背景:下腔静脉内膜肉瘤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。
方法:一名54岁女性患者到奉化区人民医院就诊,术后出现咳嗽和咯血,随后转诊至我院。
方法:患者经病理诊断为IVC内膜肉瘤合并肺内多发转移。胸部CT提示左肺恶性肿瘤伴多发肺内转移;上腹部增强CT提示IVC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。免疫组织化学染色显示PD-L1、Ki-67、CD31、Desmin和ERG阳性。
方法:患者最初接受GT化疗(吉西他滨注射液+多西他赛)。然后,根据基因检测结果(TP53基因突变),我们加入了免疫治疗(tislelizumab).
结果:接受治疗后病情稳定。
结论: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指标有助于诊断IVC内膜肉瘤。此外,在这类PD-L1表达阳性且TP53突变的患者中,tislelizumab和GT联合化疗是可行的.
BACKGROUND: 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.
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.
METHODS: 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.
METHODS: The patient initially received GT chemotherapy (gemcitabine injection + docetaxel). Then, immunotherapy (tislelizumab) was added based on the results of genetic testing (TP53 gene mutation).
RESULTS: The disease was stabilized after receiving the treatment.
CONCLUSIONS: 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.