Takayama Akiko, Nakashima Osamu, Kobayashi Keita, Kojiro Masamichi
Department of Pathology, Kurume University School of Medicine, Kurume, Fukuoka, Japan.
Pathol Int. 2003 Jul;53(7):483-8. doi: 10.1046/j.1440-1827.2003.01493.x.
A 76-year-old man complained of difficulty breathing. A solitary mass was found in the spleen by ultrasonography and the tumor was excised. Grossly, the tumor was 3.9 x 2.9 cm in size, solid and brownish in color. A stellate scar-like fibrosis was observed in the center of the tumor. Histologically, the tumor consisted of the proliferation of irregular and small lymph vessel-like spaces, with sclerotic change in the center. The lymph vessel-like spaces showed papillary projections of the lining cells. The lumen contained amorphous proteinaceous fluid. Immunohistochemically, the lining cells of lymph vessel-like spaces were positive for endothelial markers (CD31, CD34, factor VIII-related antigen), and bound Ulex europaeus agglutinin-1. The tumor was diagnosed as splenic lymphangioma, but its appearance was rather unusual for a typical splenic lymphangioma because of the presence of papillary endothelial proliferation and scar-like fibrosis. Splenic lymphangioma with papillary endothelial proliferation is uncommon, and there have been only four cases reported.
一名76岁男性主诉呼吸困难。超声检查发现脾脏有一个孤立性肿块，遂将肿瘤切除。大体上，肿瘤大小为3.9×2.9 cm，质地坚实，呈褐色。肿瘤中心可见星状瘢痕样纤维化。组织学上，肿瘤由不规则的小淋巴管样间隙增生组成，中心有硬化改变。淋巴管样间隙内衬细胞可见乳头状突起。管腔内含有无定形蛋白质液体。免疫组化显示，淋巴管样间隙的内衬细胞内皮标志物（CD31、CD34、VIII因子相关抗原）呈阳性，并结合荆豆凝集素-1。该肿瘤被诊断为脾淋巴管瘤，但其表现对于典型的脾淋巴管瘤来说相当不寻常，因为存在乳头状内皮增生和瘢痕样纤维化。伴有乳头状内皮增生的脾淋巴管瘤并不常见，仅报道过4例。
