目的：探讨分化型外阴上皮内瘤变( differentiated-type vulvar intraepithelial neoplasia, dVIN)的临床病理学特征、鉴别诊断、治疗及预后。方法回顾性分析6例dVIN的临床特点、病理形态学和免疫表型特征,并复习相关文献。结果患者均为女性,年龄53~80岁,平均62岁,临床特点大多表现为外阴白斑、激惹、瘙痒、疼痛、溃疡、触血。病理组织学特征表现：基底与副基底层显著增生,上皮角下延、吻合,细胞显著异型,核仁明显,核分裂活跃,伴异常角化；中、表层细胞高度分化,细胞间桥显著,伴明显的胞质嗜酸性改变,表层角化过度与角化不全；邻近上皮真皮水肿与胶原化,带状淋巴细胞浸润,表皮增生与角化过度。免疫表型：p53在dVIN中的阳性率为83．3%(5/6),p16不表达(0/6),dVIN基底层和副基底层细胞Ki-67增殖指数>90%。4例随访时间为6~36个月,平均17个月,其中1例术后9个月死亡,1例术后6个月复发,复发和死亡的2例同时或异时伴浸润性鳞状细胞癌,其余2例术后分别随访18个月和36个月,均无复发。结论 dVIN是一种少见的高级别外阴上皮内病变,具有高的、潜在进展危险性,p53、p16及Ki-67联合使用有助于dVIN的诊断。%Purpose To investigate the clinicopathologic characteristics, differential diagnosis, treatment and prognosis of differentia-ted-type vulvar intraepithelial neoplasia ( dVIN) . Methods Clinicopathologic findings and immunophenotypes of 6 cases diagnosed as“dVIN” were retrospectively analyzed, and the relevant literatures were also reviewed. Results 6 patients were all female ranged 53~80 years old with mean age of 62 years old. Clinical aspects included leukoplakia vulvar, pruitis, irritation, pain, ulcer and so on. The histopathological features were hyperplasia of basal and parabasal layer with elongation and anastomosing reteridges. Cells were marked atypia with obvious nucleoli, atypical mitosis, and dyskeratosis. In the middle and surface layer, cells were well differentiated with pronounced intercellular bridges, and eosinophilic cytoplasm, hyperkeratosis and parakeratosis. Oedema and band of infiltration of chronic inflammatory cells of subepidermal could been seen. Immuohistochemistry showed the expression rates of p53 and p16 in totally 6 cases were 83. 3% (5/6), 0 (0/6), respectively. The Ki-67 index was more than 90% in basal and parabasal cells. Four patients were followed up ( mean follow-up 17 months, range 6~36 months) , one patient died at 9 months later after surgery, another patient recurred at 6 months later after surgery, both of the 2 cases were all with invasive lesions after resection, and the rest two cases had no recur in 18 months and 36 months after surgery, respectively. Conclusion dVIN is a high grade squamous intraepithelial lesions of vulvar with low incidence rate, but had more risk of progression. p53, p16 and Ki-67 stain were useful in the diagnosis of dVIN.
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