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[胃丛状血管黏液样肌成纤维细胞瘤]

[Plexiform angiomyxoid myofibroblastic tumor of stomach].

作者信息

Bi Rui, Yin Wu, Liu Xin-lian, Wei Hai-ming, Sheng Wei-qi, Wang Jian

机构信息

Department of Pathology, Shanghai Medical College, Shanghai, China.

出版信息

Zhonghua Bing Li Xue Za Zhi. 2012 Nov;41(11):756-60. doi: 10.3760/cma.j.issn.0529-5807.2012.11.010.

Abstract

OBJECTIVE

To study the clinicopathologic features, immunophenotype and differential diagnosis of plexiform angiomyxoid myofibroblastic tumor (PAMT) of the stomach.

METHODS

The clinical and pathologic findings of 3 cases of PAMT in the gastric antrum were retrospectively analyzed. Immunohistochemical study was carried out and the literature was reviewed.

RESULTS

The age of patients ranged from 31 to 47 years. The male-to-female ratio was 1:2. The clinical presentation included epigastric pain and distension. Endoscopically, the tumor mass protruded into the gastric cavity at the antrum and ranged from 4.5 cm to 8.0 cm in greatest dimension. One of the tumors studied was associated with surface ulceration. Histologically, the tumors were located in the gastric wall. They were composed of bland spindle cells and small vessels arranged in a plexiform or nodular pattern within a myxoid stroma. Immunohistochemical study showed that the spindle cells were consistently positive for smooth muscle actin and muscle-specific actin. There was focal staining for h-caldesmon, desmin in case 3 and focal positive for epithelial membrane antigen, CAM5.2 in case 1. Further, CD10 and progesterone receptor were positive in case 3.

CONCLUSIONS

PAMT represents a rare novel mesenchymal tumor of the stomach, with a propensity of gastric antral involvement. The distinctive pathologic features help to differentiate this entity from other benign and malignant tumors.

摘要

目的

研究胃丛状血管黏液样肌成纤维细胞瘤(PAMT)的临床病理特征、免疫表型及鉴别诊断。

方法

回顾性分析3例胃窦部PAMT的临床及病理资料。进行免疫组织化学研究并复习相关文献。

结果

患者年龄31~47岁,男女比例为1∶2。临床表现为上腹部疼痛和腹胀。内镜检查显示,肿瘤肿块突入胃窦部胃腔,最大直径4.5~8.0 cm。其中1例肿瘤伴有表面溃疡。组织学上,肿瘤位于胃壁。由温和的梭形细胞和小血管组成,在黏液样基质中呈丛状或结节状排列。免疫组织化学研究显示,梭形细胞平滑肌肌动蛋白和肌特异性肌动蛋白始终呈阳性。h-钙调蛋白有局灶性染色,3号病例结蛋白阳性,1号病例上皮膜抗原、CAM5.2有局灶性阳性。此外,3号病例CD10和孕激素受体阳性。

结论

PAMT是一种罕见的新型胃间叶性肿瘤,倾向于累及胃窦部。其独特的病理特征有助于将该实体与其他良恶性肿瘤区分开来。

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