GIST Manifesting as a Retroperitoneal Tumor: Clinicopathologic Immunohistochemical, and Molecular Genetic Study of 112 Cases.
GIST Manifesting as a Retroperitoneal Tumor: Clinicopathologic Immunohistochemical, and Molecular Genetic Study of 112 Cases.
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DOI：
10.1097/pas.0000000000000807
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发表时间：
2017-05
期刊：
影响因子：
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通讯作者：
Lasota J
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文献类型：
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作者：
Miettinen M;Felisiak-Golabek A;Wang Z;Inaguma S;Lasota J
大多数胃肠道间质瘤(GIST)发生在管状胃肠道，但也有一些明显地出现在胃肠道外。在这项研究中，我们分析了112个位于腹膜后的GIST。这些肿瘤发生在55名女性和57名男性，平均年龄为65岁(范围：21-89岁)。根据临床或组织学发现的胃肠道连接，15个肿瘤可能来自胃，9个可能来自十二指肠，13个可能来自小肠。其余病例按部位分为胰周(n=25)、盆腔(n=11)、肠系膜(n=4)和未指明/其他部位(n=35)。肿瘤大小不一，大小为3-35厘米(中位数15厘米)，每5平方毫米有丝分裂率不同(中位数10)。组织学上，胃肠道外肿瘤41例，胃GIST 25例，9例组织学不明确。组织学变异包括梭形、上皮样、空泡化、嵌套状和粘液样，可能模拟其他肿瘤，如脂肪肉瘤和孤立性纤维瘤。大多数GIST为KIT阳性(106/112例)，其余6例GIST为Dog1/Ano1阳性。5例MDM2核局灶性阳性。检测到KIT突变42/59例，分析了4/16例KIT野生型和3/5例KIT阴性肿瘤的PDGFRA突变。1例盆腔腹膜后GIST为SDH缺乏症。在最后一次随访中，79名患者全部死亡，中位生存期为14个月，仅有少数患者存活5年。在单因素分析中，只有可手术肿瘤与不可手术肿瘤在统计学上是有利的因素(p<；0.01)。在多因素分析中，有丝分裂比率50/5mm2与较短的生存期显著相关(HR 5.25，95%CI 1.65~16.8，p<；0.01)。胃肠道后腹膜后GIST与胃肠道GIST的组织学和临床病理特征相似，提示其起源于胃肠道。GIST和其他腹膜后肿瘤之间的潜在重叠特征需要使用多个诊断标记和分子遗传学研究。
Most gastrointestinal stromal tumors (GISTs) occur in the tubular gastrointestinal 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: 21-89 years). Based on 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 3-35 cm (median, 15 cm) and by mitotic rate per 5 mm2, 0- >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 SDH-deficient. All 79 patients were dead at last follow-up with a median survival of 14 months, with few survivals > 5 years. Only operable vs. inoperable tumor was a statistically favorable factor in univariate analysis (p<0.01). In multivariate analysis, mitotic rate > 50/5 mm2 was significant for a shorter survival (HR 5.25, 95% CI 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.