Background. Intestinal-type primary vaginal adenocarcinoma is an extremely rare neoplasm, and very few cases are reported in the literature. The differential diagnosis of an intestinal-type adenocarcinoma at vaginal level (an organ almost free from glandular tissue) is particularly challenging given the anatomical complexity and different embryologic derivations of organs in this district. The main diagnostic issue consists in determining whether the vaginal neoplasm is primary, or it is a metastatic disease that extends to the vagina. A fundamental role in guiding diagnostic and therapeutic pathways is played by the pathologist. Objective. This study is a systematic review of the literature in order to summarize and analyze different diagnostic and therapeutic approaches chosen for every single case of intestinal-type primary vaginal adenocarcinoma described. Moreover, we report the case diagnosed and managed at our center. Methods. PubMed, ClinicalTrials.gov, Scopus, and Web of Science databases were systematically searched for records from January 1st, 1989, to December 1st, 2019. Results. Overall, 23 cases of intestinal-type primary vaginal adenocarcinoma are reported in the literature. This tumor often presents with atypical vaginal discharge (64.7% of cases) and it affects mainly the posterior wall (54.5%) and the lower third (83.3%) of the vagina. The average age at its presentation is 53.6 years. Diagnostic workup looks at ruling out possible primary distant sources of the disease and colonoscopy is often performed. The immunohistochemical profile of the lesion has a major role, and the key markers investigated are CEA, CK20, CK7, and CDX2. Most patients are diagnosed with early-stage disease (85% of patients FIGO I) and the lesion average size is 3 cm. Of 18 patients with available data, a surgical approach was adopted in 8 cases. Ten patients underwent radiotherapy. Patients managed surgically, compared with those who underwent radiotherapy, were younger and with a smaller mass at diagnosis, although differences were not statistically significant. Treatment options depended on clinical evaluation, patient’s comorbidities, and patient’s preferences. Conclusions. Intestinal-type primary vaginal adenocarcinoma is a rare tumor, and no specific guidelines addressing this disease are available. Ruling out a metastatic disease at the vaginal level is fundamental, and the pathologist plays a major role in the differential diagnosis. A multidisciplinary approach to the disease is of fundamental importance and the treatment choice should be tailored considering the patient’s comorbidities and the holistic evaluation of the case.
File in questo prodotto:
Non ci sono file associati a questo prodotto.