Paget's disease of the breast without associated ductal carinoma: Case report and review of the literature

Abstract

Objective: To present one case of Paget's disease (PD) of the breast without associated ductal carcinoma, and to conduct a review of the literature on its diagnosis and treatment. Materials and methods: A 59-year-old woman with a two-year history of a chronic erythematous, ulcerated, itchy lesion in the left nipple, initially treated dermatologically with topical steroids with no improvement, finally visits a primary healthcare institution in the city of Tunja, Colombia for diagnosis. A biopsy and histochemical testing were performed to make the diagnosis and the lesion was then treated with central quadrantectomy. The post-operative pathology testing was negative for malignancy, without recurrence at one-year followup. A search was conducted in the PubMed, SciELO, ProQuest, ScienceDirect and Google Scholar databases using the terms "Paget's Disease," "Breast Neoplasms," "Mammography," "Ultrasonography," Mammary," "Biopsy," and "Mastectomy," tracking bibliographic review articles, editorials, reports and clinical case series in English and Spanish, without time limitation. Results: Overall, 11 publications were included consisting of 5 case reports, 3 case series, 1 review of the literature based on a clinical case, and 2 letters to the editor describing cases related to Paget's disease of the nipple with no associated carcinoma. The diagnosis was based on the clinical suspicion of the disease and a biopsy of the lesion. Histochemistry was useful for the differential diagnosis: treatment options ranged from mastectomy to nipple-areolar complex resection. Radiotherapy has also been used. Conclusions: The diagnosis of Paget's disease with no associated ductal carcinoma is based on early clinical suspicion and histopathology of the nippleareolar complex. There is no consensus regarding surgical management of this lesion. Follow-up studies of this subgroup of patients treated with radiotherapy or conservative surgery are required.