Yang Linke, Li Pei, Liu Runze, Feng Baomin, Mao Huiqing, Tang Xiaoyong, Yang Guangjian
Department of Pathology, Shandong Cancer Hospital and Institute, Shandong First Medical University, Shandong Academy of Medical Sciences, No. 440 Jiyan Road, Jinan, 250117, People's Republic of China.
Department of Respiratory Medical Oncology, Shandong Cancer Hospital and Institute, Shandong First Medical University, Shandong Academy of Medical Sciences, No. 440 Jiyan Road, Jinan, 250117, Jinan, People's Republic of China.
Discov Oncol. 2024 Sep 27;15(1):484. doi: 10.1007/s12672-024-01375-5.
Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare subtype of inflammatory myofibroblastic tumor, characterized to be an aggressive disease with high frequency of ALK rearrangement, rapid recurrence, and poor prognosis. Primary EIMS of thoracic origin is rarely observed. Herein, we described a case of 28-year-old female developed primary EIMS in the anterior mediastinum with hepatic metastasis. The EIMS displayed sheet-like growth of epithelioid and spindle cells with enlarged nuclei, abundant and eosinophilic cytoplasm, and infiltration of inflammatory cells. Immunohistochemical staining revealed positive expression of ALK in the nuclear membrane, and ALK rearrangement was identified by polymerase chain reaction assay. Alectinib showed partial response, and achieved a meaningful survival benefit for four months. Based on this case report and literature review, ALK inhibitor reveals promising activity on the rare but aggressive EIMS. Awareness of EIMS in thoracic disease and its clinicopathological features is essential to avoid erroneous diagnosis.
上皮样炎性肌纤维母细胞肉瘤（EIMS）是炎性肌纤维母细胞瘤的一种罕见亚型，其特征为侵袭性疾病，ALK重排频率高、复发快且预后差。原发于胸部的EIMS很少见。在此，我们描述了一例28岁女性，其前纵隔发生原发性EIMS并伴有肝转移。EIMS表现为上皮样细胞和梭形细胞呈片状生长，细胞核增大，细胞质丰富且嗜酸性，并有炎性细胞浸润。免疫组织化学染色显示ALK在核膜呈阳性表达，通过聚合酶链反应检测确定存在ALK重排。阿来替尼显示出部分缓解，并使患者获得了四个月的有意义的生存获益。基于本病例报告和文献复习，ALK抑制剂对这种罕见但侵袭性强的EIMS显示出有前景的活性。了解胸部疾病中的EIMS及其临床病理特征对于避免误诊至关重要。
