Intestinal-type
adenocarcinoma is a rare primary vaginal
carcinoma. Vaginal
adenocarcinomas are most frequently a metastatic lesion, and less commonly, have clear cell histology and occur in young women with
diethylstilbestrol (DES) exposure in utero. Due to the limited diagnostic power of immunohistochemistry (IHC) in differentiating primary from metastatic
adenocarcinoma of the vagina, clinical and radiological correlation is critical in this scenario. The prognosis of this
tumor depends on the patient's age,
tumor stage,
tumor differentiation, lymph node status, and distant
metastasis. Several treatment modalities are present depending on the
tumor stage. We present a case of primary
adenocarcinoma of the vagina and describe the histopathologic features including the immunoprofile of the
tumor and discuss the clinicopathologic features, differential diagnosis, diagnostic challenges, and a brief overview of the literature about age, size, site, immunohistochemical staining, and DES exposure.