Dores Graça M, Curtis Rochelle E, Toro Jorge R, Devesa Susan S, Fraumeni Joseph F
Medical Service, Department of Veterans Affairs Medical Center, Oklahoma City, OK 73104, USA.
Cancer. 2008 Dec 15;113(12):3372-81. doi: 10.1002/cncr.23963.
Sebaceous tumors of the skin occurring in association with an internal malignancy characterize Muir-Torre syndrome (MTS), a variant of hereditary nonpolyposis colon cancer (Lynch syndrome). To the authors' knowledge, only limited information exists regarding incidence patterns of sebaceous carcinoma (SC), and no prior study has quantified risks of associated cancers.
The authors calculated cutaneous SC incidence rates (IRs) and IR ratios in 9 US Surveillance, Epidemiology, and End Results program registries (1973-2003). Indirectly standardized incidence ratios and 95% confidence intervals (95% CIs) were calculated for subsequent cancers among 2-month survivors of SC and for subsequent SC after other primary cancers.
Among 664 cases of cutaneous SC, nearly 90% were diagnosed among whites (IR, 0.11 per 100,000 person-years), with significantly lower IR noted among blacks (IR, 0.04). Whereas eyelid SC IRs demonstrated no sex differences and stabilized in recent years, IRs of noneyelid SC predominated in men and rose steadily over time. Survivors of SC had a 43% (95% CI, 15%-76%) increased risk of subsequent cancer, and risk of SC was elevated by 52% (95% CI, 24%-84%) among survivors of other cancers. Whether before or after SC, the significant excesses of other primary cancers were limited to noneyelid SC. Patterns suggestive of genetic predisposition included >20-fold risks for early-onset (diagnosed in patients aged <50 years) SC associated with colon, pancreatic, ovarian, or uterine corpus cancers, whereas late-onset SC (diagnosed in patients aged > or =50 years) predisposed to ureter cancer.
This population-based study of cutaneous SC revealed an association with a spectrum of early-onset cancers consistent with MTS. Etiologic heterogeneity was suggested by differences between eyelid and noneyelid SC in incidence patterns and associated cancer risks.
与内部恶性肿瘤相关的皮肤皮脂腺肿瘤是穆尔-托雷综合征（MTS）的特征，MTS是遗传性非息肉病性结直肠癌（林奇综合征）的一种变体。据作者所知，关于皮脂腺癌（SC）的发病模式仅有有限的信息，且此前没有研究对相关癌症的风险进行量化。
作者计算了美国9个监测、流行病学和最终结果项目登记处（1973 - 2003年）的皮肤SC发病率（IRs）和IR比值。计算了SC 2个月幸存者中后续癌症以及其他原发性癌症后后续SC的间接标准化发病率比值和95%置信区间（95% CIs）。
在664例皮肤SC病例中，近90%在白人中被诊断出来（IR，每10万人年0.11），黑人中的IR显著较低（IR，0.04）。虽然眼睑SC的IR没有性别差异且近年来趋于稳定，但非眼睑SC的IR在男性中占主导且随时间稳步上升。SC幸存者患后续癌症的风险增加了43%（95% CI，15% - 76%），其他癌症幸存者患SC的风险升高了52%（95% CI，24% - 84%）。无论是在SC之前还是之后，其他原发性癌症的显著过量仅限于非眼睑SC。提示遗传易感性的模式包括早发型（在年龄<50岁的患者中诊断）SC与结肠癌、胰腺癌、卵巢癌或子宫体癌相关的风险>20倍，而晚发型SC（在年龄≥50岁的患者中诊断）易患输尿管癌。
这项基于人群的皮肤SC研究揭示了与一系列符合MTS的早发型癌症的关联。眼睑和非眼睑SC在发病模式和相关癌症风险上的差异提示了病因异质性。
