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肝脏滑膜肉瘤1例并文献复习

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目的 报道1例肝脏滑膜肉瘤病例并进行文献复习,以提高对该病的认识。方法 回顾性分析河南大学第一附属医院收治的1例肝脏滑膜肉瘤患者的病例资料,并检索中国知网、万方数据知识服务平台及PubMed数据库中相关病例报道,时间截至2022年7月,复习相关文献,总结其影像学特征、病理特征及相关治疗和预后。结果 本例患者,女,71岁,因腹痛入院,计算机断层扫描(CT)检查示肝右叶及尾状叶混杂密度肿块,较大截面大小115 mm×87 mm,增强扫描肿块呈持续不均匀强化,考虑肝脏恶性肿瘤并肝、肺多发转移。行超声引导下穿刺活检,病理镜下见瘤细胞异型性明显,部分呈梭形。免疫组化示波形蛋白(VIM)、上皮膜抗原(EMA)、组蛋白H3赖氨酸27-三甲基化(H3K27Me3)阳性,广谱细胞角蛋白(CK-pan)、神经特异性蛋白(S-100)阴性,病理诊断为滑膜肉瘤。患者未行后续治疗,出院后失访。截至2022年7月,国内外共报道12例肝脏滑膜肉瘤病例,临床表现为腹痛或腹胀,病变多位于肝右叶,通常体积较大,密度不均,CT或磁共振成像(MRI)增强扫描呈不均匀强化。病理学检查镜下见梭形细胞,免疫组化示VIM、EMA、H3K27Me、B细胞淋巴瘤白血病-2蛋白(BCL-2)及分裂蛋白1转导蛋白样增强子(TLE1)等阳性。基因检测示SS18-SSX融合基因或SS18基因分离。11例患者行手术治疗,5例术后辅助化疗,随访期内4例复发或转移。结论 肝脏滑膜肉瘤是一种罕见的肝脏恶性肿瘤,临床征象及影像表现不具特异性,确诊需依赖病理,免疫组化及基因检测可辅助诊断。目前主要治疗方案为手术,可予辅助化疗等综合治疗,患者预后不佳。
Synovial sarcoma of the liver:a case report and literature review
Objective To report a case of synovial sarcoma of the liver and review the literature for improving the understanding of the disease.Methods The clinical data of a patient with liver synovial sarcoma admitted to the First Affiliated Hospital of Henan University were analyzed retrospectively.The imaging,pathological features,treatment and prognosis of this disease were summarized by searching the database(CNKI,Wanfang Data,PubMed,untill July 2022)and the literature results analyzed comprehensively.Results The patient was a 71-year-old female who was admitted to the hospital due to abdominal pain.Computed tomography(CT)scan showed a mass with mixed density in the right lobe and caudate lobe of the liver.The large cross section size was about 115 mm×87 mm and the mass showed continuous heterogeneous enhancement,being considered as malignant hepatic tumors with multiple metastasis of the liver and lung.Ultrasound-guided needle biopsy was performed,and microscopy showed the tumor cells were obvious atypical,and some were spindle-shaped.Immunohistochemistry showed that the patient was positive for vimentin(VIM),epithelial membrane antigen(EMA),methylation of histone at lysine 27(H3K27Me3),and negative for pan cytokeratin(CK-pan)and S-100,and pathological diagnosis of synovial sarcoma was made.The patient did not undergo subsequent treatment and was lost to follow-up after discharge.A total of 12 cases of hepatic synovial sarcoma were reported after searching the database.The clinical manifestations were abdominal pain or distention.The lesions were mostly located in the right lobe of the liver,usually large,heterogeneous density,and heterogeneous enhancement on enhanced CT or magnetic resonance imaging(MRI).Spindle-shaped cells were found at histopathologic examination.Immunohistochemistry showed the patient was positive for VIM,EMA,H3K27Me,B-cell leukemia/lymphoma-2(BCL-2)and transducer-like enhancer of split 1(TLE1).SS18-SSX fusion gene or SS18 gene isolation were detected.Eleven patients received surgical treatment,5 received adjuvant chemotherapy,and 4 had recurrence or metastasis during the follow-up period.Conclusions Synovial sarcoma of the liver is a rare malignant tumor of the liver.The clinical and imaging features are not specific.The diagnosis depends on pathology.At present,the main treatment is surgery,and comprehensive treatment such as adjuvant chemotherapy can be performed.The prognosis of the patient is poor.

liversynovial sarcomadiagnosistreatment

王晓蕾、周青、李琰、李嘉欣、郭帅康、徐淑菲

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河南大学第一附属医院放射科,河南开封 475000

肝脏 滑膜肉瘤 诊断 治疗

2024

解放军医学杂志
人民军医出版社

解放军医学杂志

CSTPCD北大核心
影响因子:1.644
ISSN:0577-7402
年,卷(期):2024.49(2)
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