包涵体性纤维瘤病临床病理分析

Clinicopathologic features of inclusion body fibromatosis

目的总结包涵体性纤维瘤病(IBF)的临床病理、特殊染色、免疫表型、诊断与鉴别诊断。方法收集5例IBF的临床资料,观察其组织病理学形态、特殊染色及免疫组织化学特征,并进行随访。结果 5例中男性3例,女性2例,年龄为4~24个月,平均10.6个月。发生于足趾3例,手指2例。1例为复发病例,表现为右无名指及小指部有3个小结节。病变体积小、实性,被覆皮肤。组织学均表现为真皮和皮下纤维母细胞增生,伴多量胶原纤维形成。其最具特征性改变为梭形纤维母细胞胞质内有数量不等的嗜酸性包涵体。Masson三色染色包涵体呈深红色,免疫组化瘤细胞SMA、desmin和calponin均(+)。结论 IBF是一种罕见的良性肌纤维母细胞性肿瘤,其胞质内嗜酸性包涵体具有特征性。其具有局部复发潜能,扩大切除是治疗的最佳手段。

Purpose To summarize the clinicopathologic features,special stain,immunophenotype,diagnosis and differential diagnosis of inclusion body fibromatosis（ IBF）. Methods Five cases of IBF were included. The clinicopathological features,immunohistochemical findings and follow-up data were evaluated. Results The patients aged from 4 to 24 months. Three cases were male and two were female. The average age was 10. 6 months. Three cases accured in the toes and two cases were fingers. One case revealed a recurrent disease,showing three small nodules in the right ring finger and the small finger. The lesions were small,solid,and covered with skin. Histology showed fibroblast proliferation with multiple collagen fibers in dermal and subcutaneous. The most characteristic pathological changes were the eosinophilic inclusions in the cytoplasm of spindle fibroblasts. The Masson staining of inclusion body was dark red. The tumor cells were positive for SMA,desmin and calponin. Conclusions IBF is a rare benign myofibroblast tumor,and the most characteristic pathological changes were the eosinophilic inclusions in the cytoplasm of spindle fibroblasts. IBF has the potential of local recurrence,and enlargement resection is the best treatment.