A rare case of metachronous ectopic breast cancer of the vulva

Ectopic breast tissue can arise at any point along the primitive milk line, which runs from the axilla to the groin. The incidence of ectopic breast cancer ranges from 0.2% to 0.6%. We report the case of a 60-year-old woman with a history of left mastectomy for breast cancer one year prior to presentation, who presented with bilateral vulvar erosion found to be a moderately differentiated adenocarcinoma on biopsy. The extension workup being negative, a radical vulvectomy was performed. The postoperative course was uneventful, and the specimen's pathologic study revealed a vulvar localization of non-specific invasive breast carcinoma. It was positive for estrogen receptor and human epidermal growth factor receptor 2/neu, and negative for progesterone receptor. Adjuvant chemoradiotherapy was given to the patient. There is no dedicated guideline for both diagnosis and management of this particular tumor; the treatment is similar to that of orthotopic breast cancer.(c) 2022 SESPM. Published by Elsevier Espana, S.L.U. All rights reserved.