浅表肢端纤维黏液瘤的临床病理特征

目的 探讨浅表肢端纤维黏液瘤(SAF)的临床病理学特点、免疫表型和鉴别诊断.方法 对1例发生于左手中指末端SAF的临床表现、组织形态和免疫学表型进行回顾性分析,并复习文献.结果 患者男,62岁.因左手中指背侧末端肿块伴疼痛就诊,曾有外伤史.术中见肿块近甲床,并深达骨膜.大体观察,肿块周界不清,直径约2 cm,切面呈灰白色,实性,质韧.镜下观察,肿瘤位于真皮层内,略呈分叶状.瘤细胞由梭形至星形纤维母细胞样细胞组成,呈杂乱状分布于黏液样基质内,局部区域可呈条束状或疏松的席纹状排列.黏液样基质内含有丰富的纤细血管,并可见较多散在的肥大细胞.瘤细胞异型性不明显或仅显示轻度的异型性,核分裂象罕见.肿瘤内也未见坏死.免疫组织化学标记显示,梭形和星形细胞表达波形蛋白、CD34和CD99,灶性表达CD10,不表达上皮细胞膜抗原、肌动蛋白、结蛋白和S-100蛋白.结论 SAF好发于成年人指趾末端.熟悉其临床病理特点则有助于与其他发生于指趾的软组织黏液性肿瘤相鉴别.临床上宜将SAF作完整性切除,以预防局部复发.

Superficial acral flbromyxoma of finger: report of a case with review of literature

Objective To study the clinicopathologic characteristics, immunophenotype and differential diagnosis of superficial acral fibromyxoma (SAF). Methods The clinical, pathologic and immunohistochemical features of a case of SAF occurring in left middle finger was studied, with review of literature. Results The patient was a 62-year-old male who presented with a solitary painful nodule located in the distal aspect of his left middle finger. The nodule lied close to the nail bed and deep to the underlying periosteum. Grossly, the tumor was poorly circumscribed, measured 2 cm in greatest dimension and had a greyish-white cut surface and rubbery consistency. On low-power examination, the tumor was centred in the dermis and displayed a vague lobular pattern. The tumor cells were spindled to stellate in shape and associated with myxoid matrix. Focal fnscicular or loose storiform patterns were also noted. A delicate vascular network was identified in the myxoid stroma. Mast cells were readily observed. On high-power examination, the tumor cells were relatively bland-looking and showed at most a mild degree of nuclear atypia. Mitotic figures were rare and coagulative tumor necrosis was absent. Immunohistochemical study showed that the tumor cells were positive for vimentin, CD34 and CD99. Focal staining for CDIO was also demonstrated. Other immunomarkers including actins, desmin and epithelial membrane antigen were negative. Conclusions SAF is a distinctive soft tissue tumor occurring mainly in the digits of adults. Awareness of this entity is helpful in distinguishing SAF from other myxoid soft tissue tumors occurring there. Complete excision with clear resection margins is the mainstay of treatment.