Paget’s disease of the breast without associated ductal carcinoma: case report and review of the literature.

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: 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 follow-up. 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 nipple- areolar 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.