栅状肌纤维母细胞瘤3例报道及文献复习

Palisaded myofibroblastoma:report of three cases and review of the literature

目的：观察栅状肌纤维母细胞瘤的临床病理特征，探索该肿瘤的组织来源及石棉样纤维的性质和来源。方法：对３ 例栅状肌纤维母细胞瘤进行组织形态学和免疫组织化学研究，结合文献对本病的诊断标准、鉴别要点及组织来源进行探讨。结果：栅状肌纤维母细胞瘤的组织学特点为梭形肿瘤细胞呈交叉束状、栅栏状排列，间质内有较多出血、散在的肥大细胞，肿瘤中出现石棉样纤维。免疫组化见肿瘤细胞及石棉样纤维星芒状突起中ａｃｔｉｎ、ｖｉｍｅｎｔｉｎ 呈阳性表达，ｄｅｓｍｉｎ、Ｓ１００ 、ＦａｃｔｏｒⅧ、ｃｙｔｏｋｅｒａｔｉｎ 呈阴性表达。结论：栅状肌纤维母细胞瘤是一种具有独特临床病理特征的肌纤维母细胞瘤，该肿瘤来源于肌纤维母细胞或特殊平滑肌细胞亚型。石棉样纤维中心为血管周围胶原的变性，星芒状纤维为增殖的肌纤维母细胞突出的中间丝和微丝。

Purpose To observe the clinical pathologic characteristics of palisaded myofibroblastoma, approach its histogenesis and the nature of the amianthoid fibers (AF). Methods Three cases of palisaded myofibroblastoma were studied by histopathology and immunohistochemistry, combining with the review of the literature. The diagnostic criteria, differential diagnosis and histogenesis of the disease were discussed. Results The lesions were histologically characterised by interlacing or palisaded fascilcles of spindle cells, interstitial hemorrhage, scattered mast cells and AF. The tumor cells and the stellate edges of the AF expressed actin and vimentin but lacked of S 100, desmin, Factor Ⅷ and cytokeratin expression. Conclusion Intronodal palisaded myofibroblastoma is a kind of distinctive clinicopathological myofibroblastoma. It originates from myofibroblasts or specialized smooth muscle cells. It is postulated that the core of the AF represents degraded interstial matrix around the vessels while the peripheral spokes are the result of vimentin and actin, directly shed by proliferating myofibroblasts.