A rare case of Ductal carcinoma in situ in ectopic breast tissue in the vulva: A case report

Ectopic breast tissue can be found along the rudimentary mammary line, which extends from the axilla to the inguinal region and disappears during the process of embryogenesis. Like normal breast tissue, this ectopic tissue is prone for malignant transformation. Most of the reported cases were invasive ductal or lobular carcinomas however, only 2 cases of ductal carcinoma in situ (DCIS) in ectopic breast tissue have been reported so far. Diagnosis and management of this rare pathological finding can be challenging. Here we report an interesting case of a 53-year lady with DCIS arising from ectopic breast tissue in the left vulva. The patient was diagnosed after surgical excision of a long standing painless left vulvar lesion. Only postoperative hormonal treatment in the form of tamoxifen was given without any evidence of disease recurrence after 3 years of follow up. We discuss the challenges faced during diagnosis and outline management options for this disease entity.