原發(fā)性肝臟間質(zhì)瘤并頸部淋巴結(jié)轉(zhuǎn)移1例并文獻(xiàn)復(fù)習(xí)
發(fā)布時間：2018-08-05 15:48
【摘要】：目的:探討原發(fā)性肝臟間質(zhì)瘤的診斷和治療方法。方法:回顧分析1例原發(fā)性肝臟間質(zhì)瘤患者的臨床資料及文獻(xiàn)復(fù)習(xí)。結(jié)果:女性患者1例,45歲,因肝臟占位6個月,介入術(shù)后4個月入院。無肝炎及肝硬化病史,AFP正常。術(shù)前檢查均提示肝內(nèi)占位。行復(fù)雜性肝癌切除+膽囊切除術(shù)。術(shù)中探查胃腸道及腹腔其余臟器未發(fā)現(xiàn)腫瘤。術(shù)中快速冷凍病檢提示腫瘤內(nèi)可見梭形細(xì)胞,不排除來自胃腸道間質(zhì)瘤。術(shù)后病理報告:左肝胃腸外間質(zhì)瘤,高危度(核分裂像約5個/50HPF)。免疫組化:瘤細(xì)胞CD117(+)、CD34(+)、DOG1(+)、Des(-)、S-100(-)、EMA小灶(+)、Ki-67約3%。PDGFRA基因12、18號外顯子序列無突變。C-KIT基因13、17號外顯子序列無突變。術(shù)后1個月余左頸部淋巴結(jié)腫大,予以穿刺活檢見梭形細(xì)胞,輕度異型,結(jié)合病史,傾向于胃腸間質(zhì)瘤轉(zhuǎn)移。予以口服甲磺酸伊馬替尼藥物治療后頸部淋巴結(jié)明顯縮小,目前已隨訪18個月,未見腫瘤復(fù)發(fā)及轉(zhuǎn)移。文獻(xiàn)報道的17例肝臟原發(fā)性間質(zhì)瘤患者,最小年齡17歲,最大年齡73歲,平均年齡48歲,男女比例為10:7;腫瘤直徑最小的5 cm,最大的44 cm,平均直徑17.8 cm,可單發(fā)或多發(fā),大多無肝炎及肝硬化病史,AFP為陰性。CD117和CD34在間質(zhì)瘤中陽性率分別為92.3%及66.7%。結(jié)論:原發(fā)性肝臟間質(zhì)瘤是一種罕見的胃腸道外間質(zhì)瘤,其診斷主要依靠組織病理及免疫組化結(jié)果,治療以手術(shù)為主,輔以化療,療效可。
[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.
【作者單位】： 湖南省郴州市第一人民醫(yī)院肝膽外科;
【分類號】：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.
【作者單位】： 湖南省郴州市第一人民醫(yī)院肝膽外科;
【分類號】：R735.7
【參考文獻(xiàn)】
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