Since the discovery of USP6 gene rearrangements in aneurysmal bone cysts nearly 20 years ago, we have come to recognize that there is a family of USP6-driven mesenchymal neoplasms with overlapping clinical, morphologic, and imaging features. This family of neoplasms now includes myositis ossificans, aneurysmal bone cyst, nodular fasciitis, fibroma of tendon sheath, fibro-osseous pseudotumor of digits, and their associated variants. While generally benign and in many cases self-limiting, these lesions may undergo rapid growth, and be confused with malignant bone and soft tissue lesions, both clinically and on imaging. The purpose of this article is to review the imaging characteristics of the spectrum of USP6-driven neoplasms, highlight key features that allow distinction from malignant bone or soft tissue lesions, and discuss the role of imaging and molecular analysis in diagnosis.

自从近 20 年前在动脉瘤性骨囊肿中发现USP6基因重排以来，我们已经认识到存在一个具有重叠临床、形态学和影像学特征的USP6驱动的间充质肿瘤家族。该肿瘤家族现在包括骨化性肌炎、动脉瘤性骨囊肿、结节性筋膜炎、腱鞘纤维瘤、手指纤维骨性假瘤及其相关变体。虽然通常是良性的并且在许多情况下是自限性的，但这些病变可能会快速生长，并且在临床上和影像学上都与恶性骨和软组织病变相混淆。本文的目的是回顾USP6光谱的成像特征驱动的肿瘤，强调可以与恶性骨或软组织病变区分开来的关键特征，并讨论成像和分子分析在诊断中的作用。