原发性肝脏间质瘤并颈部淋巴结转移1例并文献复习
发布时间：2018-08-05 15:48
【摘要】：目的:探讨原发性肝脏间质瘤的诊断和治疗方法。方法:回顾分析1例原发性肝脏间质瘤患者的临床资料及文献复习。结果:女性患者1例,45岁,因肝脏占位6个月,介入术后4个月入院。无肝炎及肝硬化病史,AFP正常。术前检查均提示肝内占位。行复杂性肝癌切除+胆囊切除术。术中探查胃肠道及腹腔其余脏器未发现肿瘤。术中快速冷冻病检提示肿瘤内可见梭形细胞,不排除来自胃肠道间质瘤。术后病理报告:左肝胃肠外间质瘤,高危度(核分裂像约5个/50HPF)。免疫组化:瘤细胞CD117(+)、CD34(+)、DOG1(+)、Des(-)、S-100(-)、EMA小灶(+)、Ki-67约3%。PDGFRA基因12、18号外显子序列无突变。C-KIT基因13、17号外显子序列无突变。术后1个月余左颈部淋巴结肿大,予以穿刺活检见梭形细胞,轻度异型,结合病史,倾向于胃肠间质瘤转移。予以口服甲磺酸伊马替尼药物治疗后颈部淋巴结明显缩小,目前已随访18个月,未见肿瘤复发及转移。文献报道的17例肝脏原发性间质瘤患者,最小年龄17岁,最大年龄73岁,平均年龄48岁,男女比例为10:7;肿瘤直径最小的5 cm,最大的44 cm,平均直径17.8 cm,可单发或多发,大多无肝炎及肝硬化病史,AFP为阴性。CD117和CD34在间质瘤中阳性率分别为92.3%及66.7%。结论:原发性肝脏间质瘤是一种罕见的胃肠道外间质瘤,其诊断主要依靠组织病理及免疫组化结果,治疗以手术为主,辅以化疗,疗效可。
[Abstract]:Objective: to investigate the diagnosis and treatment of primary hepatic stromal tumors. Methods: the clinical data and literature review of a case of primary hepatic stromal tumor were retrospectively analyzed. Results: one female patient, aged 45 years, was admitted to hospital 4 months after interventional surgery because of liver occupying for 6 months. No history of hepatitis and cirrhosis. AFP was normal. Preoperative examination indicated intrahepatic space occupying. Complicated hepatectomy and cholecystectomy were performed. No tumor was found in the gastrointestinal tract and other viscera during intraoperative exploration. Rapid intraoperative cryopreservation showed that spindle cells could be seen in the tumor, and gastrointestinal stromal tumors were not excluded. Postoperative pathological report: left hepatic parenteral stromal tumor, high risk (mitosis like about 5 / 50 HPF). Immunohistochemistry: CD117 () CD34 () doG1 (-) s (-) EMA small focus () Ki-67 about 3%.PDGFRA gene 12, exon 18 no mutation. C-KIT gene, exon 17 has no mutation. The left cervical lymph nodes were enlarged more than one month after operation. Fusiform cells were seen by puncture biopsy, mild dysplasia, combined with the history of gastrointestinal stromal tumors, tend to metastasize gastrointestinal stromal tumors (GIST). The cervical lymph nodes were significantly reduced after oral treatment of imatinib mesylate, and no recurrence or metastasis was found after 18 months follow-up. Seventeen patients with primary hepatic stromal tumors reported in the literature, the youngest age is 17 years old, the maximum age is 73 years old, the average age is 48 years old, the ratio of male to female is 10: 7, the smallest tumor diameter is 5 cm, the largest is 44 cm, the average diameter is 17.8 cm. The positive rates of AFP-negative CD117 and CD34 in stromal tumors without history of hepatitis and cirrhosis were 92.3% and 66.7% respectively. Conclusion: primary hepatic stromal tumor is a rare gastrointestinal stromal tumor. The diagnosis of primary hepatic stromal tumor mainly depends on histopathological and immunohistochemical results.
【作者单位】： 湖南省郴州市第一人民医院肝胆外科;
【分类号】：R735.7
[Abstract]:Objective: to investigate the diagnosis and treatment of primary hepatic stromal tumors. Methods: the clinical data and literature review of a case of primary hepatic stromal tumor were retrospectively analyzed. Results: one female patient, aged 45 years, was admitted to hospital 4 months after interventional surgery because of liver occupying for 6 months. No history of hepatitis and cirrhosis. AFP was normal. Preoperative examination indicated intrahepatic space occupying. Complicated hepatectomy and cholecystectomy were performed. No tumor was found in the gastrointestinal tract and other viscera during intraoperative exploration. Rapid intraoperative cryopreservation showed that spindle cells could be seen in the tumor, and gastrointestinal stromal tumors were not excluded. Postoperative pathological report: left hepatic parenteral stromal tumor, high risk (mitosis like about 5 / 50 HPF). Immunohistochemistry: CD117 () CD34 () doG1 (-) s (-) EMA small focus () Ki-67 about 3%.PDGFRA gene 12, exon 18 no mutation. C-KIT gene, exon 17 has no mutation. The left cervical lymph nodes were enlarged more than one month after operation. Fusiform cells were seen by puncture biopsy, mild dysplasia, combined with the history of gastrointestinal stromal tumors, tend to metastasize gastrointestinal stromal tumors (GIST). The cervical lymph nodes were significantly reduced after oral treatment of imatinib mesylate, and no recurrence or metastasis was found after 18 months follow-up. Seventeen patients with primary hepatic stromal tumors reported in the literature, the youngest age is 17 years old, the maximum age is 73 years old, the average age is 48 years old, the ratio of male to female is 10: 7, the smallest tumor diameter is 5 cm, the largest is 44 cm, the average diameter is 17.8 cm. The positive rates of AFP-negative CD117 and CD34 in stromal tumors without history of hepatitis and cirrhosis were 92.3% and 66.7% respectively. Conclusion: primary hepatic stromal tumor is a rare gastrointestinal stromal tumor. The diagnosis of primary hepatic stromal tumor mainly depends on histopathological and immunohistochemical results.
【作者单位】： 湖南省郴州市第一人民医院肝胆外科;
【分类号】：R735.7
【参考文献】
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