Chung Connie, Forte Antonio Jorge V, Narayan Deepak, Persing John
Section of Plastic Surgery, Yale University School of Medicine, New Haven, CT, USA.
J Craniofac Surg. 2006 Nov;17(6):1210-5. doi: 10.1097/01.scs.0000231619.95263.a2.
Congenital nevi are benign neoplasms that are present at birth and composed of nevomelanocytes. Approximately 1-3% of all newborns have congenital pigmented nevi, and the number of nevi increases with age, peaking by late adolescence to 20-40 nevi in an individual. Giant congenital nevi are often defined as nevi that are greater than 20 cm in diameter in an adult, or nevi that occupy 2% or more of the body surface area. Histologically, nevi are transformed melanocytes, which are normally highly dendritic cells interspersed among basal keratinocytes. The genetic basis of these lesions is not known. Findings of a culture of melanocytes from such a lesion from a showed chromosome rearrangements involving 1p,12q, and 19p. The giant nevi might be associated to several diseases: neurocutaneous melanosis, diffuse lipomatosis, structural brain malformations, hypertrophy of skull bones, limb atrophy, skeletal asymmetry involving both soft tissue hyper-and hypoplasia, von Recklinghausen's disease and vitiligo. The risk of malignant change in giant nevi is probably the most contentious issue in its management. The consensus is that lesions are pre-malignant, but the purported incidence of malignancy varies wildly from 0-42%. Surgical excision remains the mainstay of treatment for large congenital melanocytic nevi, and most giant nevi are managed by staged excision and resurfacing with skin grafts or tissue expanders and flaps.
先天性痣是出生时就存在的良性肿瘤,由痣黑素细胞组成。所有新生儿中约1%-3%患有先天性色素痣,痣的数量随年龄增长而增加,在青春期后期达到峰值,个体身上有20-40颗痣。巨大先天性痣通常定义为成人直径大于20厘米的痣,或占体表面积2%或更多的痣。从组织学上看,痣是转化的黑素细胞,它们通常是高度树突状细胞,散布在基底角质形成细胞之间。这些病变的遗传基础尚不清楚。对一名患者此类病变的黑素细胞培养结果显示,染色体重排涉及1p、12q和19p。巨大痣可能与多种疾病有关:神经皮肤黑素沉着症、弥漫性脂肪瘤病、结构性脑畸形、颅骨肥大、肢体萎缩、涉及软组织增生和发育不全的骨骼不对称、冯雷克林霍增氏病和白癜风。巨大痣恶变的风险可能是其治疗中最具争议的问题。共识是这些病变是癌前病变,但据称的恶变发生率差异很大,从0%到42%不等。手术切除仍然是大型先天性黑素细胞痣治疗的主要方法,大多数巨大痣通过分期切除并用皮肤移植、组织扩张器和皮瓣进行表面修复来处理。