钙化性纤维性肿瘤的临床病理学分析

Calcifying fibrous tumor:a clinicopathologic study of seven cases

目的探讨钙化性纤维性肿瘤(calcifyingfibroustumor,CFT)的临床病理学特征、免疫表型和鉴别诊断。方法回顾性分析7例CFT的临床资料、病理学形态和免疫组化标记结果。结果患者中1例为少年,6例均为成年人,年龄14～50岁,平均37岁。5例临床表现为局部缓慢性生长的无痛性肿块,2例为术中偶然发现。肿瘤分别位于腹腔/盆腔(3例)、颈部(2例)、左腹股沟(1例)和左小腿(1例)。眼观:肿块境界清楚,卵圆形或结节状,质地坚韧。镜检:肿瘤由大量胶原化的纤维结缔组织组成,其间夹杂少量梭形细胞。特征性形态学表现为在胶原化的纤维组织间可见散在的钙化灶或砂砾小体,间质内伴有多少不等的淋巴细胞和浆细胞浸润灶,部分病例中可见生发中心形成。免疫组化标记显示,梭形细胞主要表达vimentin,不表达CD34、S100蛋白、actin、desmin、hcaldesmon和ALK1等标记。随访6例,均无复发。结论CFT是一种不同于炎性肌纤维母细胞瘤的良性纤维母细胞性肿瘤。CFT不仅好发于儿童和青少年,也可发生于成年人。组织学上应与伴有钙化的纤维母细胞/肌纤维母细胞性病变相鉴别。

Purpose To study the clinicopathologic and immunohistochemical features of calcifying fibrous tumor (CFT),and to discuss the differential diagnosis. Methods The clinical,pathologic and immunohistochemical features of seven cases of CFT were reevaluated. Results One case occurred in a 14-year-old boy,whereas other 6 cases occurred in adults. Age ranged from 14 to 50 years (mean,37). The main manifestation in five cases was a slowly growing mass. The other 2 cases were found incidentally during abdominal operation for other causes. The tumors were located in the abdominal/peritoneal cavity (n=3),neck (n=2),left inguinal region (n=1) and left leg (n=1). Grossly,they were well-circumscribed with an ovoid or nodular appearance and firm consistency. Microscopically,the tumor was composed of abundant bundles of hyalinized collagens interspersed with fibroblast-like spindle cells. All 7 cases were characterized by deposition of dystrophic or psammatous calcifications accompanied with chronic inflammatory cells infiltration in the stroma. In some cases,there was formation of germinal centers. Immunohistochemically,the spindle cells were diffusely positive for vimentin,but negative for CD34,S-100 protein,actin,desmin,h-caldesmon and ALK1. Follow-up information in six cases showed a benign course. Conclusions CFT is a benign fibroblastic tumor differing from inflammatory myofibroblastic tumor. Not only can CFT occur in children and adolescents,but also in young to middle-aged adults. CFT needs to be differentiated from a variety of fibroblastic and myofibroblastic lesions with calcifications.