本文選題：腦腫瘤 + 腫瘤�。� 參考：《北京大學(xué)學(xué)報(醫(yī)學(xué)版)》2017年05期

【摘要】：報告1例嬰兒左顳葉胚胎發(fā)育不良性神經(jīng)上皮腫瘤(dysembryoplastic neuroepithelial tumor,DNT)病例,介紹其臨床病理特征、影像學(xué)特點、手術(shù)治療及預(yù)后,并復(fù)習(xí)國內(nèi)外文獻(xiàn)報道,對該病的診斷、鑒別診斷以及治療預(yù)后進(jìn)行綜合分析,以提高臨床及病理醫(yī)生對這一少見疾病的認(rèn)識。本例發(fā)病年齡早,患兒僅11個月大,臨床表現(xiàn)為部分性癲癇發(fā)作,但藥物反應(yīng)較好,影像學(xué)提示左顳葉占位并懷疑星形細(xì)胞瘤,但部分層面仍具有多見于DNT的三角形特征。本例病灶體積小,組織形態(tài)上不僅存在典型的特殊膠質(zhì)神經(jīng)元結(jié)構(gòu)-黏液柱狀結(jié)構(gòu),還存在一些非典型區(qū)域,周圍腦實質(zhì)不伴有皮層發(fā)育不良,未檢測到BRAFV600E位點突變,手術(shù)全切腫瘤6個月后癲癇無發(fā)作。DNT是一種少見、預(yù)后良好的神經(jīng)上皮腫瘤,嬰兒期發(fā)病患者有其自身的特點。手術(shù)完整切除腫瘤效果良好,無需放射治療或化學(xué)藥物治療。
[Abstract]:This paper reports a case of dysembryoplastic neuroepithelial tumor (DNT) of left temporal lobe embryogenesis in infants. The clinical and pathological features, imaging features, surgical treatment and prognosis of DNTs were introduced, and the diagnosis of the disease was reviewed. Differential diagnosis and treatment and prognosis were analyzed to improve the clinical and pathologist understanding of this rare disease. The patient was 11 months old and had early onset. The clinical manifestation was partial epileptic seizure, but the drug response was good. The left temporal lobe occupying and suspected astrocytoma were demonstrated by imaging, but some of them still had the triangle characteristic of DNT. In this case, the lesion was small in size, and there were not only typical glial neuron structure-myxoid columnar structure, but also some atypical regions. There was no cortical dysplasia in the surrounding brain parenchyma, and no mutation of BRAFV600E locus was detected. Epilepsy. DNT is a rare neuroepithelial tumor with good prognosis 6 months after total resection of tumor. The infantile patients have their own characteristics. Complete resection of tumor is effective without radiotherapy or chemotherapeutic therapy.
【作者單位】： 北京大學(xué)第一醫(yī)院病理科;北京大學(xué)第一醫(yī)院電鏡室;北京大學(xué)第一醫(yī)院影像科;北京大學(xué)第一醫(yī)院小兒外科;
【分類號】：R739.4
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本文編號：1843270