Shen Jia, Shrestha Swati, Yen Yu-Hsin, Scott Michelle A, Soo Chia, Ting Kang, Peault Bruno, Dry Sarah M, James Aaron W
School of Dentistry, University of California, Los Angeles, CA, 90095.
School of Dentistry, University of California, Los Angeles, CA, 90095; Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA, 90095.
Hum Pathol. 2016 Jan;47(1):121-31. doi: 10.1016/j.humpath.2015.09.013. Epub 2015 Sep 30.
Perivascular soft tissue tumors are relatively uncommon neoplasms of unclear lineage of differentiation, although most are presumed to originate from or differentiate to pericytes or a modified perivascular cell. Among these, glomus tumor, myopericytoma, and angioleiomyoma share a spectrum of histologic findings and a perivascular growth pattern. In contrast, solitary fibrous tumor was once hypothesized to have pericytic differentiation--although little bona fide evidence of pericytic differentiation exists. Likewise the perivascular epithelioid cell tumor (PEComa) family shares a perivascular growth pattern, but with distinctive dual myoid-melanocytic differentiation. RGS5, regulator of G-protein signaling 5, is a novel pericyte antigen with increasing use in animal models. Here, we describe the immunohistochemical expression patterns of RGS5 across perivascular soft tissue tumors, including glomus tumor (n = 6), malignant glomus tumor (n = 4), myopericytoma (n = 3), angioleiomyoma (n = 9), myofibroma (n = 4), solitary fibrous tumor (n = 10), and PEComa (n = 19). Immunohistochemical staining and semi-quantification was performed, and compared to αSMA (smooth muscle actin) expression. Results showed that glomus tumor (including malignant glomus tumor), myopericytoma, and angioleiomyoma shared a similar diffuse immunoreactivity for RGS5 and αSMA across all tumors examined. In contrast, myofibroma, solitary fibrous tumor and PEComa showed predominantly focal to absent RGS5 immunoreactivity. These findings further support a common pericytic lineage of differentiation in glomus tumors, myopericytoma and angioleiomyoma. The pericyte marker RGS5 may be of future clinical utility for the evaluation of pericytic differentiation in soft tissue tumors.
血管周围软组织肿瘤是一类相对罕见的肿瘤,其分化谱系尚不清楚,不过大多数被认为起源于周细胞或向周细胞或一种改良的血管周围细胞分化。其中,血管球瘤、肌周细胞瘤和血管平滑肌瘤具有一系列组织学表现和血管周围生长模式。相比之下,孤立性纤维瘤曾被假设具有周细胞分化——尽管几乎没有周细胞分化的确凿证据。同样,血管周围上皮样细胞瘤(PEComa)家族也具有血管周围生长模式,但具有独特的双肌样-黑素细胞分化。RGS5(G蛋白信号调节因子5)是一种新型周细胞抗原,在动物模型中的应用越来越多。在此,我们描述了RGS5在血管周围软组织肿瘤中的免疫组化表达模式,包括血管球瘤(n = 6)、恶性血管球瘤(n = 4)、肌周细胞瘤(n = 3)、血管平滑肌瘤(n = 9)、肌纤维瘤(n = 4)、孤立性纤维瘤(n = 10)和PEComa(n = 19)。进行了免疫组化染色和半定量分析,并与αSMA(平滑肌肌动蛋白)表达进行比较。结果显示,在所有检测的肿瘤中,血管球瘤(包括恶性血管球瘤)、肌周细胞瘤和血管平滑肌瘤对RGS5和αSMA具有相似的弥漫性免疫反应。相比之下,肌纤维瘤、孤立性纤维瘤和PEComa主要表现为RGS5免疫反应性局灶性至缺失。这些发现进一步支持了血管球瘤、肌周细胞瘤和血管平滑肌瘤存在共同的周细胞分化谱系。周细胞标志物RGS5可能在未来临床中用于评估软组织肿瘤的周细胞分化。