Current Model Systems for Investigating Epithelioid Haemangioendothelioma.
Current Model Systems for Investigating Epithelioid Haemangioendothelioma.
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上皮样血管内皮瘤是一种罕见的癌症，其病程不可预测，且治疗选择很少。为了更好地了解这种类型的癌症如何发展并发现可能的治疗方案，采用实验方法来研究这种癌症至关重要。在这里，我们描述并比较了目前可用于研究这种疾病的模型系统。介绍了使用每个实验模型进行的研究和取得的发现，并讨论了每个模型的优点和缺点。上皮样血管内皮瘤（EHE）是一种罕见的血管内皮肉瘤，其病程不可预测。 EHE肿瘤可以长时间保持惰性，但可能突然演变为具有广泛转移和预后不良的侵袭性疾病。两种相互排斥的染色体易位定义了 EHE 肿瘤，每种易位都涉及转录辅助因子 TAZ 和 YAP 之一。 TAZ-CAMTA1 融合蛋白由 t(1;3) 易位产生，存在于 90% 的 EHE 肿瘤中。其余 10% 的 EHE 病例存在 t(X;11) 易位，产生 YAP1-TFE3 (YT) 融合蛋白。直到最近，由于缺乏代表性的 EHE 模型，研究这些融合蛋白促进肿瘤发生的机制变得充满挑战。在这里，我们描述并比较了目前可用于研究这种癌症的最近开发的实验方法。在总结了每种实验方法获得的主要发现之后，我们讨论了这些不同模型系统的优点和局限性。我们对当前文献的调查显示了如何以不同的方式利用每种实验方法来提高我们对 EHE 发生和进展的理解。最终，这将为患者带来更好的治疗选择。
Epithelioid haemangioendothelioma is a rare type of cancer with an unpredictable disease course and very few treatment options. To better understand how this type of cancer develops and to uncover possible lines of treatment, it is critical to have experimental approaches to study this cancer. Here, we describe and compare the model systems that are currently available to study this disease. The research undertaken and the discoveries made using each of these experimental models is presented, and the advantages and disadvantages of each model are discussed. Epithelioid haemangioendothelioma (EHE) is a rare sarcoma of the vascular endothelium with an unpredictable disease course. EHE tumours can remain indolent for long period of time but may suddenly evolve into an aggressive disease with widespread metastases and a poor prognosis. Two mutually exclusive chromosomal translocations define EHE tumours, each involving one of the transcription co-factors TAZ and YAP. The TAZ-CAMTA1 fusion protein results from a t(1;3) translocation and is present in 90% of EHE tumours. The remaining 10% of EHE cases harbour a t(X;11) translocation, resulting in the YAP1-TFE3 (YT) fusion protein. Until recently, the lack of representative EHE models made it challenging to study the mechanisms by which these fusion proteins promote tumorigenesis. Here, we describe and compare the recently developed experimental approaches that are currently available for studying this cancer. After summarising the key findings obtained with each experimental approach, we discuss the advantages and limitations of these different model systems. Our survey of the current literature shows how each experimental approach can be utilised in different ways to improve our understanding of EHE initiation and progression. Ultimately, this should lead to better treatment options for patients.
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影响因子：
64.8
作者：
Chang L;Azzolin L;Di Biagio D;Zanconato F;Battilana G;Lucon Xiccato R;Aragona M;Giulitti S;Panciera T;Gandin A;Sigismondo G;Krijgsveld J;Fassan M;Brusatin G;Cordenonsi M;Piccolo S
通讯作者：
Piccolo S
影响因子：
19
作者：
Ceccaldi R;Rondinelli B;D'Andrea AD
通讯作者：
D'Andrea AD
影响因子：
78.5
作者：
Collado, Manuel;Serrano, Manuel
通讯作者：
Serrano, Manuel
TAZ通过在神经母细胞瘤细胞中结缔组织生长因子表达的上调促进上皮过渡。
影响因子：
3.4
作者：
Wang Q;Xu Z;An Q;Jiang D;Wang L;Liang B;Li Z
通讯作者：
Li Z
影响因子：
10.5
作者：
Guo X;Zhao Y;Yan H;Yang Y;Shen S;Dai X;Ji X;Ji F;Gong XG;Li L;Bai X;Feng XH;Liang T;Ji J;Chen L;Wang H;Zhao B
通讯作者：
Zhao B