Large Mammary Paget Disease without Underlying Breast Carcinoma

Abstract

Mammary Paget disease is a rare condition found in approximately 1% to 5% of all primary breast cancer and was recognized over 140 years ago. In 1874, Sir James Paget first described the clinical features of the disease as chronic eczematous skin of the nipple and areola which very often develops subsequent breast carcinoma within 1 to 2 years. 1 However, the etiology and treatment of this disease remain controversial.Approximately 50% of patients present with an associated mass. Over 90% of the women in this case have invasive breast carcinoma. 2,3 Cases with no palpable mass are more likely to be noninvasive breast cancer in about 66% to 68% of cases. 2,3 Only one other case of large mammary Paget disease without underlying breast cancer has been reported (Table 1). We report a rare case of extensive Paget disease without underlying breast carcinoma that underwent mastectomy with immediate pedicled transverse rectus abdominis myocutaneous (TRAM) flap breast reconstruction for treatment, to relieve symptoms and improve quality of life. CASE REPORTA 65-year-old Thai woman without a family history of breast cancer was referred to our hospital in July 2021. The disease had been present for 7 years. At first, it was seen as tiny red scaling patches, with an average size of 2 cm at the left nipple and areola. Despite topical and oral medicine, the patient's condition did not improve. She chose to seek alternative treatment and refused to get medical care. The illness grew worse throughout the course of the second through sixth years. She then visited the second hospital in her seventh year. An incisional skin biopsy revealed Paget disease. She was then referred to our department. The patient had a 14 × 19 cm moist erythematous, crusty, thickened, and scaling lesion on her left breast with contact bleeding. There was no mass to be seen (Fig. 1). During the clinical examination, a left axillary lymph node was discovered.Mammography of the affected breast was not possible due to extreme discomfort and bleeding. A left breast ultrasound revealed normal breast parenchyma with no tumor. An ultrasonography of the left axilla revealed numerous