Abstract

A 45-year-old female presented to the emergency room due to concern for a vulvar lesion. Physical examination revealed a two by one centimeter erythematous lesion on the right labia majora. Biopsies were performed, and the patient underwent a radical partial vulvectomy by gynecologic oncology. On excision, histopathology revealed large, pleomorphic epithelial cells with intracytoplasmic mucin within the basilar and suprabasal epidermis. An infiltrative tumor composed of nests and sheets of atypical epithelial cells with vesicular nuclei, prominent nucleoli, and a moderate amount of light eosinophilic cytoplasm was present in the papillary and reticular dermis. Areas of glandular formation were noted. Lymphovascular invasion was present, and tumor was seen in three out of seven examined lymph nodes. Tumor cells were positive for CEA, CK7 and GATA-3. CK20, PAX8, and CDX2 stains were negative. A diagnosis of invasive adenocarcinoma with associated Paget disease was rendered. Radiologic imaging did not reveal any other sites of carcinoma and this was favored to be primary extramammary Paget disease (EMPD) with associated invasive adenocarcinoma. Following her surgery, she was treated with adjuvant chemoradiation. EMPD is a rare condition that usually involves areas dense in apocrine glands such as the axillae or anogenital skin. EMPD may occur as a primary neoplasm, as a direct extension of an underlying carcinoma, or in association with metastatic adenocarcinoma. This case is unusual as invasive adenocarcinoma arising in association with EMPD of the vulva is rare and usually occurs in elderly females. While lymphovascular invasion and inguinal lymph node metastases typically portend a poor prognosis in these patients, our patient remains disease free nearly three years after diagnosis.

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