Clinical Treatment and Prognosis of External Gastrointestinal Stromal Tumor_West China Medical Journal

Authors：

YANG Hongxin , CHEN Xiufeng ,  ZHANG Bo , CHEN Haining , CHEN Zhixin , CHEN Jiaping

Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding?author：

ZHANG Bo, Email: hxyang2008@sina.com

Keywords：

胃腸道外間質瘤; 手術治療; 甲磺酸伊馬替尼

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目的　探討胃腸道外間質瘤（EGIST）的臨床表現、外科治療及預后。方法　回顧性分析2004年1月－2010年6月收治的35例EGIST患者的臨床資料。男26例，女9例；年齡33～78歲，平均56歲。病程5 d～8個月，平均2個月。臨床表現主要有腹部不適、腹痛及腹部包塊等。均在術前行CT或腹部增強CT等檢查發現病灶，其中位于系膜16例，網膜15例，腹膜后4例。35例均行手術治療。結果　術后均由病理學檢查及免疫組織化學檢測確診，腫瘤標本鏡下均以梭形細胞為主；極低危險、低危險、中危險、高危險患者分別為0、3、0、32例。免疫組織化學檢測示酪氨酸激酶受體（CD117）、DOG-1、骨髓干細胞抗原（CD34）、酸性鈣結合蛋白、平滑肌肌動蛋白、結蛋白陽性率分別為91.4%（32/35）、100%（3/3）、71.4%（25/35）、8.6%（3/35）、22.9%（8/35）、0%（0/35）。15例患者均獲隨訪，時間19～96個月，平均46個月。8例出現進展，7例病情穩定。結論　EGIST發現時往往體積較大，預后較差，手術切除是首選治療手段，甲磺酸伊馬替尼對其具有較好的治療效果。

Citation： YANG Hongxin,CHEN Xiufeng,ZHANG Bo,CHEN Haining,CHEN Zhixin,CHEN Jiaping. Clinical Treatment and Prognosis of External Gastrointestinal Stromal Tumor. West China Medical Journal, 2012, 27(9): 1342-1345. doi: Copy

