4例窦岸细胞血管肿瘤的临床病理分析

Littoral cell tumors: a clinicopathologic analysis of four cases

目的 探讨脾脏窦岸细胞血管肿瘤的临床病理学特点、诊断及鉴别诊断。方法 对4例窦岸细胞血管肿瘤病例的临床表现、组织学特征及免疫组化特点进行回顾性分析。结果 男性3例,女性1例,临床表现均有乏力、血小板减少和脾大。其中3例具有窦岸细胞血管瘤的典型镜下形态学表现和免疫组化特征,诊断为窦岸细胞血管瘤。另外1例因出现窦岸细胞异型性,合并出血、坏死及梭形细胞区域,并且梭形细胞仍表达CD8,提示肿瘤细胞起源于脾脏红髓的窦岸细胞,故诊断为窦岸细胞血管肉瘤。结论 窦岸细胞血管瘤的诊断依靠典型的形态学特点和特征性的免疫标志物,但不可忽略组织中出现的其他特殊病理学形态,因此需要广泛取材排除窦岸细胞血管肉瘤的可能。

Objective To investigate the clinicopathological characteristics and diagnosis of splenic littoral cell tumors(LCT). Methods We conducted a retrospective analysis of the clinical manifestations, histological features and immunohistochemical characteristics of 4 patients with LCT. Results There were 3 males and 1 female with clinical manifestations of fatigue, thrombocytopenia and splenomegaly. 3 cases were diagnosed as littoral cell angioma(LCA) because of typical microscopic morphological and immunohistochemical features of LCA. The other 1 case was diagnosed as littoral cell angiosarcma(LCAS) due to the occurrence of littoral cell atypia, combined with hemorrhage, necrosis and spindle cell area where CD8 still expressed, suggesting that the tumor cells originated from the littoral cells. ConclusionThe diagnosis of LCA depends on typical morphological features and characteristic immune markers, but other special pathological patterns in tissues should not be ignored. Therefore, extensive sampling is needed to exclude the possibility of LCAS.