Kiyohara Takahiro, Maruta Naoki, Iino Shiro, Ido Hideki, Tokuriki Atsushi, Hasegawa Minoru
Department of Dermatology, Kansai Medical University Medical Center, Osaka, Japan.
Department of Dermatology, University of Fukui, Fukui, Japan.
J Dermatol. 2016 Sep;43(9):1088-91. doi: 10.1111/1346-8138.13400. Epub 2016 Apr 14.
We describe a case of CD34-positive infantile myofibromatosis with hemangiopericytoma-like pattern. A 2-day-old Japanese boy presented with multiple hemispherical nodules on the extremities and back. There was a biphasic histological growth in the dermis, accompanied by a hemangiopericytoma-like pattern with antler-like branching vessels. Tumor cells were oval to spindle-shaped myoid cells with bland appearance. Immunohistochemically, vimentin, calponin and CD34 were positive, while α-smooth muscle actin, h-caldesmon, HHF35 and desmin were negative. Although CD34 was positive, the present case could be diagnosed as infantile myofibromatosis. Myopericytoma, myofibroma/myofibromatosis, glomus tumor, glomangiopericytoma and angioleiomyoma share a continuous spectrum of benign hemangiopericytoma-like pattern tumors. Myofibroma/myofibromatosis is nearly included in myopericytoma among pericytic (perivascular) tumors, and could be positive for CD34. Several immunohistochemical panels of smooth muscle markers are needed for the diagnosis of pericytic (perivascular) tumors.
我们描述了一例具有血管外皮细胞瘤样模式的CD34阳性婴儿肌纤维瘤病。一名2日龄日本男婴四肢和背部出现多个半球形结节。真皮内有双相组织学生长,伴有鹿角状分支血管的血管外皮细胞瘤样模式。肿瘤细胞为椭圆形至梭形肌样细胞,外观温和。免疫组化显示,波形蛋白、钙调蛋白和CD34呈阳性,而α平滑肌肌动蛋白、h-钙调蛋白、HHF35和结蛋白呈阴性。尽管CD34呈阳性,但本例可诊断为婴儿肌纤维瘤病。肌周细胞瘤、肌纤维瘤/肌纤维瘤病、血管球瘤、血管球血管外皮细胞瘤和血管平滑肌瘤构成了具有连续谱系的良性血管外皮细胞瘤样模式肿瘤。在周细胞(血管周围)肿瘤中,肌纤维瘤/肌纤维瘤病几乎包含在肌周细胞瘤中,且可能CD34呈阳性。诊断周细胞(血管周围)肿瘤需要几个平滑肌标志物的免疫组化检测。