Ma Cao, Wei Xiaoying, Chen Zhe, Hao Xiangzhi, Sun Yuping, Zi Jie, Chu Chunyan, Zhang Lihua
Department of Pathology, School of Medicine, Zhongda Hospital, Southeast University, Nanjing, Jiangsu, China.
Department of Pathology, Lai'an County People's Hospital, Chuzhou, China.
Diagn Pathol. 2025 Aug 13;20(1):93. doi: 10.1186/s13000-025-01701-7.
Uterine inflammatory myofibroblastic tumour (IMT) is a relatively rare mesenchymal tumour of the uterus, with recurrence and metastasis rates of 25% and 2%, respectively. As IMT frequently harbours ALK gene rearrangements, some patients may benefit from treatment with tyrosine kinase inhibitors, making accurate identification of this tumour essential. Here, we report the case of a 38-year-old female patient with a tumour clinically resembling uterine leiomyoma. Microscopically, the spindled tumour cells were arranged in orderly intersecting fascicles, accompanied by a sparse infiltrate of inflammatory cells and a notable absence of myxoid matrix. Immunohistochemistry and molecular testing revealed an ALK::SYN3 fusion, suggesting the diagnosis of a uterine leiomyoma-like inflammatory myofibroblastic tumour (UL-like IMT). UL-like IMT is exceedingly rare and can easily be misdiagnosed as smooth-muscle tumours based solely on clinical manifestations and morphology. Therefore, it is recommended that the diagnosis be based on a combination of histopathological features, immunohistochemical markers, and genetic testing results to ensure a comprehensive and accurate assessment.
子宫炎性肌纤维母细胞瘤（IMT）是一种相对罕见的子宫间叶性肿瘤，复发率和转移率分别为25%和2%。由于IMT经常存在ALK基因重排，一些患者可能从酪氨酸激酶抑制剂治疗中获益，因此准确识别这种肿瘤至关重要。在此，我们报告一例38岁女性患者，其肿瘤临床上类似子宫平滑肌瘤。显微镜下，梭形肿瘤细胞呈有序交叉束状排列，伴有稀疏的炎性细胞浸润，且明显缺乏黏液样基质。免疫组织化学和分子检测显示ALK::SYN3融合，提示诊断为子宫平滑肌瘤样炎性肌纤维母细胞瘤（UL样IMT）。UL样IMT极为罕见，仅根据临床表现和形态很容易被误诊为平滑肌肿瘤。因此，建议基于组织病理学特征、免疫组化标志物和基因检测结果进行综合诊断，以确保全面准确的评估。
