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关于发育异常痣综合征患者的痣和黑色素瘤

On naevi and melanomas in dysplastic naevus syndrome patients.

作者信息

Crijns M B, Bergman W, Berger M J, Hermans J, Sober A J

机构信息

Department of Dermatology, University Hospital Leiden, The Netherlands.

出版信息

Clin Exp Dermatol. 1993 May;18(3):248-52. doi: 10.1111/j.1365-2230.1993.tb02179.x.

Abstract

Cutaneous melanoma may occur as isolated, so-called 'sporadic' cases or in association with multiple atypical naevi and in familial clusters, in which case it is referred to as the familial dysplastic naevus syndrome (DNS). In this retrospective study (a) the number and body distribution of naevocytic naevi and (b) the body distribution of malignant melanoma (MM) in individuals with familial DNS were compared in order to study their association. In 45 patients with familial DNS aged 20-39 years naevus counts on trunk and lower extremities were compared with melanoma data and distributions from a second group of 43 patients from the same DNS families aged 12-66 years. Men had significantly more naevi of a size > or = 2 mm or > or = 5 mm on the back than women (P = 0.02). Women showed a tendency towards a greater number of naevi on the lower extremities than men, but in women no significant difference in naevi between the lower extremities and the back was found. The total number of naevi on the trunk and lower extremities in familial DNS patients was higher than that in the general population. In conclusion, it was found that predilection sites for melanoma in familial DNS patients of both sexes correspond with the distribution of naevi; in males naevi and melanoma counts and percentage distributions were higher on the back, in females both the back and the lower extremities were affected. These findings strongly suggest an association between naevus distribution and melanoma occurrence and site in familial DNS, analogous to earlier reports on sporadic melanoma.

摘要

皮肤黑色素瘤可能以孤立的、所谓“散发性”病例的形式出现,也可能与多个非典型痣以及家族性聚集相关,在后一种情况下,它被称为家族性发育异常痣综合征(DNS)。在这项回顾性研究中,为了研究家族性DNS患者中痣细胞痣的数量和身体分布以及恶性黑色素瘤(MM)的身体分布之间的关联,对二者进行了比较。在45名年龄在20 - 39岁的家族性DNS患者中,将躯干和下肢的痣计数与来自同一DNS家族的另一组43名年龄在12 - 66岁患者的黑色素瘤数据及分布进行了比较。男性背部大小≥2毫米或≥5毫米的痣明显比女性多(P = 0.02)。女性下肢的痣数量有多于男性的趋势,但女性下肢和背部的痣数量没有显著差异。家族性DNS患者躯干和下肢的痣总数高于一般人群。总之,研究发现,家族性DNS患者中,无论男女,黑色素瘤的好发部位与痣的分布相对应;男性背部的痣和黑色素瘤计数及百分比分布更高,女性背部和下肢均受影响。这些发现有力地表明,家族性DNS中痣的分布与黑色素瘤的发生及部位之间存在关联,这与之前关于散发性黑色素瘤的报道类似。

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