1.	Mazur MT, Clark HB．Gastric stromal tumors. Reappraisal of histogenesis[J]. Am J Surg Pathol, 1983, 7(6): 507-519.
2.	Joensuu H. Risk stratification of patients diagnosed with gastrointestinal stromal tumor[J]. Hum Pathol, 2008, 39(10): 1411-1419.
3.	中國胃腸道間質瘤專家組. 中國胃腸道間質瘤診斷治療共識（2008年版）[J]. 臨床腫瘤學雜志, 2009, 14(8): 746-754.
4.	Kindblom LG, Remotti HE, Aldenborg F, et al. Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal[J]. Am J Pathol, 1998, 152(5): 1259-1269.
5.	Kim KH, Nelson SD, Kim DH, et al. Diagnostic relevance of overexpressions of PKC-θ and DOG-1 and KIT/PDGFRA gene mutations in extragastrointestinal stromal tumors: a Korean six-centers study of 28 cases[J]. Anticancer Res, 2012, 32(3): 923-937.
6.	Barros A, Linhares E, Valadão M, et al. Extragastrointestinal stromal tumors (EGIST): a series of case reports[J]. Hepatogastroenterol, 2011, 58(107-108): 865-868.
7.	Emory TS, Sobin LH, Lukes L, et al. Prognosis of gastrointestinal smooth-muscle (stroma1) tumors：dependence on anatomic site[J]．Am J Surg Pathol, 1999, 23(1): 82-87.
8.	呂晉生, 喬英．胃腸道外間質瘤的CT表現和臨床病理分析[J].山西醫藥雜志(上半月), 2011, 40(1): 52-53.
9.	Peitsidis P, Zarganis P, Trichia H, et al. Extragastrointestinal stromal tumor mimicking a uterine tumor. A rare clinical entity[J]. Int J Gynecol Cancer, 2008, 18(5): 1115-1118.
10.	Miettinen M, Monihan JM, Sarlomo-Rikala M, et al. Gastrointestinal stromal tumors/smooth muscle tumors (GISTs) primary in the omentum and mesentery clinicopathologic and immunohistochemical study of 26 cases[J]. Am J Surg Pathol, 1999, 23(9): 1109-1118.
11.	賀敬紅, 李健丁, 張瑞平. 胃腸道外間質瘤的影像診斷新進展[J].世界華人消化雜志, 2010, 18(1): 54-57.
12.	Kim HC, Lee JM, Kim SH, et al. Primary gastrointestinal stromal tumors in the omentum and mesentery: CT findings and pathologic correlations [J]．AJR Am J Roentgenol, 2004, 182(6): 1463-1467.
13.	Lasota J, Miettinen M. Clinical significance of oncogenic KIT and PDGFRA mutations in gastrointestinal stromal tumors[J]. Histopathology, 2008, 53(3): 245-266.
14.	李廣麗, 陳繼紅, 劉校瑾.腹腔間質瘤17例臨床分析[J].河南外科學雜志, 2011, 17(5): 37-38.
15.	董德武, 郭素芬, 安錦丹. DOG1和PDGFRA在胃腸道間質瘤中的表達及意義[J]. 牡丹江醫學院學報, 2010, 31(6):36-38.
16.	Kang YK, Kim KM, Sohn T, et al. Clinical practice guideline for accurate diagnosis and effective treatment of gastrointestinal stromal tumor in Korea[J]. J Korean Med Sci, 2010, 25(11):1543-1552.
17.	秦新裕, 劉鳳林．胃腸道間質瘤的研究進展與展望[J]. 中華普通外科雜志, 2007, 22(8): 561-563.
18.	Demetri GD, Von Mehrea M, Blank CD, et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors[J]. N Eng J Med, 2002, 347(7): 472-480.
19.	Nishida T, Shirao K, Sawaki A, et al. Efficacy and safety profile of imatinib mesylate (ST1571) in Japanese patients with advanced gastrointestinal stromal tumors: a phaseⅡ study (STI571B1202)[J]. Int J Clin Oncol, 2008, 13(3): 244-251.
20.	Li J, Gong J, Wu AW, et al. Post-operative imatinib in patients with intermediate or high risk gastrointestinal stromal tumor[J]. Eur J Surg Oncol, 2011, 37(4): 319-324.
21.	Machlenkin S, Pinsk I, Tulchinsky H, et al. The effect of neoadjuvant Imatinib therapy on outcome and survival after rectal gastrointestinal stromal tumour[J]. Colorectal Dis, 2011, 13(10): 1110-1115.
22.	Nilsson B, Sjölund K, Kindblom LG, et al. Adjuvant imatinib treatment improves recurrence-free survival in patients with high-risk gastrointestinal stromal tumours (GIST)[J]. Br J Cancer, 2007, 96（11）：1656-1658.
23.	Reith JD, Goldblum JR, Lyles RH, et al. Extragastrointestinal (soft tissue) stromal tumors: an analysis of 48 cases with emphasis on histologic predictors of outcome[J]. Mod Pathol, 2000, 13(5): 577-585.

