指趾纤维骨性假瘤临床病理观察并文献复习

Clinicopathological observation and literature review of fibro-osseous pseudotumor of digits

目的探讨指趾纤维骨性假瘤(fibro-osseous pseudotumor of digits,FOPD)的临床病理学特征。方法收集武汉大学中南医院病理科2016年2月—2018年6月诊断的2例FOPD的临床、影像、病理资料,并复习相关文献。结果2例FOPD患者均为男性,平均年龄31岁,1例位于食指,1例位于大鱼际,病变境界较清楚,由杂乱增生的纤维母细胞/肌纤维母细胞和成熟程度不等的骨样组织构成。梭形纤维母细胞形态温和,偶可见核分裂象,形态上类似结节性筋膜炎。骨样组织成熟程度不等,形态不规则,周围可见骨母细胞围绕,条索状随机分布于纤维组织内。免疫组织化学;梭形细胞vimentin(+)、calponin(+)、SMA(+),caldesmon(-)、desmin(-)、β-catenin(-)、S-100(-)、CD34(-)、CK(-)、EMA(-),Ki-67热点区约20%;骨母细胞SATB-2(强+)。分子生物学检测:1例检测出USP6基因断裂。结论FOPD是一种较少见的良性病变,其临床病理特征与骨化性肌炎十分相似,完整切除后预后良好,目前尚未见转移或恶变的报道。

Objective To explore clinical and pathological characteristics of fibro-osseous pseudotumor of digits(FOPD).Methods 2 cases of FOPD in the department of Pathology,Zhongnan Hospital of Wuhan University from February 2016 to June 2018 were collected,followed by analysis of clinical,imaging and pathological data and review of the relative literature.Results The two patients with FOPD were male,with an average age of 31.One was on the index finger and the other was on the thenar eminence.The lesion was well-circumscribed and consisted of disordered hyperplastic fibroblasts/myofibroblast and osteoid tissue of varying maturity.The spindle fibroblasts had a mild morphology with occasional mitotic figures,similar to those in nodular fasciitis.Osteoid tissue had varying degrees of maturity and irregular shapes,surrounded by osteoblasts,and were randomly distributed in fibrous tissues.Immunohistochemical staining showed that the spindle fibroblasts were vimentin(+),calponin(+),SMA(-),caldesmon(-),desmin(-),β-catenin(-),S-100(-),CD34(-),CK(-),EMA(-),with about 20%of the index of Ki-67 hotspot,and those osteoblasts were strongly positive for SATB-2.Molecular biological detection:USP6 gene break was detected in 1 case.Conclusion FOPD is a rare benign lesion.Its clinicopathological characteristics are similar to ossifying myositis.After complete resection,the prognosis is good.There are no reports of metastasis or malignant transformation.