CAJ | 학술논문

目的探讨胃丛状血管黏液样肌纤维母细胞性肿瘤(PAMT)的临床病理学特征、免疫学表型及其鉴别诊断.方法收集3例PAMT患者的临床和病理资料,进行胃镜、肉眼及光镜观察并行免疫组织化学(EnVision法和SP法)染色,复习文献和总结.结果例1为31岁男性,例2和例3分别为47岁和42岁女性,例1、2因上腹部疼痛就诊,例3为持续性上腹胀2个月就诊.胃镜检查显示3例肿瘤均主要位于胃窦部,呈团块状突向腔内,最大径分别为8.0、4.5和4.6 cm,其中例2伴有溃疡.3例肿瘤均位于胃壁内,低倍镜下显示特征性的丛状或多结节状生长方式,由梭形细胞、纤细的小血管和黏液样的基质所组成.免疫组织化学标记显示,梭形瘤细胞主要表达平滑肌肌动蛋白和肌特异性肌动蛋白(MSA),例3灶性表达结蛋白和h-caldesmon.例1中的部分瘤细胞尚表达上皮细胞膜抗原和CAM5.2,例3灶性表达CD10、孕激素受体.结论胃PAMT是一种非胃肠道间质瘤性间叶性肿瘤,好发于胃窦部,熟悉其病理学特点有助于与其他良恶性肿瘤的鉴别诊断.
목적 탐토위총상혈관점액양기섬유모세포성종류(PAMT)적림상병이학특정、면역학표형급기감별진단.방법 수집3례PAMT환자적림상화병리자료,진행위경、육안급광경관찰병행면역조직화학(EnVision법화SP법)염색,복습문헌화총결.결과 례1위31세남성,례2화례3분별위47세화42세녀성,례1、2인상복부동통취진,례3위지속성상복창2개월취진.위경검사현시3례종류균주요위우위두부,정단괴상돌향강내,최대경분별위8.0、4.5화4.6 cm,기중례2반유궤양.3례종류균위우위벽내,저배경하현시특정성적총상혹다결절상생장방식,유사형세포、섬세적소혈관화점액양적기질소조성.면역조직화학표기현시,사형류세포주요표체평활기기동단백화기특이성기동단백(MSA),례3조성표체결단백화h-caldesmon.례1중적부분류세포상표체상피세포막항원화CAM5.2,례3조성표체CD10、잉격소수체.결론 위PAMT시일충비위장도간질류성간협성종류,호발우위두부,숙실기병이학특점유조우여기타량악성종류적감별진단.
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.