巨型先天性黑素细胞痣的研究现状与治疗策略

Research status and therapeutic strategy of giant congenital melanocytic nevus

巨型先天性黑素细胞痣(GCMN)是指出生时即存在的皮肤黑色素沉着性病变,其特征主要是病变面积大、黑素细胞浸润皮肤深处或皮下组织,具有独特的组织学表现。患者大多呈散发性,但也有家族性病例的报道,其发病机制目前尚不确切。由于严重影响外观,并可能发展为恶性黑色素瘤或神经皮肤黑变病(NCM),常对患者及其家属造成严重的心理负担。GCMN的治疗方式包括手术治疗和非手术治疗,不同方式均有其适应证和优缺点。目前对GCMN的预防及治疗效果尚不尽如人意,有研究初步表明药物治疗有望降低其并发症的发生率、改善患者的预后,可做为传统治疗的辅助疗法。

Giant congenital melanocytic nevus(GCMN)is defined as a melanotic lesion of the skin that is present at birth and is mainly characterized by a large area of the lesion,infiltration of melanocytes deep in the skin or subcutaneous tissue,with unique histological findings.Most of the patients are sporadic,but familial cases have also been reported,its pathogenesis is still unclear.It often places a serious psychological burden on patients and their families because it severely affects the appearance and may develop into malignant melanoma or neurocutaneous melanosis(NCM).The treatment methods of GCMN include surgical treatment and non-surgical treatment.Different methods have their indications and advantages and disadvantages.The prevention and treatment effect of GCMN are not satisfactory at present.Some studies have preliminarily shown that drug treatment is expected to reduce the incidence of complications and improve the recovery of patients,which can be used as an adjuvant therapy of traditional treatment methods.