Introduction/Background Primary vaginal adenocarcinoma accounts for 1–2% of all gynaecological malignancies. Adenocarcinoma of the intestinal type is an extremely rare variant of vaginal adenocarcinoma that can arise from intestinal metaplasia in foci of adenosis, heterotopic intestinal tissue, cloacal remnants, foci of endometriosis and mesonephric remnants. The aim of this systematic review is to provide data that may be useful for further studies or future clinical practice guidelines.

Methodology A systematic review was performed in agreement with PRISMA statement. No restrictions on the publication period were applied. English articles were considered eligible.

Results The literature search retrieved 15 articles reporting a total of 16 cases. Data were gathered from articles published from 1986 to 2022. The median age was 49 (range 32–70). Most patients (87.5%) presented symptoms such as vaginal discharge and vaginal bleeding. The most common localization sites are the posterior lower third of the vagina (62.5%) followed by the anterior lower third (25%) and the middle and upper third (12.5%). The mean size of the lesion diameter is 3.04 cm (range 1–7 cm). FIGO stage I disease was found in 50% of patients, stage II and stage IVB in 6.25% of cases, respectively. As for treatment, 62.5% underwent surgery, 18.75% received concomitant chemoradiation and 6.25% radiation. Immunohistochemistry revealed positivity to CK20, CEA, CDX2, CK7, CA 15–3 and EMA. 43.75% of patients received adjuvant treatment, of which 25% receiving radiotherapy, 6.25% brachytherapy and 6.25% concomitant chemoradiation therapy. Only one patient was in-utero exposed to DES. Mean follow-up is 15.3 months (range 1.5–32) where 43.75% live with no evidence of disease, 18.75% are alive with evidence of disease, 6.25% died from disease. In one case there was ureteral relapse.

Conclusion The intestinal type of vaginal adenocarcinoma is exceptionally rare. The lack of guidelines results in varied clinical and treatment management.

Disclosures No disclosures