腱鞘纤维瘤39例临床病理学分析

Clinicopathological study of fibroma of tendon sheath

目的探讨腱鞘纤维瘤(fibroma of tendon sheath,FTS)的临床病理学特征。方法对39例FTS的临床表现、影像学及病理学特征进行观察。结果男性24例(61.54%),女性15例(38.46%),男女比为1.6∶1,年龄5～69岁,平均34.56岁。74.36%的病例累及上肢,尤以掌指部为主。临床上,66.67%病例表现为局部无痛性缓慢生长的肿块,33.33%病例伴局部压痛、肿痛症状。10.26%病例曾有局部外伤史,患者术后复发率为14.29%。以MRI为代表的影像学成像显示病变与肌腱、腱鞘或关节囊紧密相连。大体上,病变包膜纤薄或无包膜。镜下病变呈分叶状,每个小叶由稀疏散在的纤维母细胞、裂隙状血管腔隙和大量玻璃样变胶原间质所组成。部分病变形态多样,局部与促结缔组织增生性纤维母细胞瘤、纤维组织细胞瘤或纤维瘤病、结节性筋膜炎及微静脉型血管瘤病变相似。免疫组化示所有成分均表达vimentin,部分梭形及星形细胞表达α-SMA、calponin及CD163,少数病变局灶弱阳性表达β-catenin(胞质)、CD68、MSA及h-Caldesmon,裂隙状腔隙内衬扁平细胞表达CD34、CD31及FⅧRAg,提示为血管性腔隙。结论 FTS是一种相对少见的发生于肌腱、腱鞘或关节滑囊的良性纤维母/肌纤维母细胞增生性病变。典型的FTS多发生于青年人及成年人,以男性居多,好发于上肢,掌指部占多数,少数病变切除后可复发。

Purpose To study the clinicopathological features of fibroma of tendon sheath（FTS）. Methods 39 cases of FTS were an- alyzed by the clinical manifestations, imaging features and histopathological characteristics. Results Of 39 cases, 24 （61.54%）were male and 15（38.46% ）were female, male-female ratio is 16 ： 1. The ages of all the patients at the time of the first surgical procedure ranged from 5 to 69 years, with a average of 34. 56 years and with a peak incidence at 20 - 60 years of age. All of the lesions arose in the extremities and in 74. 36% of cases the lesion involved the upper extremity which the hands and fingersbeing the most commonly in- volved sites. Clinically, 66. 67% of patients presented no particular clinical symptoms other than a painless mass. Tenderness or mild pain was present in 33.33% of cases. A history of trauma related to the development of the tumor was known in 10.26% of cases. 14. 29% of cases have a recurrence with 1 to 95 months of follow-up after resection. MRI as a representative of imagings with FTS ap- peared as focal lobular masses adjacent to tendon, tendon sheaths or articular capsule. The lesions were gray-white in general. Most were firm or hard in consistency. Sometimes, they were soft, ＂mucoid＂ and focally cystic. Histologically, it is typically characterized for the lobuled lession by a dense fibrocllagenous stroma with scattered spindle-shaped fibroblasts and narrow slit-like vascular spaces. Histological appearances in some cases may change muhiterminally, similar to desmoplastic fibroblastoma, fibrous histiocytoma, fibro- matosis, nodular faseiitis or microvenular hemangioma. Immunophenotype showed all elements strongly positive for vimentin, partly pos- itive for ot-SMA, ealponin and CD163, and focally, spindle cells stained weakly for 13-catenin（cytoplasm-type）, CD68, MSA and h- ealdesmon, pinacocytes lining in the slit-like spaces stained for CD34, CD31 and FVI^RAg. Conclusions FFS consists of a relatively rare, benign proliferation of fibroblasts/ myofibroblasts firmly attached to tendon or tendon sheath and sometimes attached to articular synovial capsule. It is unclear whether it represents a reactive fibrosis or a benign neoplasm. FTS typically develops in young, adult males. In majority of the cases the lesion involved the upper extremity, with the hands and fingers being the most commonly involved sites and few eases may recur one or twice in a uncertain period after resection.