我们呈现一组硬化上皮样纤维肉瘤（SEF）的病例系列，以进一步描述其临床和病理特征。本研究共纳入了21名患有SEF的患者，其中男性12名，女性9名，年龄范围为25-63岁，中位数为38岁。肿瘤位于肾脏（n=5）、大腿（n=3）、胸壁（n=3）、头颈部（n=2）、骨（n=2）、腹壁（n=1）、腰大肌（n=1）、腹膜后（n=1）、网膜（n=1）、腘窝（n=1）和肺（n=1）。肿瘤大小范围为2.5至16厘米，中位数为7厘米。在显微镜下，上皮样肿瘤细胞呈巢状和索状排列，在紧密的硬化基质中嵌入。一些肿瘤显示有粘液样区域、纤维瘤样区域、腺泡生长模式和血管平滑肌细胞瘤样外观。少数肿瘤细胞呈横纹肌肉样形状。在一些病例中观察到钙化、骨化、囊肿和坏死。通过对MUC4的免疫反应和进一步荧光原位杂交（FISH）或下一代测序（NGS）分析，确诊了该疾病。对16例患者进行了临床随访（中位时间24个月，范围6-62个月）。其中7名患者发生肺部 (n=3)、骨骼 (n=3)、脑 (n=2) 和背部 (n=1) 的转移。4名患者发生局部复发。3名患者死于该疾病。SEF的总生存率（OS）与患者年龄（p=0.001）有关，无进展生存期（PFS）与肿瘤大小（p=0.046）有关。除软组织外，SEF更可能涉及内脏和腹腔，并具有形态学变异。熟悉其独特的临床和病理特征有助于避免误诊。版权所有 ©2022澳大利亚皇家病理学学院。Elsevier B.V.保留所有权利。

We present a case series of sclerosing epithelioid fibrosarcoma (SEF) to further characterise its clinical and pathological features. Twenty-one patients with SEF were included in this study. There were 12 males and nine females (range 25-63 years; median 38 years). Tumours were located in the kidney (n=5), thigh (n=3), chest wall (n=3), head and neck (n=2), bone (n=2), abdominal wall (n=1), psoas major (n=1), retroperitoneum (n=1), omentum (n=1), popliteal space (n=1) and lung (n=1). Tumour sizes ranged from 2.5 to 16 cm (median 7 cm). Microscopically, epithelioid tumour cells were arranged in nests and cords and embedded in a dense sclerotic stroma. Some tumours showed myxoid areas, fibroma-like areas, acinar growth patterns and haemangiopericytoma-like appearance. A few tumour cells presented a rhabdomyoid shape. Calcification, ossification, cystic and necrosis were observed in some cases. The diagnosis was confirmed by immunoreactivity for MUC4, and by further fluorescence in situ hybridisation (FISH) or next generation sequencing (NGS) analysis. Clinical follow-up was available for 16 cases (median, 24 months; range 6-62 months). Seven patients developed metastases to lung (n=3), bone (n=3), brain (n=2) and back (n=1). Four patients developed a local recurrence. Three patients died of disease. Overall survival (OS) of SEF was related to patient age (p=0.001) and progression-free survival (PFS) was related to tumour size (p=0.046). In addition to soft tissue, SEF is more likely to involve the viscera and the abdominal cavity and has morphological variants. Familiarity with its distinctive clinical and pathological features helps avoid misdiagnosis.Copyright © 2022 Royal College of Pathologists of Australasia. Published by Elsevier B.V. All rights reserved.