• Haihong Liao, Yong Fang, Da Li, Yuefen Pan, Zhongfeng Niu, Tianhong Fu, Zhuoxuan Wu, Jin Sheng, Yong Dong, Shuwen Han, Quan Qi, and Yulong Liu.
• Department of Medical Oncology, Huzhou Central Hospital, Huzhou, Zhejiang, China.
• Medicine (Baltimore). 2024 May 24; 103 (21): e38056e38056.

RationaleIntimal 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.Patient ConcernsA 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.DiagnosesThe 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.InterventionsThe patient initially received GT chemotherapy (gemcitabine injection + docetaxel). Then, immunotherapy (tislelizumab) was added based on the results of genetic testing (TP53 gene mutation).OutcomesThe disease was stabilized after receiving the treatment.LessonsGiven 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.

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