RESUMEN
Paget's disease of the breast is rare among breast cancers. Secondary Paget's disease of the breast presenting as local recurrence is even rarer, with limited published information on the overall prevalence. In this report, we present a case of secondary Paget's disease of the breast presenting as a pruritic rash and skin changes with normal imaging. This patient's case was unique as her presentation involved invasive Paget's disease of the breast presenting as a local recurrence, with a diffuse rash covering the entirety of the right breast including the nipple-areolar complex, pathology examination showing dermal invasion, and a 20-year time interval between her initial treatment and presentation at our institution. Furthermore, diagnostic mammogram and breast MRI revealed no underlying suspicious findings within the breast tissue. In this case, the patient benefitted from mastectomy with removal of the affected skin, resulting in a clear margin and clinically favorable outcome. The patient did well postoperatively, did not receive any systemic adjuvant treatment, and is now under surveillance. Currently, there is insufficient data on the incidence of diffuse Paget's disease of the breast with dermal invasion. It is important to recognize this atypical presentation to ensure timely diagnosis and treatment of affected patients.
RESUMEN
PURPOSE: The increased breast cancer risk conferred by a diagnosis of lobular carcinoma in situ (LCIS) is poorly understood. Here, we review our 29-year longitudinal experience with LCIS to evaluate factors associated with breast cancer risk. PATIENTS AND METHODS: Patients participating in surveillance after an LCIS diagnosis are observed in a prospectively maintained database. Comparisons were made among women choosing surveillance, with or without chemoprevention, and those undergoing bilateral prophylactic mastectomies between 1980 and 2009. RESULTS: One thousand sixty patients with LCIS without concurrent breast cancer were identified. Median age at LCIS diagnosis was 50 years (range, 27 to 83 years). Fifty-six patients (5%) underwent bilateral prophylactic mastectomy; 1,004 chose surveillance with (n = 173) or without (n = 831) chemoprevention. At a median follow-up of 81 months (range, 6 to 368 months), 150 patients developed 168 breast cancers (63% ipsilateral, 25% contralateral, 12% bilateral), with no dominant histology (ductal carcinoma in situ, 35%; infiltrating ductal carcinoma, 29%; infiltrating lobular carcinoma, 27%; other, 9%). Breast cancer incidence was significantly reduced in women taking chemoprevention (10-year cumulative risk: 7% with chemoprevention; 21% with no chemoprevention; P < .001). In multivariable analysis, chemoprevention was the only clinical factor associated with breast cancer risk (hazard ratio, 0.27; 95% CI, 0.15 to 0.50). In a subgroup nested case-control analysis, volume of disease, which was defined as the ratio of slides with LCIS to total number of slides reviewed, was also associated with breast cancer development (P = .008). CONCLUSION: We observed a 2% annual incidence of breast cancer among women with LCIS. Common clinical factors used for risk prediction, including age and family history, were not associated with breast cancer risk. The lower breast cancer incidence in women opting for chemoprevention highlights the potential for risk reduction in this population.