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Primary signet-ring cell carcinoma (PSRCC) of the breast is a rare entity and classified under mucin producing tumors in the WHO classification. However, little is known about the clinicopathological characteristics and clinical outcomes of PSRCC as opposed to mucinous carcinoma. Eleven patients with PSRCC in our center from 1995 to 2010 were evaluated in this study, as compared to 50 cases of mucinous breast cancer (MBC) during the same period. The clinicopathologic features of PSRCC compared to MBC were assessed. Furthermore, overall survival (OS) and disease-free survival (DFS) were calculated at 5 years of follow up. Patients with PSRCC showed more frequent lymphatic metastasis, higher Ki67 labeling index and more advanced stage disease than that of MBC (P = 0.018, p = 0.023, P = 0.000, respectively), although there was no difference in age, tumor size, and ER, PR expression between PSRCC and MBC. In addition, PSRCC was associated with simultaneous vimentin upregulation and E-cadherin downregulation. The 5-year OS of PSRCC (54.5%) was significantly lower than that of MBC (88%) (P = 0.004). Similarly, the DFS of PSRCC was poorer than that of MBC significantly (5-year DFS: 27.3% vs. 80%, P = 0.000).   Conclusions  Our results confirmed the more aggressive behavior of PSRCC compared to MBC. This tumor is frequently associated with more frequent lymphatic metastasis, higher Ki67 labeling index, more advanced stage disease as well as simultaneous vimentin upregulation and E-cadherin downregulation. Different management guidelines should be considered for the two types.

Poorer Prognosis of Primary Signet-Ring Cell Carcinoma of the Breast Compared with Mucinous Carcinoma