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Embryonal tumors with abundant neuropil and true rosettes: 2 illustrative cases and a review of the literature

机译:具有丰富的神经绒毛和真玫瑰花结的胚胎肿瘤:2例病例并文献复习

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Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a recently identified variant of primitive neuroectodermal tumor, with fewer than 50 cases reported in the literature to date. Histologically, this tumor has features of ependymoblastoma and neuroblastoma, demonstrating areas of fine fibrillary neuropil intermingled with ependymoblastic rosettes and zones of undifferentiated neuroepithelial cells. However, ETANTR is distinguished pathologically from other embryonal tumors by the striking abundance of neuropil. Clinically, ETANTRs have shown high malignant potential and poor clinical outcome despite aggressive treatment. The authors describe 2 illustrative surgical cases of ETANTR, one involving the longest reported survival in the literature to date. The other had a poor outcome despite high-dose adjuvant chemotherapy with sequential autologous hematopoietic stem cell rescue. The authors review the natural history and treatment strategies available for this unusual malignant pediatric brain tumor.
机译:具有丰富的神经绒毛和真玫瑰花结(ETANTR)的胚胎肿瘤是最近发现的原始神经外胚层肿瘤的变体,迄今为止,文献报道的病例不到50例。从组织学上看,该肿瘤具有上皮细胞母细胞瘤和神经母细胞瘤的特征,表现出细纤维状神经纤维与表皮间质玫瑰花结交织的区域以及未分化的神经上皮细胞区域。然而,ETANTR在病理学上与其他胚胎肿瘤的区别在于其神经纤维的数量惊人。临床上,尽管积极治疗,但ETANTRs仍显示出高恶性潜能和不良的临床预后。作者描述了2例ETANTR手术病例,其中1例是迄今为止文献报道的最长生存期。尽管进行了大剂量辅助化疗并进行了自体造血干细胞抢救,但另一例的结局却很差。作者回顾了这种罕见的恶性小儿脑肿瘤的自然病史和治疗策略。

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