Millar Barbara-Ann M, Kerba Marc, Youngson Bruce, Lockwood Gina A, Liu Fei-Fei
Department of Radiation Oncology, Princess Margaret Hospital, University of Toronto, University Health Network, Ont., Canada.
Breast Cancer Res Treat. 2004 Oct;87(3):225-32. doi: 10.1007/s10549-004-8693-z.
Adenoid cystic carcinoma (ACC) of the breast is a rare breast cancer variant and optimal management is unclear. A review of this unusual tumour was performed at our Institution, to assess the role of breast conservation in the management of this disease.
A review of all cases of ACC of breast (1960-2000) treated at Princess Margaret Hospital (PMH) was undertaken. Information was collected on age at diagnosis, presenting features, tumour size and treatment modalities. Treatment outcomes were evaluated.
Eighteen female and one male patient were identified. Median age at diagnosis was 58 years (range 35-76 years). Four patients had lymph-node positive disease at presentation; the single male patient presented with metastatic disease. Surgery was either a lumpectomy (10 cases) or a simple, radical or modified radical mastectomy (9 patients). Nine of 19 patients received adjuvant radiotherapy (RT). The median follow-up time was 14 years; the recurrence rate at 10 years was 31% (95% CI 7-54%) with a range in time of recurrence from 2.3 to 11.9 years. Seven recurrences were identified (4 local, 1 regional, 2 metastatic). Two of these patients developed metastatic spread and died. Six of the 19 cases went on to develop second malignancies of whom four died. Among the 18 female patients, the 10-year overall (OS), cause-specific (CSS), and relapse free survival (RFS) rates were 75, 100, and 46% respectively.
ACC of the breast has a relatively prolonged natural history, and responds well to conservative management at presentation, with good outcome, even following local recurrence.
乳腺腺样囊性癌（ACC）是一种罕见的乳腺癌亚型，最佳治疗方案尚不清楚。我们机构对这种不寻常的肿瘤进行了回顾，以评估保乳治疗在该疾病管理中的作用。
对玛格丽特公主医院（PMH）1960年至2000年期间治疗的所有乳腺ACC病例进行了回顾。收集了诊断时的年龄、临床表现、肿瘤大小和治疗方式等信息，并评估了治疗结果。
共识别出18例女性和1例男性患者。诊断时的中位年龄为58岁（范围35 - 76岁）。4例患者初诊时伴有淋巴结阳性疾病；唯一的男性患者初诊时即有转移性疾病。手术方式包括乳房肿块切除术（10例）或单纯乳房切除术、根治性乳房切除术或改良根治性乳房切除术（9例）。19例患者中有9例接受了辅助放疗（RT）。中位随访时间为14年；10年复发率为31%（95%CI 7 - 54%），复发时间范围为2.3至11.9年。共发现7例复发（4例局部复发，1例区域复发，2例远处转移）。其中2例患者发生远处转移并死亡。19例患者中有6例发生了第二原发恶性肿瘤，其中4例死亡。在18例女性患者中，10年总生存率（OS）、疾病特异性生存率（CSS）和无复发生存率（RFS）分别为75%、100%和46%。
乳腺腺样囊性癌具有相对较长的自然病程，初诊时对保守治疗反应良好，即使局部复发后预后也较好。