1. 　Mazur MT, Clark HB．Gastric stromal tumors. Reappraisal of histogenesis[J]. Am J Surg Pathol, 1983, 7(6): 507-519.
2. 　Joensuu H. Risk stratification of patients diagnosed with gastrointestinal stromal tumor[J]. Hum Pathol, 2008, 39(10): 1411-1419.
3. 　中國胃腸道間質瘤專家組. 中國胃腸道間質瘤診斷治療共識（2008年版）[J]. 臨床腫瘤學雜志, 2009, 14(8): 746-754.
4. 　Kindblom LG, Remotti HE, Aldenborg F, et al. Gastrointestinal pacemaker cell tumor (GIPACT): gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal[J]. Am J Pathol, 1998, 152(5): 1259-1269.
5. 　Kim KH, Nelson SD, Kim DH, et al. Diagnostic relevance of overexpressions of PKC-θ and DOG-1 and KIT/PDGFRA gene mutations in extragastrointestinal stromal tumors: a Korean six-centers study of 28 cases[J]. Anticancer Res, 2012, 32(3): 923-937.
6. 　Barros A, Linhares E, Valadão M, et al. Extragastrointestinal stromal tumors (EGIST): a series of case reports[J]. Hepatogastroenterol, 2011, 58(107-108): 865-868.
7. 　Emory TS, Sobin LH, Lukes L, et al. Prognosis of gastrointestinal smooth-muscle (stroma1) tumors：dependence on anatomic site[J]．Am J Surg Pathol, 1999, 23(1): 82-87.
8. 　呂晉生, 喬英．胃腸道外間質瘤的CT表現和臨床病理分析[J].山西醫藥雜志(上半月), 2011, 40(1): 52-53.
9. 　Peitsidis P, Zarganis P, Trichia H, et al. Extragastrointestinal stromal tumor mimicking a uterine tumor. A rare clinical entity[J]. Int J Gynecol Cancer, 2008, 18(5): 1115-1118.
10. 　Miettinen M, Monihan JM, Sarlomo-Rikala M, et al. Gastrointestinal stromal tumors/smooth muscle tumors (GISTs) primary in the omentum and mesentery clinicopathologic and immunohistochemical study of 26 cases[J]. Am J Surg Pathol, 1999, 23(9): 1109-1118.
11. 　賀敬紅, 李健丁, 張瑞平. 胃腸道外間質瘤的影像診斷新進展[J].世界華人消化雜志, 2010, 18(1): 54-57.
12. 　Kim HC, Lee JM, Kim SH, et al. Primary gastrointestinal stromal tumors in the omentum and mesentery: CT findings and pathologic correlations [J]．AJR Am J Roentgenol, 2004, 182(6): 1463-1467.
13. 　Lasota J, Miettinen M. Clinical significance of oncogenic KIT and PDGFRA mutations in gastrointestinal stromal tumors[J]. Histopathology, 2008, 53(3): 245-266.
14. 　李廣麗, 陳繼紅, 劉校瑾.腹腔間質瘤17例臨床分析[J].河南外科學雜志, 2011, 17(5): 37-38.
15. 　董德武, 郭素芬, 安錦丹. DOG1和PDGFRA在胃腸道間質瘤中的表達及意義[J]. 牡丹江醫學院學報, 2010, 31(6):36-38.
16. 　Kang YK, Kim KM, Sohn T, et al. Clinical practice guideline for accurate diagnosis and effective treatment of gastrointestinal stromal tumor in Korea[J]. J Korean Med Sci, 2010, 25(11):1543-1552.
17. 　秦新裕, 劉鳳林．胃腸道間質瘤的研究進展與展望[J]. 中華普通外科雜志, 2007, 22(8): 561-563.
18. 　Demetri GD, Von Mehrea M, Blank CD, et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors[J]. N Eng J Med, 2002, 347(7): 472-480.
19. 　Nishida T, Shirao K, Sawaki A, et al. Efficacy and safety profile of imatinib mesylate (ST1571) in Japanese patients with advanced gastrointestinal stromal tumors: a phaseⅡ study (STI571B1202)[J]. Int J Clin Oncol, 2008, 13(3): 244-251.
20. 　Li J, Gong J, Wu AW, et al. Post-operative imatinib in patients with intermediate or high risk gastrointestinal stromal tumor[J]. Eur J Surg Oncol, 2011, 37(4): 319-324.
21. 　Machlenkin S, Pinsk I, Tulchinsky H, et al. The effect of neoadjuvant Imatinib therapy on outcome and survival after rectal gastrointestinal stromal tumour[J]. Colorectal Dis, 2011, 13(10): 1110-1115.
22. 　Nilsson B, Sjölund K, Kindblom LG, et al. Adjuvant imatinib treatment improves recurrence-free survival in patients with high-risk gastrointestinal stromal tumours (GIST)[J]. Br J Cancer, 2007, 96（11）：1656-1658.
23. 　Reith JD, Goldblum JR, Lyles RH, et al. Extragastrointestinal (soft tissue) stromal tumors: an analysis of 48 cases with emphasis on histologic predictors of outcome[J]. Mod Pathol, 2000, 13(5): 577-585.

West China Medical Journal

Clinical Treatment and Prognosis of External Gastrointestinal Stromal Tumor

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