-grade gliomas broadly overlap such as ganglioglioma, pilocytic astrocytomas and oligodendroglioma et al., differential diagnosis should be made carefully. The glioneuronal element was the histopathological hallmark of DNT. In addition, some untypical regions should also be called attention. Although BRAFV600E mutation didn’t exist in this case, it played a role in differential diagnosis because it has been previously recorded that BRAFV600E mutation was a common feature of DNT. Infant patients have their own characteristics. For example, drug therapy worked well and the imaging data was untypical. Doctors should improve the understanding of this disease to avoid unnecessary radiothe-rapy or chemotherapy.

Key words: Brain neoplasms, Neoplasms, neuroepithelial, Epilepsy, Pathology, clinical, Prognosis

CLC Number: 

  • R446.8
[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 thirty-nine cases [J]. Neurosurgery, 1988, 23(5): 545-556.
[2] Nowell M, Miserocchi A, McEvoy AW. Tumors in epilepsy [J]. Semin Neurol, 2015, 35(3): 209-217.
[3] 齐雪岭, 罗麟, 姚坤, 等. 胚胎发育不良性神经上皮肿瘤12例临床病理分析 [J]. 诊断病理学杂志, 2011, 18(6): 424-427.
[4] 张建国, 胡文忠, 李玉, 等. 胚胎发育不良性神经上皮肿瘤的临床病理分析 [J]. 中华病理学杂志, 2012, 41(8): 534-537.
[5] Honavar M, Janota I, Polkey CE. Histological heterogeneity of dysembryoplastic neuroepithelial tumor: identification and differential diagnosis in series of 74 cases [J]. Histopathology, 1999, 34(4): 342-356.
[6] Thom M, Toma A, An S, et al. One hundred and one dysembryoplastic neuroepithelial tumors: an adult epilepsy series with immunohistochemical, molecular genetics, and clinical correlations and a review of the literature [J]. J Neuropathol Exp Neurol, 2011, 70(10): 859-878.
[7] Stanescu Cosson R, Varlet P, Beuvon F, et al. Dysembryoplastic neuroepithelial tumors: CT, MR findings and imaging follow up: a study of 53 cases [J]. J Neuroradiol, 2001, 28(4): 230-240.
[8] Fernandez C, Girard N, PazParedes A, et al. The usefulness of MR imaging in the diagnosis of dysembryoplastic neuroepithelial tumor in children: a study of 14 cases [J]. Am J Neuroradiol, 2003, 24(5): 829-834.
[9] Cervera-Pierot P, Varlet P, Chodkiewicz JP, et a1. Dysembryoplastic neuroepithelial tumors located in the caudate nucleus area: report of four cases[J]. Neurosurgery, 1997, 40(5): 1065-1070.
[10] 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 ten cases [J]. Am J Surg Pathol, 2001, 25(4): 494-499.
[11] 许硕果, 梁文, 贾洪顺, 等. 胚胎发育不良性神经上皮肿瘤的MRI表现 [J]. 中华神经医学杂志, 2011, 10(1): 84-87.
[12] 武春雪, 高陪毅. 脑内胚胎发育不良性神经上皮瘤与低级别星形胶质瘤的MRI比较研究 [J]. 放射学实践, 2012, 27(3): 279-283.
[13] Campos AR, Clusmann H, von Lehe M, et al. Simple and complex dysembr yoplastic neuroepithelial tumors (DNT) variants: clinical profile, MRI, and histopathology [J]. Neuroradiology, 2009, 51(7): 433-443.
[14] Dougherty MJ, Santi M, Brose MS, et al. Activating mutations in BRAF characterize a spectrum of pediatric low-grade gliomas [J]. Neuro Oncol, 2010, 12(7): 621-630.
[15] Dimitriadis E, Alexiou GA, Tsotsou P, et al. BRAF alterations in pediatric low grade gliomas and mixed neuronal-glial tumors [J]. J Neurooncol, 2013, 113(3): 353-358.
[16] Prabowo AS, Iyer AM, Veersema TJ, et al. BRAF V600E mutation is associated with mTOR signaling activation in glioneuronal tumors [J]. Brain Pathol, 2014, 24(1): 52-66.
[17] Chappe C, Padovani L, Scavarda D, et al. Dysembryoplastic neuroepithelial tumors share with pleomorphic xanthoastrocytomas and gangliogliomas BRAF (V600E) mutation and expression [J]. Brain Pathol, 2013, 23(5): 574-583.
[18] Lee D, Cho YH, Kang SY, et al. BRAF V600E mutations are frequent in dysembryoplastic neuroepithelial tumors and subependymal giant cell astrocytomas [J]. J Surg Oncol, 2015, 111(3): 359-364.
[19] Prayson RA. Composite ganglioglioma and dysembryoplastic neuroepithelial tumor [J]. Arch Pathol Lab Med, 1999, 123(3): 247-250.
[20] Shimbo Y, Takahashi H, Hayano M, et al. Temporal lobe lesion demonstrating features of dysembryoplastic neuroepithelial tumor and ganglioglioma: a transitional form? [J]. Clin Neuropathol, 1997, 16(2): 65-68.
[21] Prayson RA, Napekoski KM. Composite ganglioglioma/dysembryoplastic neuroepithelial tumor: a clinicopathologic study of 8 cases [J]. Hum Pathol, 2012, 43(7): 1113-1118.
[22] Ishizawa K,Terao S, Kobayashi K, et al. A neuroepithelial tumor showing combined histological features of dysembryoplastic neuroepithelial tumor and pleomorphic xanthoastrocytoma: a case report and review of the literature [J]. Clin Neuropathol, 2007, 26(4): 169-175.
[23] 姜涛, 王军梅, 薛超强, 等. 胚胎发育不良性神经上皮肿瘤的外科治疗方式及癫痫预后[J]. 中华神经外科杂志, 2013, 29(4): 350-353.
[24] 虚建堃, 单永治, 朴月善, 等. 胚胎发育不良性神经上皮肿瘤致癫痫的手术治疗[J]. 中华神经外科杂志, 2014, 30(9): 904-907.
[25] Nolan MA,Sakuta R, Chuang N, et al. Dysembryoplatic neuroepithelial tumors in childhood: long term outcome and prognostic features [J]. Neurology, 2004, 62(12): 2270-2276.
[26] Ray WZ, Blackburn SL,Casavilcazambrano S, et al. Clinico-pathologic features of recurrent dysembryoplastic neuroepithelial tumor and rare malignant transformation: a report of 5 cases and review of the literature [J]. J Neurooncol, 2009, 94(2): 283-292.
[27] Zakrzewski K, Biernat W, Liberski PP, et al. Pilocytic astrocytoma as a predominant component of a recurrent complex type DNT [J]. Folia Neuropathol, 2009, 47(3): 284-288.
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