Isler Cihan, Erturk Cetin Ozdem, Ugurlar Doga, Ozkara Cigdem, Comunoglu Nil, Kizilkilic Osman, Oz Buge, Kayadibi Yasemin, Tanriverdi Taner, Uzan Mustafa
a Neurosurgery , Istanbul Universitesi Cerrahpasa Tip Fakultesi , Istanbul , Turkey.
b Neurology , Istanbul Universitesi Cerrahpasa Tip Fakultesi , Istanbul , Turkey.
Br J Neurosurg. 2018 Aug;32(4):436-441. doi: 10.1080/02688697.2018.1476671. Epub 2018 May 24.
To analyse the clinical, imaging and histopathological data of patients who were diagnosed to have Dysembrioplastic Neuroepithelial Tumour (DNET) and underwent surgery between 1995-2015.
Age at seizure onset, age at surgery, gender, disease duration, seizure outcome of 44 patients were analysed together with Magnetic Resonance Imaging (MRI) of 21 patients. MRI types were classified as type 1 (cystic/polycystic-like, well-delineated, strongly hypointense T1), type 2 (nodularlike,heterogeneous), type 3 (dysplastic-like, iso/hyposignal T1, poor delineation, gray-white matter blurring).
Histopathological classification revealed simple form in 19, complex in 14 and non-specific in 11 patients. Lobar distribution of the lesions was as follows: 21 Temporal (47.7%), 12 parietal (27.3%), 8 frontal (18.2%) and 3 occipital (6.8%). Type 1 MRI was observed in 10, type 2 was in 7, and type 3 in 4 patients on radiological evaluation. All cases with type 1 MRI corresponded to either simple or complex forms and all cases with type 3 MRI corresponded to nonspecific form. The histopathological distribution of cases with type 2 MRI was 4 as non-specific, 2 as simple, 1 as complex. There was no significant difference in the age of onset, age at operation and duration of epilepsy between the patients with different MRI subtypes. The majority of patients (N:36) had Engel I outcome (81,8%). In groups with Engel II and III outcome, duration of epilepsy was significantly higher (p:0,014) and simple form of DNET has significantly higher seizure freedom after surgery compared to complex and nonspecific forms of DNET (p:0,002).
Patients with DNET constitute a group with favorable outcomes after epilepsy surgery especially with early referral to surgery. Longer duration of epilepsy was associated with worse seizure outcome for DNET patients. There was significant correlation between radiological and histopathological types of DNET especially in type 1 and 3.
分析1995年至2015年间被诊断为胚胎发育不良性神经上皮肿瘤（DNET）并接受手术治疗的患者的临床、影像学和组织病理学数据。
分析44例患者的癫痫发作起始年龄、手术年龄、性别、病程、癫痫发作结果，并对21例患者进行磁共振成像（MRI）检查。MRI类型分为1型（囊性/多囊样，边界清晰，T1加权像呈明显低信号）、2型（结节样，不均匀）、3型（发育异常样，T1加权像呈等信号/低信号，边界不清，灰白质界限模糊）。
组织病理学分类显示，19例为单纯型，14例为复杂型，11例为非特异性型。病变的叶分布如下：颞叶21例（47.7%），顶叶12例（27.3%），额叶8例（18.2%），枕叶3例（6.8%）。影像学评估显示，10例患者为1型MRI，7例为2型，4例为3型。所有1型MRI病例均对应单纯型或复杂型，所有3型MRI病例均对应非特异性型。2型MRI病例的组织病理学分布为非特异性型4例，单纯型2例，复杂型1例。不同MRI亚型患者的发病年龄、手术年龄和癫痫病程无显著差异。大多数患者（n = 36）的Engel I级结果（81.8%）。在Engel II级和III级结果的组中，癫痫病程显著更长（p = 0.014），与DNET的复杂型和非特异性型相比，DNET的单纯型术后癫痫发作缓解率显著更高（p = 0.002）。
DNET患者是癫痫手术后预后良好的一组患者，尤其是早期转诊手术的患者。癫痫病程较长与DNET患者的癫痫发作结果较差相关。DNET的影像学和组织病理学类型之间存在显著相关性，尤其是在1型和3型中。
