The role of CD133+ cells in a recurrent embryonal tumor with abundant neuropil and true rosettes (ETANTR).
The role of CD133+ cells in a recurrent embryonal tumor with abundant neuropil and true rosettes (ETANTR).
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DOI：
10.1111/bpa.12079
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发表时间：
2014-01
期刊：
影响因子：
--
通讯作者：
Camelo-Piragua S
中科院分区：
文献类型：
--
作者：
Hervey-Jumper SL;Altshuler DB;Wang AC;He X;Maher CO;Robertson PL;Garton HJ;Fan X;Muraszko KM;Camelo-Piragua S
胚胎性肿瘤伴丰富的神经纤维瘤和真神经纤维瘤（ETANTR）是一种新近发现的中枢神经系统胚胎性肿瘤，由边界清楚的婴儿期胚胎性肿瘤组成，具有室管膜母细胞瘤（多层室管膜母细胞瘤和假室管膜母细胞瘤）和神经母细胞瘤（神经母细胞瘤）的混合特征，并存在神经纤毛样岛。我们报告一个小孩子的恶性肿瘤，在全切除、化疗和放疗后迅速复发。显著的血管硬化和局限性肿瘤导致恶性星形细胞瘤的诊断;然而，这种大肿瘤在大体全切除后的快速复发和进展促使对原始病理进行审查。在存在神经元和室管膜母细胞瘤以及局灶性神经元岛的情况下，ETANTR与局灶性GFAP和突触素表达在组织学上不同。这些结构特征，结合独特的染色体19q.13.42扩增，证实了诊断。在这份报告中，我们描述了肿瘤干细胞（TSC）标志物CD 133，CD 15，和巢蛋白的变化在ETANTR化疗前后。我们发现，TSC标志物CD 133在复发性ETANTR中化疗后丰富表达，而与原始肿瘤中表达的相比，CD 15被耗尽，这表明CD 133+细胞可能在初始治疗中存活，进一步促进复发性肿瘤的形成。
Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a recently described embryonal neoplasm of the central nervous system, consisting of a well-circumscribed embryonal tumor of infancy with mixed features of ependymoblastoma (multilayer ependymoblastic rosettes and pseudorosettes) and neuroblastoma (neuroblastic rosettes) in the presence of neuropil-like islands. We present the case of a young child with a very aggressive tumor that rapidly recurred after gross total resection, chemotherapy, and radiation. Prominent vascular sclerosis and circumscribed tumor led to the diagnosis of malignant astroblastoma; however, rapid recurrence and progression of this large tumor after gross total resection prompted review of the original pathology. ETANTR is histologically distinct with focal GFAP and synaptophysin expression in the presence of neuronal and ependymoblastic rosettes with focal neuropil islands. These architectural features, combined with unique chromosome 19q.13.42 amplification, confirmed the diagnosis. In this report, we describe tumor stem cell (TSC) marker CD133, CD15, and nestin alterations in ETANTR before and after chemotherapy. We found that TSC marker CD133 was richly expressed after chemotherapy in recurrent ETANTR, while CD15 is depleted compared to that expressed in the original tumor, suggesting that CD133+ cells likely survived initial treatment, further contributing to formation of the recurrent tumor.
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