皮革样胃肥大细胞增生症临床病理观察

Mastocytosis of stomach resembling linitis plastica

目的探讨罕见的皮革样胃肥大细胞增生症的临床病理特点。方法报道1例皮革样胃肥大细胞增生症,回顾分析其临床表现、影像学、胃镜下表现及病理组织学特点,应用免疫组化方法及特殊染色进一步确认诊断,探讨诊断及鉴别诊断,同时随访患者。结果临床无特殊表现。影像学显示胃底后壁明显弥漫性均匀增厚。胃镜显示黏膜粗大。病理检查大体示胃呈皮革样改变;镜下胃壁全层见肥大细胞弥漫浸润并部分累及局部淋巴结。免疫组化示肿瘤细胞CD117、CD43、CD68和LCA(+);甲苯胺兰染色示瘤细胞胞质呈紫红色。随诊44个月,患者健康。结论胃肥大细胞增生症是罕见的系统性肥大细胞增生,胃呈皮革样改变和惰性生长是其特殊之处。

Objective To evaluate the clinicopathological features of a rare case with mastocytosis of stomach resembling linitis plastica.Methods A retrospective study was performed to analyse the clinical manifestations,radiological,endoscopic and histopathological features of a case of gastric mastocytosis.Immunohistochemical and special stains were used to further confirm the diagnosis.The diagnosis and differential diagnosis about mastocytosis were discussed,while the patient was followed up.Results The gastric lesion in the patient was found accidently without any clinical symptoms and signs.CT scan showed posterior fundus of stomach with apparent homogeneously and diffusely thickened wall and endoscopy also showed thickened mucous membrane of stomach.Pathological examination showed the stomach had linitis plastica-like changes with diffuse mast cells infiltration in the lesion meanwhile mast cells partially involved the regional lymph nodes.Tumor cells expressed CD117,CD43,CD68 and LCA.Toluidine blue staining showed purple-red cytoplasm of the tumor cells.The patient survived and remained healthy after following up for 44 months.Conclusion This case of gastric mast cell hyperplasia is a rare case of systematic mast cell hyperplasia with special features,such as linitis plastica-like changes,without symptoms and signs,no involvement in the skin and bone marrow and indolent growth.