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[散发性和家族性发育异常痣细胞综合征中多发性黑色素瘤发病率增加]

[Increased incidence of multiple melanoma in sporadic and familial dysplastic nevus cell syndrome].

作者信息

Sigg C, Pelloni F, Schnyder U W

机构信息

Dermatologische Klinik, Universitätsspital Zürich.

出版信息

Hautarzt. 1989 Sep;40(9):548-52.

PMID:2807914
Abstract

In 280 melanoma patients all data concerning familial and personal history, histology, and therapy were verified. All patients underwent total-body skin examination to check for the presence of dysplastic nevus syndrome (DNS). In 257/280 patients (91.8%) solitary melanomas were found, while in 23/280 patients (8.2%) multiple melanomas occurring simultaneously or consecutively were ascertained. Surprisingly, among the 12/280 patients (4.2%) with familial variants of melanoma, multiple melanomas were not found in a increased frequency. In patients with DNS (regardless of whether sporadic or familial) the frequency of multiple melanomas is higher: in patients with solitary melanomas DNS was found in 27/257 (10.5%), while in patients with multiple melanomas DNS was diagnosed in 11/23 (47.8%) (P less than 0.0005). In both groups (solitary and multiple melanomas) the mean age of patients with DNS was around 10 years lower. The frequency of additional primary malignancies in patients with cutaneous melanomas was 8.6%, and did not vary according as whether patients had solitary or multiple melanomas with or without DNS.

摘要

对280例黑色素瘤患者的家族史、个人史、组织学及治疗相关的所有数据进行了核实。所有患者均接受了全身皮肤检查,以检查是否存在发育异常痣综合征(DNS)。在280例患者中,257例(91.8%)发现为单发黑色素瘤,而23例(8.2%)为同时或先后发生的多发黑色素瘤。令人惊讶的是,在280例中有12例(4.2%)黑色素瘤家族变异患者中,多发黑色素瘤的发生率并未增加。在DNS患者中(无论散发性还是家族性),多发黑色素瘤的发生率更高:单发黑色素瘤患者中27/257(10.5%)发现有DNS,而多发黑色素瘤患者中11/23(47.8%)诊断有DNS(P<0.0005)。在两组(单发和多发黑色素瘤)中伴有DNS的患者平均年龄均低约10岁。皮肤黑色素瘤患者中其他原发性恶性肿瘤的发生率为8.6%,且无论患者是单发还是多发黑色素瘤,有无DNS,该发生率均无差异。

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