Liu Yong-Qiao, Yue Jun-Qiu
Department of Pathology, People's Hospital of Yingcheng Yingcheng, Hubei, China.
Int J Clin Exp Pathol. 2013 Sep 15;6(10):2247-50. eCollection 2013.
Paraganglioma is a neuroendocrine neoplasm, which is extremely rare in the vulva and only one case has been reported. Here we present a case of vulvar paraganglioma in a 48-year-old woman and a literature review. The patient found a lump located in the genitals below the symphysis pubis 3 months before presentation when she complained that the lump was increasing in size. A 3.2 cm x 2.3 cm x 1.5 cm nodule was excised from subcutaneous soft tissue in the vulva. Microscopy showed a diversity of cell morphologies and structures in the rich vascular network of the tumor separated the chief cells into round cell nests (Zellballen pattern). Some areas of the tumor presented epithelioid and spindle-shaped cells with increased cell density and indistinct structural characteristics. Hyaline degeneration of collagen fibers or mucoid degeneration was found in tumor interstitium. Immunohistochemical staining showed diffused expression of synaptophysin in the chief cells, focal expression of S-100 protein in the sustentacular cells and high expression of CD34 in the vascular components. Based on morphological and immunohistochemical results, a rare paraganglioma of the vulva was diagnosed.
副神经节瘤是一种神经内分泌肿瘤，在外阴极为罕见，仅有一例报道。在此，我们报告一例48岁女性的外阴副神经节瘤病例并进行文献复习。患者在就诊前3个月发现耻骨联合下方生殖器处有一肿块，当时她主诉肿块在增大。从外阴皮下软组织切除一个3.2 cm×2.3 cm×1.5 cm的结节。显微镜检查显示肿瘤丰富血管网络中有多种细胞形态和结构，主细胞被分隔成圆形细胞巢（Zellballen模式）。肿瘤部分区域可见上皮样细胞和梭形细胞，细胞密度增加，结构特征不明显。肿瘤间质可见胶原纤维玻璃样变性或黏液样变性。免疫组化染色显示主细胞中突触素弥漫性表达，支持细胞中S-100蛋白局灶性表达，血管成分中CD34高表达。根据形态学和免疫组化结果，诊断为罕见的外阴副神经节瘤。
