[496] Pathological and Clinical Characteristics of Mammary Paget Disease: 25-Year Experience from a Major Tertiary Referral Center

Ashley E Gullett, Nour Sneige, Victor G Prieto, Catherine M Kelly, Roland L Bassett, Erika Resetkova, Xiuzhen Duan, Yong Li, Daniel Rosen, Yun Wu, Lei Huo, Kimberly Klein, Isabelle Bedrosian, Banu Arun, Kelly Hunt, Constance T Albarracin. The University of Texas at Houston, Houston, TX; M.D.Anderson Cancer Center, Houston, TX

Background: Mammary Paget disease (MPD) is a rare entity. Previous reports have been limited by the small numbers of patients included. This retrospective study was designed to evaluate the clinicopathologic features of MPD and the outcomes of patients diagnosed with MPD at our institution over a 25-year period.
Design: We retrospectively examined MPD cases in our institution from 5/3/85 to 1/13/10. Available pathological characteristics, clinical presentation, radiological characteristics and outcome data were recorded.
Results: We identified 322 patients with MPD; 16 (5%) Paget without underlying breast carcinoma (PD), 97 (30%) Paget with DCIS (PDCIS) and 204 (63%) Paget with invasive ductal carcinoma (PIDC). Five MPD cases associated with lobular carcinoma were excluded. The median age at diagnosis of MPD was 52 years (range, 24 to 90). Skin changes were present in 92% (11/12) PD cases, 69% (46/67) PDCIS and 44% (61/139) PIDC (p<0.0001). Palpable tumor and abnormal mammograms were more likely to be found in PIDC (p<0.0001). Hormone receptor status was positive in 46% (20/44) PDCIS and 53% (81/153) PIDC tested. More than half of MPD-associated tumors were HER2-positive, 79% (19/24) and 61% (68/112) for PDCIS and PIDC, respectively. HER2 status was available for both Paget cells and underlying IDC or DCIS in 25 cases. All HER2-positive PDCIS and PIDC were associated with HER2-positive Paget cells (13/13). In contrast, HER2-negative PDCIS and PIDC were associated with HER-2 negative Paget cells in 67% (8/12) of cases tested and with HER2-positive Paget cells in 33% (4/12) of cases tested. Outcome data showed no locoregional or distant breast cancer recurrences in the fully excised PD group after 15 years of follow-up. There were more locoregional (p=0.004) and distant recurrences (p<0.0001) in patients with PIDC as compared to PDCIS.
Conclusions: Most MPDs were associated with an underlying cancer. PDCIS and PIDC are heterogenous tumors with regard to hormonal and HER2 status. HER2-positive IDC was associated with HER2-positive Paget cells in all cases with HER2 testing of both lesions available. MPD without an underlying carcinoma was rare and was not associated with recurrence, provided the lesion was completely excised. The three major groups of MPD have different clinicopathologic characteristics and survival rates, suggesting that these features can be incorporated into the management of MPD. Future work will compare PDCIS and PIDC to DCIS and IDC, adjusting for stage and treatment.
Category: Dermatopathology

Monday, March 19, 2012 11:00 AM

Platform Session: Section F, Monday Morning


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