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家族性发育异常痣综合征。自然病史及筛查对预后的影响。荷兰九个家族的研究。

The familial dysplastic nevus syndrome. Natural history and the impact of screening on prognosis. A study of nine families in the Netherlands.

作者信息

Vasen H F, Bergman W, van Haeringen A, Scheffer E, van Slooten E A

机构信息

Foundation for the Detection of Hereditary Tumours, Utrecht, The Netherlands.

出版信息

Eur J Cancer Clin Oncol. 1989 Feb;25(2):337-41. doi: 10.1016/0277-5379(89)90027-8.

DOI:10.1016/0277-5379(89)90027-8
PMID:2702987
Abstract

Since 1982, nine families with the dysplastic nevus syndrome have been identified in the Leiden area (The Netherlands). A total of 50 primary melanomas were diagnosed in 38 persons. Nineteen of these melanomas had been diagnosed before the start of the screening programme (category I), 11 were detected at the initial examination of the families (category II), and 20 were found during the course of follow-up (category III). To assess the effect of screening, we compared these categories with respect to the developmental stage of the melanomas. One of the 19 melanomas in category I, two of the 11 in category II and seven of the 20 in category III were melanoma in situ. The average thickness of the invasive melanomas in categories I, II and III was 1.75, 0.80 and 0.54 mm respectively. Sixteen of the 19 melanomas in category I (84%) were Clark III or IV, whereas 15 of the 20 melanomas in category III (75%) were Clark I or II. From these findings it may be concluded that screening can lead to the detection of melanomas at an earlier stage, which in turn can permit curative treatment and improvement of both prognosis and life expectancy. The need for supervision based on central registration of affected families to guarantee the continuity of screening is discussed.

摘要

自1982年以来,在荷兰莱顿地区已确认9个患有发育异常痣综合征的家庭。共38人被诊断出50例原发性黑色素瘤。其中19例黑色素瘤在筛查项目开始前就已确诊(I类),11例在对这些家庭进行初次检查时被发现(II类),20例在随访过程中被发现(III类)。为评估筛查效果,我们比较了这些类别黑色素瘤的发展阶段。I类19例黑色素瘤中有1例,II类11例中有2例,III类20例中有7例为原位黑色素瘤。I类、II类和III类侵袭性黑色素瘤的平均厚度分别为1.75、0.80和0.54毫米。I类19例黑色素瘤中有16例(84%)为克拉克III级或IV级,而III类20例黑色素瘤中有15例(75%)为克拉克I级或II级。从这些发现可以得出结论,筛查可导致在更早阶段发现黑色素瘤,进而可以进行治愈性治疗并改善预后和预期寿命。文中还讨论了基于对受影响家庭进行中央登记以保证筛查连续性的监督必要性。

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