非典型褥疮性纤维组织增生

Atypical decubital fibroplasia

目的 :探讨非典型褥疮性纤维组织增生的病理形态学特点。方法 :对 2例非典型褥疮性纤维组织增生进行临床病理学分析和免疫组化研究 ,并复习文献。结果 :2例均为行动不便的老年患者 ,临床上分别表现为臀部和肩部深部软组织内的无痛性肿块。镜下 ,病变界限不清 ,呈多结节状 ,并大致呈现区带性 ,即由位于中央的成片纤维素性坏死区和位于周边的肉芽肿样组织组成 ,间质呈明显的黏液样变性。肉芽肿样区内的增生性纤维母细胞形态各异 ,有一定的异型性 ,并常见节细胞样细胞 ,似增生性筋膜炎。免疫组化显示强阳性表达vimentin ,部分弱阳性表达α SMA或MSA。除增生的纤维母细胞外 ,周边还可见增生的薄壁小血管 ,其内皮多肿胀 ,部分区域内似与周围的增生性纤维母细胞有移行。部分血管壁伴有透明样变性或可见纤维素性沉着。结论 :非典型褥疮性纤维组织增生属于一种少见类型的假肉瘤性纤维母细胞性增生 ,由局部软组织长期间歇性受压引起局部缺血所致 ,有别于褥疮性溃疡。因增生的纤维母细胞常显示一定的异型性 ,容易被误诊为肉瘤性病变 ,诊断时应特别加以注意。

Purpose To study the morphological characteristics of atypical decubital fibroplasia (ADF). Methods Two cases of ADF were investigated by clinical and pathological evaluation and immunohistochemistry, together with a review of the literature. Results Both cases were physically debilitated old patients, and presented clinically as deeply located painless masses of the buttock and shoulder respectively. Histologically, the lesion was ill defined and multi nodulated, which showed a vague zonation with central areas composed of fibrinoid necrosis and peripheral areas composed of granulation-like tissue. The proliferative fibroblasts within the granulation like zones varied in shape and displayed cellular atypia to some extent. They showed strong immunoreactivity to vimentin and partial,weak staining to α SMA or MSA. Besides the fibroblasts, there was also a proliferation of small thin walled ectatic vessels with plump endothelial cells. In some areas, the endothelial cells merged with perivascular fibroblasts, and in addition, hyalinization of the vessel walls and fibrinoid deposit were also noted. Conclusions ADF represents a rare form of the so called pseduosarcomatous fibroblastic proliferation, which was caused by local ischemia resulted from a prolonged intermittent pressure. It differred from the decubital ulcer. As the proliferative fibroblasts usually exhibit cellular atypia, it is very important not to misinterpret the ADF as a sarcoma, or on the hand, as other pseduosarcomatous fibroblastic lesions, such as proliferative fasciitis.