Dysembryoplastic Neuroepithelial Tumor의 임상 및 수술 경험
Neuroepithelial Tumor
채종희, 한승규, 김기중, 황용승, 조병규,
소속 상세정보
채종희 ( Chae Jong-Hee )
서울대학교 의과대학 소아과학교실
한승규 ( Han Seung-Kyoo )
서울대학교 의과대학 소아과학교실
김기중 ( Kim Ki-Joong )
서울대학교 의과대학 소아과학교실
황용승 ( Hwang Yong-Seung )
서울대학교 의과대학 소아과학교실
조병규 ( Cho Byung-Kyu )
서울대학교 의과대학 신경외과학교실
KMID : 0877219980020010013
Abstract
Backgrounds : Dysembryoplastic neuroepithelial tumor (DNT) is a newly recognized brain tumor with distinctive clinicopathologic features and favorable prognosis in the aspect of cure of brain mass as well as control of intractable seizure. We report our experiences of 10 patients with DNT, who underwent surgical excision.
Methods: We reviewed clinical presentation, EEG features, neuroimage findings and postoperative outcome of 10 patients with pathologically proven DNTs.
Results: All patients except one had complex partial seizure and mean age of seizure onset was 5 year and 9 months old. They all had normal developmental milestone, normal intelligences and no abnormalities in neurologic examination. Seven of 8 patients showed abnormal interictal EEG findings : Six of 7 patients had focal EEG abnormalities such as focal spike or focal background slowing. In brain MRI the lesion was mainly located at intra/subcortical area. They all showed low signal intensities in T1 weighted image and high signal intensities in T2 weighted image. The lesion involved temporal lobe in 7 of 10 patients, 2 in frontal and 1 in parietal lobe. All but one had no enhancements in Gadolinium enhanced MRI. Other MRI features included : associated cysts formation (6/10), calcification (3/10), surrounding brain gray/white blurring (3/ 10) and hippocampal sclerosis (1/10). All patients underwent gross total removal of mass or lesionectomy with standard temporal lobectomy. They all had not experienced further seizures or recurrence of tumor mass after operation.
Conclusions: DNT is a distinctive brain mass lesion that arise in young hosts, involves the predominantly temporal lobe and cortex, and exhibits stable behavior. The recognition and correct diagnosis of DNT is important, because surgical excision itself is curable and unnecessary aggressive therapy can be avoided. In the aspect of complete seizure control, DNT has an excellent post operative prognosis.
키워드
Dysembryoplastic neuroepithelial tumor;Epilepsy;Children;Complex partial seizure
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