Primary large cell neuroendocrine carcinoma of the vagina: cytomorphology of previously unreported case.

Squamous carcinoma is the most common malignancy of the vagina. Other malignancies include adenocarcinoma, melanoma, lymphoma, and very rarely, neuroendocrine carcinoma/small-cell carcinoma. Large cell neuroendocrine carcinoma (LCNEC) has not been reported in this location. In this report, we describe a case of LCNEC of the vagina, which is believed to be the first case to date in the English literature. The patient is a 53-year old gravida 3, para 2, African-American woman who had a 4 month history of severe pelvic pain and difficulty voiding and was found to have a firm plate-like mass on the anterior vaginal wall. Thin prep of vaginal swap was interpreted as atypical glandular cells; however, the biopsies showed a large cell neuroendocrine carcinoma which was confirmed by diffuse strong immunoreactivity to AE1/3, CAM5.2, CK7, and CD56 in the tumor cells. Subsequent clinical workup showed that the patient also had numerous metastatic nodules in the bilateral lungs and a vaginal-urethral fistula caused by the tumor. The patient underwent palliative radiation of pelvis for local pain control and then chemotherapy. Although the vaginal tumor increased in size even after radiation, her symptoms were under control and she was doing well for a short period of time. The patient is still alive but developed brain metastasis a year later after initial diagnosis. Despite its rarity, large cell neuroendocrine cell carcinoma should be included in the differential diagnosis when cytomorphology shows features suggestive of neuroendocrine differentiation.