Background Male breast cancer (BC) is rare, managed by extrapolation from female BC. The International Male BC Program aims to better characterize and manage this disease. We report the results of part I, a retrospective joint analysis of cases diagnosed during a 20-year period. Methods Patients with follow-up and tumor samples, treated between 1990 and 2010, in 93 centers/9 countries. Samples were centrally analyzed in three laboratories (the United Kingdom, the Netherlands and the United States). Results Of 1822 patients enrolled, 1483 were analyzed; 63.5% were diagnosed between 2001 and 2010, 57 (5.1%) had metastatic disease (M1). Median age at diagnosis: 68.4 years. Of 1054 M0 cases, 56.2% were node-negative (N0) and 48.5% had T1 tumors; 4% had breast conserving surgery (BCS), 18% sentinel lymph-node biopsy; half received adjuvant radiotherapy; 29.8% (neo)adjuvant chemotherapy and 76.8% adjuvant endocrine therapy (ET), mostly tamoxifen (88.4%). Per central pathology, for M0 tumors: 84.8% ductal invasive carcinomas, 51.5% grade 2; 99.3% estrogen receptor (ER)-positive; 81.9% progesterone receptor (PR)-positive; 96.9% androgen receptor (AR)-positive [ER, PR or AR Allred score ≥3]; 61.1% Ki67 expression low (<14% positive cells); using immunohistochemistry (IHC) surrogates, 41.9% were Luminal-A-like, 48.6% Luminal-B-like/HER-2-negative, 8.7% HER-2-positive, 0.3% triple negative. Median follow-up: 8.2 years (0.0-23.8) for all, 7.2 years (0.0-23.2), for M0, 2.6 years (0.0-12.7) for M1 patients. A significant improvement over time was observed in age-corrected BC mortality. BC-specific-mortality was higher for men younger than 50 years. Better overall (OS) and recurrence-free survival (RFS) were observed for highly ER+ (P = 0.001), highly PR+ (P = 0.002), highly AR+ disease (P = 0.019). There was no association between OS/RFS and HER-2 status, Ki67, IHC subtypes nor grade. Conclusions Male BC is usually ER, PR and AR-positive, Luminal B-like/HER2-negative. Of note, 56% patients had T1 tumors but only 4% had BCS. ER was highly positive in >90% of cases but only 77% received adjuvant ET. ER, PR and AR were associated with OS and RFS, whereas grade, Ki67 and IHC surrogates were not. Significant improvement in survival over time was observed.

中文翻译：

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男性乳腺癌的特征：EORTC 10085 / TBCRC / BIG / NABCG国际男性乳腺癌计划的结果。

背景技术男性乳腺癌（BC）很少见，可以通过女性BC的推断进行管理。国际男性不列颠哥伦比亚省计划旨在更好地表征和控制这种疾病。我们报告第一部分的结果，这是对20年内诊断出的病例进行的回顾性联合分析。方法在1990年至2010年之间，对93个中心/ 9个国家/地区的患者进行了随访和肿瘤标本治疗。在三个实验室（英国，荷兰和美国）集中分析了样品。结果共纳入1822例患者，分析1483例。在2001年至2010年之间，该病的诊断率为63.5％，其中57例（5.1％）患有转移性疾病（M1）。诊断中位年龄：68.4岁。在1054例M0病例中，淋巴结阴性（N0）为56.2％，T1肿瘤为48.5％；4％做过保乳手术（BCS），18％前哨淋巴结活检；一半接受了辅助放疗；29.8％（新）辅助化疗和76.8％辅助内分泌治疗（ET），主要是他莫昔芬（88.4％）。根据中心病理，对于M0肿瘤：84.8％导管浸润性癌，21.5.5％等级；99.3％雌激素受体（ER）阳性；孕激素受体（PR）阳性率为81.9％；96.9％的雄激素受体（AR）阳性[ER，PR或AR Allred评分≥3]；Ki67表达低61.1％（阳性细胞<14％）; 使用免疫组织化学（IHC）替代物时，Luminal-A样占41.9％，Luminal-B样/ HER-2-阴性占48.6％，HER-2阳性占8.7％，三重阴性为0.3％。中位随访：全部8.2年（0.0-23.8），M0 7.2年（0.0-23.2），M1患者2.6年（0.0-12.7）。观察到年龄校正的BC死亡率随时间显着改善。50岁以下男性的BC特异性死亡率更高。对于较高的ER +（P = 0.001），较高的PR +（P = 0.002），较高的AR +疾病（P = 0.019），观察到更好的总体（OS）和无复发生存（RFS）。OS / RFS与HER-2状态，Ki67，IHC亚型或等级之间没有关联。结论男性BC通常为ER，PR和AR阳性，管腔B样/ HER2阴性。值得注意的是，有56％的患者患有T1肿瘤，但只有4％的患者患有BCS。在超过90％的病例中，ER呈高度阳性，但只有77％的患者接受了辅助性ET。ER，PR和AR与OS和RFS相关，而等级，Ki67和IHC替代则不相关。随着时间的推移，观察到存活率显着提高。结论男性BC通常为ER，PR和AR阳性，管腔B样/ HER2阴性。值得注意的是，有56％的患者患有T1肿瘤，但只有4％的患者患有BCS。在超过90％的病例中，ER呈高度阳性，但只有77％的患者接受了辅助性ET。ER，PR和AR与OS和RFS相关，而等级，Ki67和IHC替代则不相关。随着时间的推移，观察到存活率显着提高。结论男性BC通常为ER，PR和AR阳性，管腔B样/ HER2阴性。值得注意的是，有56％的患者患有T1肿瘤，但只有4％的患者患有BCS。在超过90％的病例中，ER呈高度阳性，但只有77％的患者接受了辅助性ET。ER，PR和AR与OS和RFS相关，而等级，Ki67和IHC替代则不相关。随着时间的推移，观察到存活率显着提高。