韩国巨大多发性先天性黑素细胞痣患者的临床特征和黑素瘤发病风险：一项全国性回顾性研究。

Clinical characteristics and risk of melanoma development from giant congenital melanocytic naevi in Korea: a nationwide retrospective study.

机构信息

Department of Dermatology, Chonnam National University Medical School, 8 Hak-Dong, Dong-gu, Gwangju, Korea.

出版信息

Br J Dermatol. 2012 Jan;166(1):115-23. doi: 10.1111/j.1365-2133.2011.10636.x.

DOI:10.1111/j.1365-2133.2011.10636.x

PMID:21923752

Abstract

BACKGROUND

Giant congenital melanocytic naevi (GCMN) are known risk factors for the development of melanoma. However, melanoma risk among Asians is rarely evaluated.

OBJECTIVES

To evaluate the clinical characteristics and risk of melanoma development from GCMN in Koreans, we performed a nationwide retrospective cohort study in Korea. GCMN were defined as those comprising ≥5% body surface area in children or measuring ≥20cm in adults.

METHODS

In total, 131 patients with GCMN were enrolled, with a mean age of 10·3years (range: birth-70years).

RESULTS

The posterior trunk was the most common site (67, 51·1%), followed by lateral trunk, anterior trunk, legs, both anterior and posterior trunk, buttocks, and arms. Satellite naevi were present in 69 cases (52·7%), and axial areas were more commonly involved in patients with satellite naevi than in those without satellite lesions. Atypical features such as rete ridge elongation and bridges were seen, and, among these, pagetoid spread and ballooning cell changes were more common in patients <4years old. Proliferative nodules were found in three cases. Melanomas had developed in three of 131 patients (2·3%; a 6-year-old girl, a 14-year-old girl and a 70-year-old man), and the incidence rate was 990 per 100000 person-years. Melanomas in these three patients consisted of two cutaneous melanomas and one extracutaneous meningeal melanoma.

CONCLUSIONS

We should be aware of melanoma development from GCMN, and lifelong follow-up is required due to the risk of melanoma arising in GCMN.

摘要

背景

巨大先天性黑素细胞痣（GCMN）是黑色素瘤发生的已知危险因素。然而，亚洲人群中黑色素瘤的发病风险很少被评估。

目的

为了评估韩国人群中 GCMN 发生黑色素瘤的临床特征和风险，我们在韩国进行了一项全国性的回顾性队列研究。GCMN 定义为儿童中占体表面积≥5%或成人中≥20cm 的痣。

方法

共纳入 131 例 GCMN 患者，平均年龄为 10.3 岁（范围：出生至 70 岁）。

结果

后躯是最常见的部位（67 例，51.1%），其次是侧躯干、前躯干、腿部、前后躯干、臀部和手臂。69 例（52.7%）存在卫星痣，卫星痣患者比无卫星病变患者更常累及轴区。存在非典型特征，如网嵴伸长和桥接，其中，匐行性扩展和气球样细胞改变在<4 岁的患者中更为常见。3 例发现增生性结节。131 例患者中有 3 例（2.3%；1 例 6 岁女孩、1 例 14 岁女孩和 1 例 70 岁男性）发生黑色素瘤，发病率为 990/100000 人年。这 3 例患者的黑色素瘤包括 2 例皮肤黑色素瘤和 1 例脑脊髓膜黑色素瘤。