Nearly 20 years ago, the first description of a translocation involving chromosome 17 on which USP6 resides was described. Since then, not only the culprit gene but also many fusion partners, leading to transcriptional activation of USP6, have been detected. The first neoplasm known to harbor USP6 rearrangements was aneurysmal bone cyst. Since then, other entities like nodular fasciitis, myositis ossificans, fibro-osseous pseudotumor of digits, and a subgroup of fibromas of tendon sheath, probably representing tenosynovial nodular fasciitis, have been added to the list of USP6-rearranged lesions. Remarkably, all of them share clinical as well as morphological characteristics, and authors have suggested that these entities actually belong to the same spectrum. This review summarizes the current knowledge regarding USP6-rearranged lesions and further elaborates on how these neoplasms relate to one another. We propose to call these lesions UAN (Usp6-associated neoplasm).

译文

:近20年前,首次描述了涉及USP6所在的17号染色​​体的易位。从那时起,不仅发现了罪魁祸首基因,而且还发现了许多融合伴侣,导致USP6的转录激活。已知具有USP6重排的第一个肿瘤是动脉瘤性骨囊肿。从那时起,其他实体,如结节性筋膜炎,骨化性肌炎,指骨纤维性假瘤以及可能代表腱鞘结节性筋膜炎的肌腱鞘纤维瘤亚组,已被添加到USP6重排病变中。值得注意的是,它们都具有临床和形态特征,并且作者建议这些实体实际上属于同一光谱。这篇综述总结了有关USP6重排病变的当前知识,并进一步阐述了这些肿瘤之间的相互关系。我们建议将这些病变称为UAN(与Usp6相关的肿瘤)。

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