摘要
目的探讨原发性肝平滑肌肉瘤的诊治方法。方法回顾性分析2017年10月在西安交通大学第一附属医院诊治的1例原发性肝平滑肌肉瘤患者临床资料。患者女,49岁,因“超声检查发现肝占位性病变”入院。超声检查示肝脏尾叶低回声区。腹部CT检查示肝右叶及尾状叶可见多发结节状及团块状低密度影。肝穿刺活检、免疫组化染色确诊原发性肝平滑肌肉瘤。治疗方案为TACE联合化疗。结果患者于2017年12月14日行TACE治疗,术后给予6个疗程的辅助化疗(吉西他滨+多西他赛)。2019年4月16日复查发现病变较前略扩大,化疗方案改为吉西他滨+白蛋白结合型紫杉醇,化疗3个周期。2020年5月20日复查提示病灶明显增大,再次行TACE治疗,术后患者恢复良好。截至2020年7月1日,患者生存质量良好,随访未见肿瘤复发。检索2000~2020年Pubmed共收录15例成年原发性肝平滑肌肉瘤患者,其中男6例,女9例;年龄25~77岁,中位年龄54岁。初发症状多表现为腹痛伴不适,肿瘤多发于右叶。实验室检查和影像学缺乏特异性表现。病理学检查示梭形组织细胞聚集呈束状排列;免疫组化染色示组织细胞SMA、desmin、vimentin阳性,S-100、细胞角蛋白阴性。手术切除9例,化疗2例,其中1例手术后联合化疗,肝移植术1例,TACE 2例,保守治疗2例。随访3~82个月,失访2例,死亡6例,存活7例。结论原发性肝平滑肌肉瘤是一种罕见疾病,病理学检查是确诊的唯一有效方式,手术切除可获得较好的疗效。
Objective To investigate the diagnosis and treatments of primary hepatic leiomyosarcoma(PHL).Methods Clinical data of 1 patient with PHL who was diagnosed and treated in the First Affiliated Hospital of Xi'an Jiaotong University in October 2017 were analyzed retrospectively.The female patient,aged 49 years,was admitted to our hospital due to"space-occupying lesions of the liver detected by ultrasound examination".A hypoechoic region in the caudate lobe of liver was found by ultrasound examination.Multiple nodule-like and lumpy low-density shadows in the right lobe and caudate lobe were observed by abdominal CT scan.The diagnosis of PHL was confirmed by liver biopsy and immunohistochemical staining.TACE combined with chemotherapy was adopted.Results The patient received TACE on December 14,2017,and was given with 6 cycles of adjuvant chemotherapy(gemcitabine+docetaxel)after TACE.The lesion was found slightly enlarged in the reexamination on April 16,2019 and the chemotherapy regimen was modified to gemcitabine+albumin-bound paclitaxel for 3 cycles.On May 20,2020,the reexamination found that the lesion was obviously enlarged.After the secondary TACE,the patient recovered well.As of July 1,2020,the patient had lived a high quality of life,and no tumor recurrence was found during postoperative follow-up.Clinical data of 15 adult patients with PHL were retrieved from PubMed database between 2000 and 2020.6 cases were male and 9 female,aged from 25 to 77 years,with a median age of 54 years.The primary symptoms were mainly abdominal pain and discomfort,and the tumors were mainly located in the right lobe.No specific manifestations was observed in laboratory and imaging examination.Pathological examination showed that spindle-shaped tissue cells were arranged in bundles.Immunohistochemical staining found positive results for SMA,desmin and vimentin,whereas negative results for S-100 and cytokeratin in the tissue cells.Surgical resection was performed in 9 cases,chemotherapy in 2,including 1 of surgical resection combined with chemotherapy,liver transplantation in 1,TACE in 2 and conservative treatments in 2,respectively.During 3-82 month follow-up,2 cases were lost to follow-up,6 cases died and 7 survived.Conclusions PHL is a rare disease.Pathological examination is the only effective approach to confirm the diagnosis.Surgical resection can achieve relatively better clinical efficacy.
作者
武洋
苗润晨
张兴
张月浪
马小华
刘昌
Wu Yang;Miao Runchen;Zhang Xing;Zhang Yuelang;Ma Xiaohua;Liu Chang(Department of Hepatobiliary Surgery,the First Affiliated Hospital of Xi'an Jiaotong University,Xi'an 710061,China;Department of Imaging,the First Affiliated Hospital of Xi'an Jiaotong University,Xi'an 710061,China)
出处
《中华肝脏外科手术学电子杂志》
CAS
2021年第6期618-621,共4页
Chinese Journal of Hepatic Surgery(Electronic Edition)
关键词
肝肿瘤
平滑肌肉瘤
诊断
治疗
Liver neoplasms
Leiomyosarcoma
Diagnosis
Treatment
作者简介
通信作者:刘昌,Email:eyrechang@126.com。
