Axillary Paget disease with a visible satellite: a case report and literature review

Abstract

Background: Extramammary Paget disease (EMPD) is an uncommon malignancy affecting apocrine gland–bearing skin, such as vulvar, perianal, axillary and penoscrotal areas. Paget cells are sometimes detected outside clinical border in a phenomenon called subclinical extension. Satellite is one of the patterns of subclinical extension which is likely to be invisible. The standard management strategy for EMPD without distant metastasis is a complete surgical removal, sometimes called wide local excision. However, there is no consensus regarding surgical margin width to decrease the high recurrence rate. Here we describe the first macroscopically visible satellite of axillary EMPD and wide local excision of both main lesion and the satellite lesion with only 0.5 cm margin, succeeded by a short review of the literature. Case presentation: A 48-year-old female with a red macule in the right axilla was presented to our clinic. A well-demarcated 4 cm × 3 cm erythematous plaque was observed in the right axilla, and a similar lesion measuring 0.5 cm × 0.3 cm was found 3.5 cm away from the primary site. Breast and axillary node examination was unremarkable. Biopsy of the large plaque revealed Paget disease, then we performed a local extended excision of both lesions with a 0.5 cm margin, all margins negative indicated, by frozen pathology. Pathology revealed the nature of the satellite beside the main lesion also as Paget disease. The patient is currently followed-up for 20 months and has shown no signs of recurrence, with normal shoulder motor function. Conclusion: We have report the first visible satellite of extramammary Paget disease, indicating the necessity of an extended local resection of both the main leision and the satellite lesion. Considering the anatomical structure of axillary Paget disease, a 0.5 cm negative surgical margin indicated by frozen pathology might be sufficient to sustain the shoulder motor function.