Nikaido Takashi, Endo Yasuhiko, Nimura Satoshi, Ishikura Hiroshi, Ushigome Shinichiro
Department of Pathology, Chiba University Hospital, Chiba, Japan.
Pathol Int. 2004 Apr;54(4):256-60. doi: 10.1111/j.1440-1827.2004.01616.x.
An inferior vena cava (IVC) tumor was incidentally found in a 67-year-old Japanese man. The resected tumor was lobulated and multinodular, measuring 14.0 x 6.5 x 7.0 cm, showing a dumbbell-shaped appearance with a central constriction. The tumor showed both intra- and extra-luminal growth. The tumor was primarily composed of well-differentiated leiomyosarcoma. Spindle tumor cells in the well-differentiated area were positive for vimentin, muscle actin, alpha-smooth muscle actin, and desmin. Foci of rhabdoid cells and osteoclast-type multinucleated giant cells were also found. Rhabdoid cells ultrastructurally had paranuclear aggregates or whorls of intermediate filaments that were positive for vimentin, low molecular weight cytokeratin, and desmin. Osteoclast-type multinucleated giant cells were positive for only CD68 antigen, suggesting a reactive histiocytic lineage. To the best of our knowledge, this is the first case of IVC leiomyosarcoma accompanied by both rhabdoid tumor cells and osteoclast-type reactive multinucleated giant cells. These unusual features should be kept in mind in the diagnosis of dumbbell-shaped retroperitoneal tumors that involve the IVC.
一名67岁的日本男性偶然发现患有下腔静脉（IVC）肿瘤。切除的肿瘤呈分叶状且多结节状，大小为14.0×6.5×7.0厘米，呈哑铃状外观，中央有狭窄。肿瘤表现为腔内和腔外生长。肿瘤主要由高分化平滑肌肉瘤组成。高分化区域的梭形肿瘤细胞波形蛋白、肌动蛋白、α平滑肌肌动蛋白和结蛋白呈阳性。还发现了横纹肌样细胞灶和破骨细胞型多核巨细胞。横纹肌样细胞超微结构显示核旁有中间丝的聚集或漩涡，波形蛋白、低分子量细胞角蛋白和结蛋白呈阳性。破骨细胞型多核巨细胞仅CD68抗原呈阳性，提示为反应性组织细胞谱系。据我们所知，这是首例伴有横纹肌样肿瘤细胞和破骨细胞型反应性多核巨细胞的IVC平滑肌肉瘤。在诊断累及IVC的哑铃状腹膜后肿瘤时应牢记这些不寻常的特征。
