先天性色素痣伴增生性结节10例临床病理分析

Clinicopathological features of 10 cases of congenital melanocytic nevi complicated by proliferative nodules

目的探讨先天性色素痣伴增生性结节的临床特点及组织病理特征。方法收集第四军医大学西京皮肤医院2015—2019年经临床和病理确诊的10例先天性色素痣伴增生性结节患者的临床及病理资料,并进行回顾性分析。结果10例患者年龄2~45岁(平均15岁),9例增生性结节发生于婴儿,1例发生于成人。皮损位于四肢4例,头面部3例,躯干2例,躯干及四肢同时受累1例。临床表现为黑色斑片或斑块中出现1个或多个结节,6例增生性结节为多发,4例为单发,单个结节直径0.2~1.5 cm,仅1例出现溃疡。组织病理检查显示增生性结节内黑素细胞均存在成熟现象,核分裂象少,细胞无明显异型性,无坏死现象,免疫组化检查显示痣细胞弥漫表达Melan-A,不表达或仅部分表达HMB45,Ki67增殖指数<5%。结论先天性色素痣伴增生性结节可发生于四肢、头面部及躯干;临床表现为原先天性色素痣皮损上的单发或多发结节;病理上增生性结节内黑素细胞可见成熟现象,免疫组化HMB45及Ki67染色有助于诊断,其预后有待长期随访。

Objective To investigate clinical and histopathological features of congenital melanocytic nevi(CMN)complicated by proliferative nodules(PN).Methods Ten patients with clinically and pathologically confirmed CMN complicated by PN were collected from Department of Dermatology,the Fourth Military Medical University from 2015 to 2019,and their clinical and pathological data were analyzed retrospectively.Results The 10 patients were aged from 2 to 45 years,with an average age of 15 years.Nine patients developed PN in infancy,and 1 in adulthood.The skin lesions were located on the extremities in 4 cases,on the head and face in 3 cases,and on the trunk in 2 cases,and the trunk and extremities were both involved in 1 case.Skin lesions clinically manifested as 1 or more nodules arising in black patches or plaques.Six patients presented with multiple PN,4 with solitary PN,with the diameter of a single nodule being 0.2-1.5 cm,and only 1 case presented with ulcers.Histopathological examination showed mature melanocytes in the PN,with few mitotic figures,no obvious cytological atypia,and no necrosis.Immunohistochemical study showed that nevus cells diffusely expressed Melan-A,but did not express or partially expressed HMB45,and the Ki67 proliferation index was below 5%.Conclusion CMN complicated by PN can occur on the extremities,head,face,and trunk,clinically manifesting as solitary or multiple nodules on pre-existing CMN;histopathologically,mature melanocytes can be observed in PN,immunohistochemical staining for HMB45 and Ki67 can facilitate the diagnosis,and its prognosis needs long-term follow-up.