伴有TFCP2重排的横纹肌肉瘤是一种近期被确认的恶性肿瘤，其免疫组化证据表明存在横纹肌样分化、角蛋白表达、ALK上调和临床病情恶化等特征。该肿瘤有影响颅颌骨的倾向，仅有少数报道其在骨外的肿瘤例子。本文中，我们提出了一个35岁女性患者的皮肤横纹肌肉瘤病例，其中具有FUS::TFCP2融合。值得注意的是，该肿瘤呈现出一种病理学谱系，初始时呈现为硬化性皮炎相似，但在8个月内逐渐进展为高级别恶性肿瘤。这种肿瘤的独特免疫表型突出了早期分子研究对于诊断的重要性，即使在低级别的细胞形态学出现时也是如此。早期发现可能为肿瘤变得无法切除之前提供时机。© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Rhabdomyosarcoma with TFCP2 rearrangement is a recently identified malignant neoplasm characterized by immunohistochemical evidence of rhabdomyoblastic differentiation, keratin expression, upregulation of ALK, and an aggressive clinical course. This neoplasm has a tendency to affect craniofacial bones, with only a few reported cases of extra-osseous tumors. Here, we present a case of cutaneous rhabdomyosarcoma with FUS::TFCP2 fusion in a 35-year-old female. Notably, the tumor exhibited a pathologic spectrum, initially resembling sclerosing dermatitis at presentation but progressing into a high-grade malignant tumor within 8 months. The distinctive immunoprofile of this neoplasm highlights the importance of early molecular studies for diagnosis, even in the presence of low-grade cytomorphology. Early detection may offer an opportunity for timely resection before the tumor becomes unresectable.© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.