自发性孤立性肠系膜纤维瘤病2例报道及文献复习

目的探讨自发性孤立性肠系膜纤维瘤病(SIFM)的临床病理特点。方法通过形态学和免疫组化对2例SIFM进行分析并复习相关文献。结果肿块均位于小肠系膜，病变无包膜，切面灰白、暗红色，质地韧，编织状。镜下肿瘤细胞呈梭形，似纤维母细胞，排列成束状，相互交错，可形成大量胶原纤维；细胞核无异型性。肿瘤向肠壁肌层及周围组织浸润性生长。病变无坏死。增生的梭形细胞Vim、Des( )，而CD34、CD117、actin和S-100均(-)。术后随访无复发。结论虽然SIFM具有侵袭性生长的生物学特点，但若切除不彻底易复发，但是并不转移。

Spontaneous isolated fibromatosis of mesentery:two cases report with review of the literature

Objective To explore the clinicopathologic features of spontaneous isolated fibromatosis of mesentery (SIFM) . Methods Two cases of SIFM were studied with morphological and immunohistochemical approaches. Results Both tumors lo-cated in the mesentery. Macroscopically, the tumors showed pale gray to dark red in color on cut surface, with tenant resistance and without capsulation. Microscopically, the neoplasm was composed of blandeness spindle cells which arranged in facular pattern on a collagnous background. There were abundant collagen bundles in stroma. There were no mitotic figure and necrosis found. The tumors infiltrated muscular layer of the bowel wall and surrounding tissue. In one case, the lesion showed myxoid change and small blood vessels were dilated in stroma, which were surrounded by some lymphocytes. Immunohistochemical staining revealed that tumor cells were positively reacted with vimentin and desmin, but negatively with CD34, CD117, smooth muscle actin and S-100 antibodies. Follow-up data of both patients revealed no recurrence. Conclusion Even though SIFM has biologic behaviors of aggressive growth and highly recurrence rate if not resected completely, the tumor never metastasizes.