Zheng Yan, George Mary, Chen Frank
Department of Pathology, Buffalo General Medical Center, 100 High Street, Buffalo, NY 14203, USA.
BMC Res Notes. 2014 Feb 28;7:118. doi: 10.1186/1756-0500-7-118.
Intravascular fasciitis is an uncommon variant of nodular fasciitis, which is a reactive proliferative lesion of myofibroblasts. Since its identification in 1981, only 32 cases of intravascular fasciitis have been reported in the English literature. The lesion is commonly located in the head, neck, and extremities, with only three cases arising from the trunk. Here we report the fourth case involving the trunk (the flank area).
A 21-year-old African-American female presented with a subcutaneous mass on her flank. Grossly, the mass was red-tan, oval, and well-demarcated, measuring approximately 0.5 cm in diameter. Microscopically, the mass was composed of spindle cells arranged in a swirling and intersecting pattern inside the lumens of two blood vessels. It extended through the vascular walls into the surrounding fibroadipose tissue; in some sections, the spindle cells were intermixed with the perivascular fibrous tissue. Elastin stain revealed remnants of elastic lamina partially surrounding the lesion. The nuclei of the spindle cells were relatively uniform with tapered ends and prominent nucleoli. No significant mitotic activity was observed. Multinucleated giant cells were scattered among the spindle cells, along with infiltrating lymphocytes and extravasated red blood cells. Immunohistochemical stains showed the spindle cells were positive for smooth muscle actin, focally positive for muscle specific actin, and negative for S-100, confirming their myofibroblastic differentiation. The overall morphological and immunohistochemical features are consistent with intravascular fasciitis.
By reporting this rare case, we would like to raise the awareness of this non-neoplastic lesion to avoid misdiagnosing it as a sarcoma with vascular invasion. Previously reported similar cases were also reviewed and compared with this case.
血管内筋膜炎是结节性筋膜炎的一种罕见变体,结节性筋膜炎是肌成纤维细胞的一种反应性增生性病变。自1981年被发现以来,英文文献中仅报道了32例血管内筋膜炎病例。该病变通常位于头、颈和四肢,仅有3例发生于躯干。在此,我们报告第四例累及躯干(侧腹区域)的病例。
一名21岁的非裔美国女性,侧腹出现一个皮下肿块。大体上,肿块呈红棕色,椭圆形,边界清晰,直径约0.5厘米。显微镜下,肿块由梭形细胞组成,这些细胞在两条血管腔内呈漩涡状和交叉状排列。它穿过血管壁延伸至周围的纤维脂肪组织;在一些切片中,梭形细胞与血管周围纤维组织混合。弹性蛋白染色显示弹性膜残余部分围绕着病变。梭形细胞的细胞核相对均匀,两端逐渐变细,核仁明显。未观察到明显的有丝分裂活性。多核巨细胞散在于梭形细胞之间,伴有浸润的淋巴细胞和外渗的红细胞。免疫组织化学染色显示梭形细胞平滑肌肌动蛋白呈阳性,肌肉特异性肌动蛋白局灶性阳性,S-100呈阴性,证实其肌成纤维细胞分化。总体形态和免疫组织化学特征与血管内筋膜炎一致。
通过报告这一罕见病例,我们希望提高对这种非肿瘤性病变的认识,以避免将其误诊为伴有血管侵犯的肉瘤。还对先前报道的类似病例进行了回顾并与本病例进行了比较。