Hu Guiming, Chen Huiping, Liu Qiuyu, Wei Jianguo, Feng Yikun, Fu Wengjing, Zhang Ming, Wu Huifang, Gu Bin, Ren Jingli
Department of Pathology, The Second Affiliated Hospital of Zhengzhou University Zhengzhou, Henan Province, China.
Department of Pathology, The Maternal and Child Health Hospital of Zhengzhou Zhengzhou, Henan Province, China.
Int J Clin Exp Pathol. 2017 Nov 1;10(11):10926-10933. eCollection 2017.
Plexiform fibromyxoma (PF) is a unique mesenchymal tumor of the stomach. The molecular characteristics of these tumors remain unclear. Here, we report 10 cases of PF with clinicopathological features and molecular features in detail. The patients ranged in age from 26 to 72 years (mean, 49 y) and most commonly presented with abdominal pain and distension, black stool, and anemia. Eight tumors were located at the antrum while two in the fundus of stomach. Histologically, tumor cells exhibited a plexiform growth pattern with multiple nodules in the muscularis propria of stomach wall and infiltrative borders. Immunohistochemically, all tumors were strongly positive for vimentin and smooth muscle actin (SMA), some were staining for CD10 (5/10), desmin (5/10), H-caldesmon (6/10) and progesterone receptor (PR, 6/10), however, CD34, S-100, Estrogen Receptor (ER), ALK, CD117 and DOG-1 were all negative in our cases. The glioma-associated oncogene homologue 1 () gene translocation was detected in eight cases by FISH with three positive and five negative. Mutation analyses of and platelet-derived growth factor receptor alpha () genes were performed on five cases and all of which were wild-type for mutation. Our follow-up indicated that all of the patients made an uneventful recovery at 24 to 95 months after diagnosis. In summary, the distinctively histological features and immunohistochemical positivity of SMA, CD10 and PR can help differentiate PF from other gastrointestinal mesenchymal tumors. gene translocation offers an additional molecular instrument for the diagnosis. The expression of PR and the existence of gene translocation in PF highlights of our article.
丛状纤维黏液瘤（PF）是一种独特的胃间叶性肿瘤。这些肿瘤的分子特征仍不清楚。在此，我们详细报告10例PF的临床病理特征和分子特征。患者年龄在26至72岁之间（平均49岁），最常见的表现为腹痛、腹胀、黑便和贫血。8个肿瘤位于胃窦，2个位于胃底。组织学上，肿瘤细胞呈丛状生长模式，胃壁固有肌层有多个结节，边界浸润。免疫组化方面，所有肿瘤波形蛋白和平滑肌肌动蛋白（SMA）均呈强阳性，部分肿瘤CD10（5/10）、结蛋白（5/10）、H - 钙调蛋白（6/10）和孕激素受体（PR，6/10）呈阳性染色，然而，在我们的病例中CD34、S - 100、雌激素受体（ER）、ALK、CD117和DOG - 1均为阴性。通过荧光原位杂交（FISH）在8例中检测到胶质瘤相关癌基因同源物1（）基因易位，其中3例阳性，5例阴性。对5例进行了和血小板衍生生长因子受体α（）基因的突变分析，所有病例均为野生型突变。我们的随访表明，所有患者在诊断后24至95个月均恢复良好。总之，独特的组织学特征以及SMA、CD10和PR的免疫组化阳性有助于将PF与其他胃肠道间叶性肿瘤区分开来。基因易位为诊断提供了另一种分子手段。PF中PR的表达和基因易位的存在是我们文章的重点。
