Bone Metastases as Presenting Sign of Mammary-like Vulvar Adenocarcinoma

Mammary-like vulvar adenocarcinoma represents an extremely rare disease. Breast tissue develops from the embryonic ectoderm on the so called “milk-line”. During embryogenesis, this tissue undergoes spontaneous regression except in the chest region, where it gives rise to breasts in adults. When regression of “milk line” remnants fails to occur in the vulva, accessory breast structures may arise and could be susceptible to the physiologic, dysplastic and malignant changes seen in normal breast parenchyma. We report a 52-years old woman who had pelvic bone metastases (left ischiopubic ramus and the homolateral femur trochanter and acetabulum) as presenting sign of small primary vulvar apocrine adenocarcinoma. She received surgery, chemotherapy and radiotherapy but died of disseminated disease 18 months after diagnosis