Yang J S, Ko J W, Suh K S, Kim S T
Department of Dermatology, Kosin Medical College, Pusan, South Korea.
Am J Dermatopathol. 1999 Oct;21(5):454-7. doi: 10.1097/00000372-199910000-00009.
Congenital glomus tumor is a rare clinical variant of glomus tumor, and glomangiomyoma is the least frequent histologic type of glomus tumor. We report a case of congenital multiple plaque-like glomangiomyoma in a 38-year-old man with multiple nodules and plaques on his left arm and forearm. Histopathologic study showed an angiomatous, nonencapsulated tumor with numerous highly folded dilated vascular lumina scattered throughout the dermis. The lumina were lined by a single layer of flat endothelial cells, and one to several rows of glomus cells were observed adjacent to the endothelial cells. Around large vessels, there was a gradual transition from glomus cells to elongated mature smooth muscle cells with thin and long "blunt-ended" nuclei. Immunohistochemically, there were strong positive reactions for cytoplasmic alpha-smooth muscle actin in glomus cells and smooth muscle cells, vimentin in glomus cells and endothelial cells, and desmin in the smooth muscle cells only. To our knowledge, this is the first case report of congenital multiple plaque-like glomus tumor with the microscopic appearance of a glomangiomyoma.
先天性血管球瘤是血管球瘤的一种罕见临床变异型，而血管球血管肌瘤是血管球瘤中最不常见的组织学类型。我们报告一例38岁男性先天性多发性斑块状血管球血管肌瘤病例，其左臂和前臂有多个结节和斑块。组织病理学研究显示，肿瘤为血管瘤样，无包膜，真皮内散在许多高度折叠的扩张血管腔。血管腔由单层扁平内皮细胞衬里，在内皮细胞旁可见一到几排血管球细胞。在大血管周围，从血管球细胞到细长成熟平滑肌细胞有逐渐过渡，平滑肌细胞核薄而长，呈“钝端”状。免疫组织化学检查显示，血管球细胞和平滑肌细胞的细胞质α平滑肌肌动蛋白、血管球细胞和内皮细胞的波形蛋白呈强阳性反应，仅平滑肌细胞的结蛋白呈阳性反应。据我们所知，这是首例具有血管球血管肌瘤微观表现的先天性多发性斑块状血管球瘤病例报告。
