Ectopic lobular breast cancer of the vulva.

ABSTRACT

BACKGROUND: Primary breast cancer of the vulvar region is a rare tumor entity. Only 12 cases have been published so far in the literature. PATIENT AND METHODS: We report on a case of a 60-year old woman with an invasive lobular breast cancer localized to the vulva. The woman with elevated CA 15-3 and CEA markers underwent local wide excision of the tumor and bilateral inguinal lymphonodectomy, adjuvant anthracycline containing chemotherapy and locoregional irradiation including both inguinal regions and subsequent endocrine therapy with tamoxifen. RESULTS: The resected specimen showed an estrogen receptor-positive diffuse infiltrating lobular breast cancer of the vulva with metastases in both groins. Original tissue of the breast adjacent to the malignant vulvar transformation was lacking. Imaging of the breast was lacking any pathology. Our patient has been followed up for 20 months without any signs of progressive disease. CONCLUSION: Due to the rare occurrence, we are not able to give general guidelines for the treatment of ectopic breast cancer. However, adjustment of treatment to the therapeutic recommendations for orthotopic breast cancer seems reasonable and effective.