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GIST Manifesting as a Retroperitoneal Tumor: Clinicopathologic Immunohistochemical, and Molecular Genetic Study of 112 Cases.

表现为腹膜后肿块的胃肠道间质肿瘤:112例临床病理、免疫组织化学和分子遗传学研究

Miettinen M,Felisiak-Golabek A,Wang Z,Inaguma S,Lasota J

Abstract

Most gastrointestinal stromal tumors (GISTs) occur in the tubular gastrointestinal (GI) tract, but some present apparently outside the GI tract. In this study, we analyzed 112 GISTs located in the retroperitoneum. These tumors occurred in 55 women and 57 men with a median age of 65 years (range: 21 to 89 y). On the basis of clinically or histologically detected connections to GI tract, 15 tumors were considered likely of gastric, 9 duodenal, and 13 of small intestinal origin. The remaining cases were categorized by location as peripancreatic (n=25), pelvic (n=11), mesenteric (n=4), and of unspecified/miscellaneous sites (n=35). The tumors varied in size 3 to 35 cm (median, 15 cm) and by mitotic rate per 5 mm, 0 to >100 (median, 10). Histologically the tumors apparently arising outside the GI tract had features of intestinal (n=41) and gastric GISTs (n=25); 9 cases had indeterminate histology. The histologic variants included spindled, epithelioid, vacuolated, nested, and myxoid potentially simulating other tumors such as liposarcoma and solitary fibrous tumor. Most GISTs were KIT-positive (106/112 cases), and the remaining 6 tumors were DOG1/Ano1-positive. Five cases showed focal nuclear positivity for MDM2. KIT mutations were detected in 42/59 cases, and PDGFRA mutations in 4/16 KIT wild-type and 3/5 of the KIT-negative tumors analyzed. One pelvic retroperitoneal GIST was succinate dehydrogenase deficient. All 79 patients were dead at last follow-up with a median survival of 14 months, with few survivals >5 years. Only operable versus inoperable tumor was a statistically favorable factor in univariate analysis (P<0.01). In multivariate analysis, mitotic rate >50/5 mm was significant for a shorter survival (hazard ratio, 5.25; 95% confidence interval, 1.65-16.8; P<0.01). Histologic and clinicopathologic similarity of extragastrointestinal retroperitoneal GISTs with GISTs of GI tract suggests their GI tract origin. Potentially overlapping features between GIST and other retroperitoneal tumors necessitate use of multiple diagnostic markers and molecular genetic studies.

摘要

大部分胃肠道间质肿瘤(GISTs)发生在胃肠道(GI),但有一部分是明确发生在胃肠道外的。本文中,我们分析了112例位于腹膜后的GISTs。这些病例中有55名女性和57名男性,中位年龄为65岁(范围:21-89岁)。根据临床和组织学分析肿瘤与胃肠道的关系,发现15例可能是胃起源、9例十二指肠起源、13例小肠起源。其余病例按位置分为胰腺周围(n = 25)、盆腔(n = 11)、肠系膜(n = 4)以及部位不明确/多发(n = 35)。肿瘤大小为3-35cm(中位数,15cm),核分裂像为0->100个/5mm(中位数,10个)。组织学上,这些明确的胃肠道外的肿瘤表现出肠(n = 41)和胃(n = 25)的GISTs特征;其余9例组织形态不确定。组织学变异包括梭形、上皮样、空泡状、巢状和粘液样,可能类似其他肿瘤如脂肪肉瘤和孤立性纤维性肿瘤。大部分GIST为KIT阳性(106/112例),其余6例为DOG1 / Ano1阳性。5例显示MDM2局灶核阳性。42/59例中检测到KIT突变,4/16例KIT野生型和3/5例KIT阴性的肿瘤中检测到PDGFRA突变。1例盆腔的腹膜后GIST为琥珀酸脱氢酶缺陷。所有79例患者都在最后的随访中死亡,中位生存期为14个月,很少存活期大于5年。单因素分析中只有可手术切除相对于不能手术的肿瘤在统计学上是有利的因素(P<0.01)。在多因素分析中,核分裂像>50个/5mm2会显著缩短患者生存期(危险度,5.25;95%可信区间,1.65-16.8;P<0.01)。胃肠道外腹膜后GISTs与胃肠道GISTs在组织学和临床病理学上的相似性提示其胃肠道起源。GIST和其他腹膜后肿瘤的潜在重叠特征需要使用多种诊断标记和分子遗传学研究。


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