盆腔上皮样炎性肌纤维母细胞肉瘤合并腹膜转移1例  

Pelvic epithelioid inflammatory myofibroblastic sarcoma complicated with peritoneal metastasis:a case report

作　　者：黄文鹏 宋乐乐 肖晓燕 李莉明[2] 邱永康 高剑波[2] 康磊[1] Huang Wenpeng;Song Lele;Xiao Xiaoyan;Li Liming;Qiu Yongkang;Gao Jianbo;Kang Lei(Department of Nuclear Medicine,Peking University First Hospital,Beijing 100034,China;Department of Radiology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]北京大学第一医院核医学科,北京100034 [2]郑州大学第一附属医院放射科,郑州450052

出　　处：《中国医师杂志》2025年第3期357-360,366,共5页Journal of Chinese Physician

基　　金：国家自然科学基金(82171970、81871385);北京市杰出青年科学基金(JQ21025);北京大学第一医院跨学科交叉研究专项(2023IR17)。

摘　　要：目的分析1例女性盆腔上皮样炎性肌纤维母细胞肉瘤(EIMS)合并腹膜转移患者的临床特点、影像特征及诊疗过程,以提高临床对该病的认识水平和诊断能力,避免误诊和漏诊。方法回顾性分析郑州大学第一附属医院收治的1例女性盆腔EIMS合并腹膜转移患者的临床资料。结果该患者三合诊触及盆腔内大小约8.0 cm×9.0 cm的不规则实性包块。实验室检查显示人附睾蛋白4(HE4)154.00 pmol/L。超声表现为盆腔深部内多个低回声团块,边界不清,内回声不均,彩色多普勒血流显像(CDFI)示其内血流信号丰富。增强CT显示病灶呈不均匀明显强化。病理检查显示黏液间质背景中炎症细胞浸润,肿瘤细胞呈圆形、上皮样,细胞核大、深染、核仁明显;免疫组化显示间变性淋巴瘤激酶(ALK)阳性,分子病理学荧光原位杂交检测结果显示ALK基因扩增(阳性)。结合病理、免疫组化和基因检测结果诊断为EIMS。结论当盆腔出现单发或多发实性或囊实性结节或肿块,强化明显,呈浸润性生长并伴有腹膜种植转移时,应想到EIMS的可能,根据病理学表现、免疫组织化学及基因检测结果可做出正确诊断。ObjectiveTo analyze the clinical features,imaging features and diagnosis and treatment process of a female pelvic epithelioid inflammatory myofibroblastic sarcoma(EIMS)with peritoneal metastasis,so as to improve the clinical understanding and diagnostic ability of the disease and avoid misdiagnosis and missed diagnosis.MethodsThe clinical data of a female patient with pelvic EIMS combined with peritoneal metastasis in the First Affiliated Hospital of Zhengzhou University were retrospectively analyzed.ResultsThe triad examination of the patient involved an irregular solid mass in the pelvic cavity about 8.0 cm×9.0 cm in size.Laboratory examination revealed human epididymal protein 4(HE4)154.00 pmol/L.The ultrasonography showed multiple low-echo masses in deep pelvic cavity with unclear boundary and uneven internal echo,and color doppler flow imaging(CDFI)showed abundant internal blood flow signals.Enhanced CT showed uneven and obvious enhancement of the lesion.Pathological examination showed the infiltration of inflammatory cells in the mucous interstitial background.The tumor cells were round and epithelioid,with large nuclei,deep staining and obvious nucleolus.Immunohistochemistry showed anaplastic lymphoma kinase(ALK)positive,and molecular pathology fluorescence in situ hybridization showed ALK gene amplification(positive).Combined with pathological,immunohistochemical and genetic tests,EIMS was diagnosed.ConclusionsEIMS should be considered when there are single or multiple solid or cystic nodules or masses in the pelvic cavity with obvious enhancement,invasive growth and peritoneal implantation metastasis,and the correct diagnosis can be made according to the pathological findings,immunohistochemistry and genetic test results.

关 键 词：盆腔肿瘤 上皮样炎性肌纤维母细胞肉瘤 体层摄影术 X线计算机 

分 类 号：R737.3[医药卫生—肿瘤]