下腔静脉内膜肉瘤（IVC）是一种罕见的软组织肉瘤，早期无典型症状和特异性影像学特征，缺乏规范的治疗和方法。一名54岁的女性患者就诊于奉化术后因主动咳嗽、咯血被区人民医院转诊至我院。病理诊断为下腔静脉内膜肉瘤并发多发肺内转移。胸部CT显示左肺恶性肿瘤并伴有肺内多发转移；上腹部增强CT显示下腔静脉癌栓延伸至右心房及双侧肾静脉。此外，苏木精和伊红染色提示静脉内膜肉瘤。免疫组化染色显示PD-L1、Ki-67、CD31、Desmin和ERG阳性。患者最初接受GT化疗（吉西他滨注射液  多西紫杉醇）。然后根据基因检测结果（TP53基因突变）加用免疫治疗（替雷利珠单抗）。治疗后病情稳定。鉴于IVC内膜肉瘤患者缺乏特征性临床表现，影像学检查结合免疫组化该指标有助于IVC内膜肉瘤的诊断。此外，替雷利珠单抗和 GT 化疗的联合治疗对于 PD-L1 表达阳性且 TP53 突变的患者是可行的。版权所有 © 2024 作者。由 Wolters Kluwer Health, Inc. 出版

Intimal sarcoma of inferior vena cava (IVC) is a rare soft tissue sarcoma with no typical symptoms and specific imaging features in the early stage, and there is a lack of standardized treatment and methods.A 54-year-old female patient presented to Fenghua District People's Hospital with a post-active cough and hemoptysis and was subsequently referred to our hospital.The patient was pathologically diagnosed as intimal sarcoma of IVC complicating multiple intrapulmonary metastases. Chest CT revealed left lung malignant tumor with multiple intrapulmonary metastases; while enhanced upper abdominal CT showed cancer embolus of IVC with extension to right atrium and bilateral renal veins. Besides, hematoxylin and eosin staining suggested intimal sarcoma of veins. Immunohistochemical staining showed positivity for PD-L1, Ki-67, CD31, Desmin and ERG.The patient initially received GT chemotherapy (gemcitabine injection + docetaxel). Then, immunotherapy (tislelizumab) was added based on the results of genetic testing (TP53 gene mutation).The disease was stabilized after receiving the treatment.Given the lack of characteristic clinical manifestations in patients with intimal sarcoma of IVC, imaging examination combined with immunohistochemical index were helpful for diagnosis of intimal sarcoma of IVC. Furthermore, the combination of tislelizumab and GT chemotherapy was feasible in such patients with positive PD-L1 expression and TP53 mutation.Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.