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蕈样肉芽肿合并恶性黑色素瘤和发育异常痣综合征。

Mycosis fungoides associated with malignant melanoma and dysplastic nevus syndrome.

作者信息

Pielop J A, Brownell I, Duvic M

机构信息

Baylor College of Medicine, Houston, TX, USA.

出版信息

Int J Dermatol. 2003 Feb;42(2):116-22. doi: 10.1046/j.1365-4362.2003.01697.x.

Abstract

BACKGROUND

The increased risk of second malignancies, including nonmelanoma skin cancers, in cutaneous T-cell lymphoma (CTCL) patients has been well documented. However, relatively few studies of malignant melanoma in CTCL patients have been reported.

METHODS

A database of 250 CTCL patients registered over a 3-year period was searched to identify patients with diagnoses of both mycosis fungoides (MF) and malignant melanoma.

RESULTS

We identified six cases of MF associated with malignant melanoma and one associated with dysplastic nevus syndrome, which is a marker of increased risk of melanoma. In four patients, melanoma was diagnosed along with or before MF. In the remaining two patients, MF was diagnosed prior to melanoma, although dysplastic nevi were noted at the time MF was diagnosed. These two patients received treatment for their MF (one with topical nitrogen mustard and another with radiation therapy and nitrogen mustard) prior to the histologic confirmation of melanoma. Six patients had early stages of MF (IA or IB), while one patient presented with simultaneous erythrodermic mycosis fungoides involving the lymph nodes as well as melanoma metastatic to the lymph nodes from an unknown primary.

CONCLUSION

There is an elevated prevalence of malignant melanoma in MF patients compared to the general US population (P < 0.00001) with a relative risk of 15.3 for observing malignant melanoma in MF patients (95% confidence interval 7.0-33.8). Possible pathologic links between the two diagnoses include effects of mycosis fungoides therapies, immunosuppression secondary to mycosis fungoides, and genetic alterations in the p16 tumor suppressor protein.

摘要

背景

皮肤T细胞淋巴瘤(CTCL)患者发生包括非黑色素瘤皮肤癌在内的第二恶性肿瘤的风险增加,这已得到充分记录。然而,关于CTCL患者发生恶性黑色素瘤的研究报道相对较少。

方法

检索了一个在3年期间登记的250例CTCL患者的数据库,以确定同时诊断为蕈样肉芽肿(MF)和恶性黑色素瘤的患者。

结果

我们识别出6例与恶性黑色素瘤相关的MF病例以及1例与发育异常痣综合征相关的病例,发育异常痣综合征是黑色素瘤风险增加的一个标志。在4例患者中,黑色素瘤与MF同时诊断或在MF之前诊断。在其余2例患者中,MF在黑色素瘤之前诊断,尽管在诊断MF时已注意到发育异常痣。这2例患者在黑色素瘤组织学确诊之前接受了MF治疗(1例接受局部氮芥治疗,另1例接受放射治疗和氮芥治疗)。6例患者处于MF早期阶段(IA或IB期),而1例患者同时出现红皮病型蕈样肉芽肿累及淋巴结以及黑色素瘤从未知原发灶转移至淋巴结。

结论

与美国普通人群相比,MF患者中恶性黑色素瘤的患病率升高(P < 0.00001),MF患者发生恶性黑色素瘤的相对风险为15.3(95%置信区间7.0 - 33.8)。这两种诊断之间可能的病理联系包括蕈样肉芽肿疗法的影响、蕈样肉芽肿继发的免疫抑制以及p16肿瘤抑制蛋白的基因改变。

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