Objectives Primary adenocarcinoma of vagina (PAV) accounts less than 15% of Primary carcinoma of the vagina (PCV). The objective of this study was to report the treatment management of PAV and to identify the prognosis factors affecting the overall survival (OS) and the disease free survival (DFS).

Methods A retrospective analysis of Salah Azaiz institute data base was performed analyzing women with PAV treated and diagnosed between 1994 and 2015.

Results Eleven patients were diagnosed with PAV, the mean age was 56.6 year, the mean tumor size was 4.6 cm, the histological types were clear cell carcinoma in 4 cases, mucinous in 3 cases, intestinal in one case, endocervical in one case and in two cases immunohistochimical typing wasn’t performed. The patients were staged: 3 stage I, 1 stage II, 5 stage III and 2 stage IV of FIGO. Treatment consisted on radiotherapy ± chemotherapy followed by surgery in 3 cases and a primary surgery in two cases. The mean follow up period was 51.9 months. Six patients achieved a complete response and 4 of them experienced relapse, 3 patients didn’t show any treatment response and 2 died of progressive disease. The 5 year overall survival (OS) and the disease free survival (DFS) were respectively 45.5% and 66.6%. Prognosis factors affecting OS were radiotherapy dose, the occurrence of recurrence. Prognosis factors affecting DFS were the tumor size the chemotherapy treatment.

Conclusions PAV is rare, little is known about its etiology and behavior. The treatment management still to establish to define the best guidelines.