青春期前型外阴纤维瘤临床病理学观察  

Prepubertal-type vulva fibroma： a clinicopathological study of two cases

作　　者：刘霞[1] 马遇庆[1] 王坚[2] 

机构地区：[1]新疆医科大学第一附属医院病理科,乌鲁木齐830054 [2]复旦大学附属肿瘤医院病理科复旦大学肿瘤医院肿瘤学系

出　　处：《中华病理学杂志》2010年第1期40-43,共4页Chinese Journal of Pathology

摘　　要：目的探讨青春期前型外阴纤维瘤（PVF）的临床病理学特征、免疫表型、诊断和鉴别诊断。方法分析2例PVF的临床表现、病理形态和免疫表型，并复习文献。结果1例发生于8．5岁幼女，1例发生于54岁成年女性，均以右侧大阴唇复发性肿块就诊。大体上，肿块不明显，切面呈灰白色，纤维组织样。组织学上，病变位于真皮层内，周界不清。细胞密度低，由稀疏的梭形纤维母细胞样细胞和大量的胶原纤维样间质组成。梭形细胞无异型性，核分裂象罕见。病变向皮下组织延伸，在邻近的脂肪组织之间、血管之间或在神经周围穿插性生长，形成类似错构瘤样的结构。免疫组织化学标记显示，梭形细胞主要表达波形蛋白，部分弱阳性表达CD34，不表达仪平滑肌肌动蛋白、肌特异性肌动蛋白、结蛋白、高分子钙调蛋白（h-caldesmon）、CD99、S-100蛋白、bcl-2、β-catenin、ER和PR。结论PVF是一种好发于青春期前幼女外阴的良性间叶性病变，偶可发生于成年人。PVF可能代表了一种外阴正常间叶组织的过度增生。临床上近1／3的病例如切除不净可发生局部复发，偶可自发性消退。Objective To study the clinicopathological features, immnophenotype and differential diagnosis of prepubertal-type vulva fibroma （PVF）. Method Clinical, radiological and pathological features of 2 cases of PVF were analyzed along with a literature review. Results Both patients were females of 8.5 and 54 years of age, presented with recurrent unilateral mass at the labium major respectively. Grossly, cut surface of the tumor appeared as the gray fibrous tissue without any definited lump detected. Histologically, the ill-circumscribed lesion located predominantly in the deep dermis with an extension into the subcutaneous tissue. They had a low cell density consisting of scattered spindle shaped fibroblast-like cells and a large amount of collagen fiber matrix, nuclear atypia not seen and mitotic figures scanty. The tumor cells extended downward under the epithelium and infiltrated between the fat tissue, nerve fibers as well as the capillaries making a lesion looked somewhat like a harmatoma. Immunohistochemically, the spindle cells were uniformly positive for vimentin, weakly positive for CD34, and negative for a-smooth muscle actin （SMA）, muscle specific actin （ HHF35）, desmin, h-caldesmon,CD99, S-100 protein, bcl-2, 13-catenin, estrogen and progesterone receptors. Conclusions PVF is a benign mesenchymal lesion with a predilection of involving the vulva of prcpubertal girls or adults in rare cases. PVF may represent an overgrowth of normal stromal tissue of vulva. Approximately one third of the tumors develop local recurrence due to incomplete excision, however, there is also occasionally spontaneous regression.

关 键 词：青春期 纤维瘤 外阴肿瘤 

分 类 号：R737.35[医药卫生—肿瘤]