Primary ectopic breast cancer of the vulva, treated with local excision of the vulva and sentinel lymph node biopsy: a case report

Abstract

Primary breast cancer fairly infrequently occurs in ectopic breast tissue, and primary ectopic breast cancer of the vulva is particularly rare. Only 26 cases have been published in the English-language literature, and there has been no report of primary breast carcinoma of the vulva in Japan. We report a rare case of primary ectopic breast cancer of the vulva that was treated with local excision of the vulva and sentinel lymph node biopsy (SLNB). The patient was a 72-year-old woman who had noticed a right vulvar tumor 10 years earlier. The tumor was excised by the Department of Plastic Surgery of our hospital. The histology of the vulvar tumor revealed an invasive ductal carcinoma of the breast, and immunohistochemical staining of the vulvar specimen showed the tumor cells to be 100% estrogen-receptor-positive and 100% progesterone-receptor-positive. All margins of resection were positive for neoplastic involvement. An additional local excision of the vulva and right inguinal SLNB were performed in our department. The intraoperative frozen section was negative for metastasis, and lymph node dissection was not performed. The final pathology was negative for residual disease, and a partially normal ductal component was present. Adjuvant hormonal therapy with an aromatase inhibitor was indicated post-operatively. The patient was asymptomatic and free of detectable disease at a 6-month follow-up. Due to the rarity of this diagnosis, there are no established guidelines for treatment. Although cases in which SLNB was performed are rare, we consider SLNB to be an effective alternative to inguinal node dissection for ectopic primary breast cancer of the vulva.

Primary breast cancer of the vulva: A case report and literature review

“…The most common clinical presentation was a painless, solitary nodule, arising most often in the labia majora . Among the histological subtypes of primary breast cancer that can arise in the vulva, including infiltrating ductal, lobular, mucinous, mixed and carcinoma in situ (DCIS), invasive ductal carcinoma is the predominantly reported histology . The following criteria have been proposed to differentiate between primary and metastatic vulval cancer: (i) a histological pattern consistent with breast carcinoma; (ii) the positivity of ER and/or PgR; (iii) positive immunohistochemical test for common breast cancer‐associated markers, and (iv) the presence of non‐malignant breast tissue or carcinoma in situ.…”

“…For patients with ER‐positive vulva cancer, adjuvant hormonal therapy has been used since it has been proven of survival benefit in ER‐positive breast cancer . The use of Trastuzumab (Herceptin) in HER2 positive cases has been reported only once in the literature . The average survival for patients who did not receive adjuvant treatment was 1–4 months, with the longest reported survival was 48 months for IDC treated with adjuvant radiation and hormonal therapy .…”

Periclitoral accessory breast tissue in a lactating woman

“…Wide local resection is the main treatment strategy for these patients who have accessory breast tissues, since the masses have no use for people [ 10 ] and they have opportunities to develop many kinds of breast diseases, such as adenocarcinoma, Paget disease, fibroadenoma, etc. [ 11 – 15 ]…”

Bilateral Vulvar Ectopic Breast Tissue in a 38-Year-Old Woman Who is Breastfeeding: Case Report and Review of the Literature

“…Ectopic breast tissue is used to describe all types of breast tissue found outside its normal location in the thoracic mammary glands [4]. This tissue is hormonally responsive and may develop benign and malignant pathologic processes similar to those seen in normally located breast tissue, such as fibroadenoma, intraductal papilloma, fibrocystic disease, lactating adenoma and carcinoma [1,4].…”

“…Ectopic mammary tissue may occur anywhere along the milk line, which extends bilaterally from the axilla to the groin. The frequency of ectopic breast tissue in females is 1 to 6% and is relatively common in the axilla or the chest wall but is unusual in the vulva [1].…”