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Title: [Clinicopathologic features of gastric plexiform fibromyxoma].
Author: Hu GM, Chen HP, Liu QY, Shi XC, Wu HF, Feng YK, Ren JL, Wang CF.
Journal: Zhonghua Bing Li Xue Za Zhi; 2018 Apr 08; 47(4):258-262. PubMed ID: 29690664.
Abstract:
Objective: To analyse the clinicopathologic features of gastric plexiform fibromyxoma (PF) including diagnosis, differential diagnosis, immunohistochemistry and molecular pathology. Methods: Eight cases of PF were collected from June 2006 to June 2017 at the Second Affiliated Hospital of Zhengzhou University and the First Affiliated Hospital of Zhengzhou University. The clinicopathologic findings of eight cases of PF were retrospectively analyzed, and immunohistochemistry (EnVision method) and molecular detection of glioma-associated oncogene homologue 1 (GLI1) gene translocation were performed. All cases were histologically reviewed with immunohistochemical staining for smooth muscle actin (SMA), CD10, CD117, DOG1, CD34, ER, PR, ALK and S-100. Fluorescence in situ hybridization (FISH) was used to detect the GLI1 gene translocation, and mutation of CKIT exons 9, 11, 13 and 17; and PDGFRA exons 12, 14 and 18 were identified by Sanger sequencing in four cases. Relevant literature was reviewed. Results: The study included four men and four women, age ranged from 26 to 72 years (mean 51 years). Histologically, the tumors were rich in small thin-walled blood vessels and myxoid matrix, and exhibited multiple nodular growth pattern in the gastric wall. The tumor cells were bland, spindled or oval. Immunohistochemically, all cases strongly expressed vimentin and SMA, and some expressed CD10 (4/8), desmin (3/8), H-caldesmon (5/8) and PR (5/8), but were negative for CD34, S-100, ER, ALK, CD117 and DOG1. The GLI1 gene translocation detection was performed in eight cases by FISH with three positive cases and five negative cases. Mutation analyses for exons 9, 11, 13, and 17 of CKIT genes and exons 12, 14, and 18 of the PDGFRA genes were performed and the tumors all of four tested cases were wild-type. Seven patients were followed up (ranged from 24 to 95 months, mean 50 months) after diagnosis and none of the patients had recurrence or metastasis. Conclusions: PF is a rare novel mesenchymal tumor of the stomach. Its distinct clinicopathologic features and immunohistochemical positivity for SMA, CD10 and PR can help differentiating this entity from other gastrointestinal mesenchymal tumors. FISH detection of GLI1 gene translocation offers an additional molecular diagnostic marker for the diagnosis. 目的： 探讨胃丛状纤维黏液瘤(plexiform fibromyxoma，PF)的临床病理学待征、诊断及鉴别诊断。 方法： 收集郑州大学第二附属医院及郑州大学第一附属医院2006年6月至2017年6月期间8例手术病例胃PF的临床病理资料及随访资料，光镜下观察HE切片、免疫组织化学染色(EnVision法)、荧光原位杂交(FISH)方法检测GLI1基因易位情况，Sanger测序法检测CKIT基因(第9、11、13和17号外显子)及PDGFRA基因(第12、14和18号外显子)的突变状态，并结合文献进行分析。 结果： 男性4例，女性4例；年龄26～72岁(平均年龄51岁)；胃窦6例，胃底2例；肿瘤最大径1.2～7.0 cm，平均3.1 cm；肉眼观肿瘤边界较清，切面灰黄色或灰白色，质韧。低倍镜下肿瘤呈丛状或多结节状，浸润性生长；间质富含薄壁小血管及黏液样基质。高倍镜下肿瘤呈束状、席纹状及编织状排列，瘤细胞梭形或卵圆形，未见肿瘤性坏死，核分裂象(0～2)/50 HPF。免疫组织化学显示，波形蛋白(8/8)、平滑肌肌动蛋白(SMA，8/8)均阳性，CD10(4/8)、结蛋白(3/8)、H－caldesmon(5/8)、孕激素受体(PR，5/8)部分阳性，CD117、DOG1、CD34、间变性淋巴瘤激酶、雌激素受体及S－100蛋白均阴性。分子检测结果：3例检测到GLI1基因易位(3/8)，4例行CKIT及PDGFRA基因突变检测均为野生型。7例获得随访资料，随访时间24～95个月(平均50个月)，均无瘤生存。 结论： 胃PF是一种伴有肌样分化的间叶源性肿瘤，主要发生于胃窦部，目前的随访证据表明其生物学行为呈惰性临床经过，诊断时需与胃肠道间质瘤等多种间叶源性肿瘤鉴别。综合考虑肿瘤的丛状生长方式及免疫组织化学染色表达SMA、PR及CD10对胃PF的诊断与鉴别诊断有较大价值，GLI1基因易位检测亦可作为重要的辅助诊断手段。.

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