Adenocarcinoma of intestinal type of the vulva

Primary adenocarcinoma of the vulva is a rare disease, and usually arises in a Bartholin gland or occurs in association with Paget’s disease. Furthermore, adenocarcinoma of intestinal type of the vulva is an extremely rare neoplasm, and few cases have been reported. The appropriate treatment for optimal prognosis is unclear. We report a case of adenocarcinoma of intestinal type of the vulva occurring in a 63-year-old female. The tumor was found near the urethra, and biopsy specimen showed a proliferation of signet ring cells embedded in an abundant myxoid stroma and irregular tubular structures of atypical columnar epithelium. An extensive workup showed no metastases. Local excision with a 2-cm lesion in the vulva side, bilateral superficial inguinal lymph node dissection and Cloquet lymph node biopsy were performed. Cancer cells contained mucinous materials in the cytoplasm, which exhibited diffuse positive staining for cytokeratin 20 and CDX2. The final pathologic diagnosis was adenocarcinoma of intestinal type of the vulva (pT1bN0M0). The patient received adjuvant external irradiation because of positive urethral surgical margin. She is well 1 year after therapy. Immunohistochemical staining for cytokeratin 20 and polyclonal CDX2 is helpful with investigation of adenocarcinoma of intestinal type, but long-term prognosis remains unclear.