Primary signet ring cell carcinoma of the breast: A rare entity with unique biological behavior-A clinical study based on pure signet ring cell carcinoma cohort
Primary signet ring cell carcinoma of the breast: A rare entity with unique biological behavior-A clinical study based on pure signet ring cell carcinoma cohort
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乳腺癌原发性印戒细胞癌：具有独特生物学行为的罕见实体——基于纯印戒细胞癌队列的临床研究
DOI：
10.1016/j.prp.2020.152948
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发表时间：
2020-06-01
影响因子：
2.8
通讯作者：
Liu, Fangfang
中科院分区：
文献类型：
--
作者：
Wang, Tong;Shen, Beibei;Liu, Fangfang
背景资料：乳腺原发性印戒细胞癌（SRCC）是一种罕见的肿瘤，英文文献中仅有少数病例报道。我们总结了一个相对较大的队列的纯乳腺SRCCs的临床病理特征为第一时间。方法：我们回顾了23例纯乳腺SRCCs的中位随访时间为70 month.Results：3例患者有双侧原发性乳腺癌（BC），其中2例有双侧纯SRCCs的病历。30%的患者有恶性肿瘤家族史，其中2例为双侧BC。恶性钙化在35.3%的乳腺X线摄影图像中观察到。26.1%的病例在显微镜下发现多灶性病变。63.6 45.5%的病例为Ⅲ期，69.6%的病例Ki-67指数高值，34.8%的病例为三阴性亚型。19.0局部复发率为%，远处转移率为52.6%。有阳性肿瘤家族史和随访资料的5例患者中有4例复发。5年总生存率为73.7%，5年无复发生存率为54.3%，5年乳腺癌特异性生存率为78.3%。新辅助化疗可考虑，保乳手术应慎重选择，腋窝淋巴结清扫可能是必要的。高阳性肿瘤家族史率和高双侧发病率对预后有不良影响，表明SRCC具有独特的遗传负担。
Background: Primary signet ring cell carcinoma (SRCC) of the breast is a rare entity, and only a few case reports of the pure SRCC cases could be found in English literatures. We summarized the clinicopathological characteristics of a relatively large cohort of pure breast SRCCs for the first time.Methods: We reviewed the medical records of 23 cases of pure breast SRCC with a median follow-up time of 70 months.Results: Three patients had bilateral primary breast cancer (BC) and two of them had bilateral pure SRCCs. 30 % of patients had the malignant tumor family history including two bilateral BC patients. Malignant calcification was observed in 35.3 % of mammography images. Multifocal lesions were microscopically found in 26.1 % of cases. 63.6 % of the cases had lymph node metastasis, 45.5 % were classified as Stage III, 69.6 % had high value of Ki-67 index, and 34.8 % were triple negative subtype. 19.0 % of patients had local recurrence, and 52.6 % had distant metastasis. Four in five patients with positive tumor family history and follow-up data had relapse of SRCC. The 5-year overall survival rate was 73.7 %, the 5-year relapse-free survival rate was 54.3 %, and the 5-year breast cancer specific survival rate was 78.3 %.Conclusion: Pure SRCC of the breast showed an aggressive behavior. Neoadjuvant chemotherapy could be considered, breast-conserving surgery should be prudently chosen, and axillary lymph node dissection may be necessary. The high rate of positive tumor family histories and high bilateral incidence, which showed an adverse effect on prognosis, indicate the unique genetic burden of SRCC.