巨大先天性黑素细胞痣（GCMN）的主要诊断标准是直径大于20厘米。然而，其组织病理学起源、发病机制和GCMN进展尚不完全清楚。与其他中小型浅表病变不同，组织形态学评估对于GCMN病理分型、恶变评估和早期发现预后具有重要意义。本研究旨在探讨GCMN的病理特征，包括其卫星病变。23个巨痣和17个巨痣收集2018年至2020年上海市第九人民医院1~10岁儿童的“卫星病变”，进行组织学研究，评价其组织学形态。对所有观察和记录的数据进行统计分析。 23例GCMN原发性痣中，痣细胞主要分布在真皮区，有黑素细胞增生，真皮与表皮交界处存在痣巢。然而，在卫星痣中，发现了交界生长模式。此外，其他组织病理学特征，包括表皮轮廓、细胞形态和结构紊乱也显示出原发性痣和卫星痣之间的显着差异。我们证明主痣的先天性模式比卫星痣之一更明显，这表明主痣的先天性模式比卫星痣更明显。卫星痣和主痣可能比主痣稍晚出现。 “卫星痣”是由单独的遗传事件引起的。© 2023 作者。 《美容皮肤病学杂志》由 Wiley periodicals LLC 出版。

The major diagnostic criterion for the giant congenital melanocytic nevus (GCMN) is a size larger than 20 cm in diameter. However, the histopathological origin, pathogenesis, and GCMN progression are not yet completely clear. Unlike other medium or small superficial lesions, histomorphological evaluation is significant for GCMN pathological classification, malignant transformation assessment, and early detection of prognosis.This study aimed to investigate the pathological features of GCMN, including its satellite lesions.Twenty-three giant naevi and seventeen "satellite lesions" were collected from children aged 1 to 10 in Shanghai Ninth People's Hospital from 2018 to 2020. A histological study was conducted to evaluate their histological appearance. All the data observed and recorded data were statistically analyzed.In 23 cases of GCMN primary nevus, nevus cells were mainly distributed in the dermal region, with melanocyte proliferation and the presence of nevus nests at the dermal-epidermal junction. However, in satellite nevus, a junctional growth pattern was noted. Additionally, other histopathologic features, including epidermal contour, cell morphology, and architecture disorder also showed significant differences between primary nevus and satellite nevus.We demonstrated that the congenital pattern of the main nevus is more obvious than one of the satellite nevus, suggesting that the satellite nevus and the main nevus may occur slightly later than the main nevus. "Satellite nevus" happens as a result of a separate genetic event.© 2023 The Authors. Journal of Cosmetic Dermatology published by Wiley Periodicals LLC.