[507] Type of Mammary Paget Disease Is Associated with Disease Free Survival

AE Gullett, VG Prieto, A Lopez, K Klein, B Arun, I Bedrosian, E Resetkova, Y Wu, N Sneige, CT Albarracin. UTHSC, Houston, TX; The University of Texas MDACC, Houston, TX

Background: Mammary Paget disease (MPD) is a relatively rare entity with a heterogeneous clinical presentation. Previous reports on this tumor have focused on their differential diagnosis and histogenesis on a limited population. This retrospective study was designed to evaluate the clinicopathologic features and outcomes of a cohort of patients diagnosed with MPD and seen at a single institution.
Design: A search for Paget in the nipple or skin of breast recovered 546 patients between 1985 and 2009. Pathology features, clinical presentation, radiologic findings, recurrence rates, and disease-free (DFS) and overall survival (OS) were available in 321 cases. At the time of submission information on all variables was obtained on 173 patients.
Results: Three groups of MPD patients were observed: 15 Paget without underlying breast carcinoma (PD), 67 Paget with DCIS (PDCIS), and 91 Paget with invasive cancer (PInv). The mean age at diagnosis for MPD was 54 yrs (range 21 to 90 yrs). Clinically, nipple discharge was present in 13% to 28% and nipple inversion was present in 0% to 12% of MPD. Skin changes were present in 60% of PD, 58% of PDCIS and 35% of PInv. Abnormal radiologic findings were found in 50% of PD, 81% of PDCIS and 90% of PInv. Palpable tumor was present in 9% of PD, 25% of PDCIS and 60% of PInv. Hormone receptor was positive in 51% to 54% in PDCIS and PInv. In tumors associated with MPD, HER2 was overexpressed in 58% of PInv and 67% of PDCIS. In Paget cells, HER2 was expressed in 50% of PD, 75% of PDCIS, and 64% of PInv. Coexpression of HER2 in both Paget cells and carcinoma was seen in 40% of PInv and 60% of PDCIS. There was no significant difference in OS within MPD. The median DFS was different among the three groups. Only 1 of 15 PD recurred. The median DFS was 9.9 yrs for PDCIS and 4.5 yrs for PInv. Further studies are required due to the small sample size of PD.
Conclusions: Skin changes were more likely to be present in PD and PDCIS as compared to PInv. However, abnormal radiologic findings and palpable tumor were most frequently found in PInv. The Paget cells in MPD expressed HER2 in the majority of cases. Moreover, HER2 was more likely to be positive in the nipple as compared to the underlying carcinoma. Although, OS was not significantly different within MPD, median DFS in PInv was shorter than PDCIS and PD. In conclusion, our results suggest that the types of MPD have different clinicopathologic characteristics and disease free survival.
Category: Dermatopathology

Monday, March 22, 2010 9:30 AM

Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 67, Monday Morning


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