Association of melanoma and neurocutaneous melanocytosis with large congenital melanocytic naevi-results from the NYU-LCMN registry
Association of melanoma and neurocutaneous melanocytosis with large congenital melanocytic naevi-results from the NYU-LCMN registry
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DOI：
10.1111/j.1365-2133.2005.06316.x
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发表时间：
2005-03-01
影响因子：
10.3
通讯作者：
Polsky, D
中科院分区：
文献类型：
--
作者：
Hale, EK;Stein, J;Polsky, D
背景先天性大黑色素细胞痣（LCMN）发生于子宫内，大约每20000名新生儿中就有1例，与皮肤黑色素瘤、软脑膜黑色素瘤和神经皮肤黑色素细胞增多症（NCM）的风险显著增加相关。并估计这些患者患黑色素瘤和NCM的风险。方法对纽约大学登记处登记的205名LCMN患者进行了研究。对其中170例患者进行了前瞻性随访。其余35例患者在进入登记研究时患有黑色素瘤（n = 6），或随访信息不足（n = 29）。结局指标为黑色素瘤和NCM的发生率。这些结果和临床协变量（LCMN的解剖位置，大小的LCMN，卫星病变的数量，黑色素瘤家族史，患者性别和治疗）之间的关联进行了assessed.Results四个170（2.3%）前瞻性随访的患者发展黑色素瘤，代表一个标准化的发病率为324。在整个队列（n = 205）中，卫星痣数量增加与黑色素瘤的发生（P = 0.04）和NCM的存在（P = 0.06）之间存在相关性。与未发生这些疾病的患者相比，发生黑色素瘤（49与39 cm）和NCM（55与46 cm）的患者中LCMN直径中位数较大。结论在LCMN患者中，卫星病变数量增加和LCMN直径较大与黑色素瘤和NCM相关。
Background Large congenital melanocytic naevi (LCMN), which develop in utero and are present in approximately one in 20 000 newborns, are associated with markedly increased risks of cutaneous melanoma, leptomeningeal melanoma and neurocutaneous melanocytosis (NCM).Objectives This study examined clinical characteristics associated with melanoma and NCM among patients with LCMN, and estimated the risk of developing melanoma and NCM in these patients.Methods Two hundred and five LCMN patients enrolled in the New York University registry were studied. One hundred and seventy of these patients were followed prospectively. The remaining 35 patients had either melanoma at the time of entry into the registry (n = 6), or had insufficient follow-up information (n = 29). The outcome measures were the occurrence of melanoma and NCM. The associations between these outcomes and the clinical covariates (anatomical location of the LCMN, size of the LCMN, number of satellite lesions, family history of melanoma, patient sex and treatment) were assessed.Results Four of 170 (2.3%) prospectively followed patients developed melanomas, representing a standardized morbidity ratio of 324. Among the entire cohort (n = 205), there were associations between increasing numbers of satellite naevi and the occurrence of melanoma (P = 0.04), and the presence of NCM (P = 0.06). Compared with patients who did not develop these diseases, median LCMN diameters were larger among patients who developed melanoma (49 vs. 39 cm) and NCM (55 vs. 46 cm).Conclusions In LCMN patients, increasing numbers of satellite lesions and larger LCMN diameters are associated with melanoma and NCM.