Extramammary Paget's disease involving the axilla: case series and literature review

Abstract

Background: Axillary extramammary Paget's disease (EMPD) is a rare condition with only a few cases reported in the literature.

Methods: We performed a retrospective review and identified 16 cases of EMPD with axillary involvement. We summarized the clinical and histopathological characteristics, treatment, and prognosis, as well as reviewed the literature.

Results: Of the included patients, eight were male and eight were female with an average age of 63.9 years at diagnosis. Eleven patients presented with unilateral axillary lesions, two patients with bilateral axillary lesions, and three patients with both axillary and genital involvement. Four male patients had a history of secondary malignancies. Axillary EMPD exerted the typical histological and immunohistochemical features of Paget's disease. All except for one patient underwent Mohs micrographic surgery with a mean final margin of 1.3 cm, and the tumor was cleared 76.5% of the time with 1 cm margins. None of the patients developed recurrence or metastasis after surgery at a mean follow-up of 63.6 months.

Conclusions: Axillary EMPD shares similar clinicopathological features with typical EMPD. Careful clinical and pathological examinations are mandatory to detect possible associated malignancies and to make a correct diagnosis. Axillary EMPD usually has a good prognosis. Due to the complete margin assessment and better recurrence rates for EMPD in general, Mohs micrographic surgery is the treatment of choice.