Primary fallopian tube carcinoma (PFTC) is a rare gynecological malignancy that accounts for 0.14 to 1.8% of all gynecological cancers [
1]. It was first described in 1847 by Renaud and a little over 2000 cases have been reported to date. Its prevalence is believed to be higher than reported [
2] because of its histological pattern (similar to epithelial ovarian cancer) and late stage diagnosis presenting with extensive pelvic dissemination (the ovary being considered the primary tumor). PFTC usually presents in the sixth decade of life [
1] and the predisposing factors are not well determined, although nulliparity, infertility and inflammatory pelvic disease may have an influence [
3]. The presence of
BRCA genetic mutation confers an augmented risk of developing PFTC. Some suggest that in all PFTC cases encountered in their series 16% have
BRCA mutations [
4]. Symptoms include profuse vaginal discharge, abnormal vaginal bleeding, pelvic or abdominal pain (usually colicky pain due to tube distension) and a pelvic and/or abdominal mass can be palpable [
5]. Latzke’s triad, which comprises watery vaginal discharge, vaginal bleeding with pelvic and/or abdominal pain, presents in only 15% of cases [
6]. Preoperative diagnosis is difficult and imaging can be misleading because it may not identify the primary tumor. Pelvic ultrasound can be used to assess “sausage-like” images, related to tubal distension, as well as vascular abnormalities using Doppler ultrasound [
7]. Depending on the series, there is a 3 to 4% rate of preoperative diagnosis [
8]. Diagnosis is usually histological and has to meet specific criteria [
9]. Tumor dissemination in cases of PFTC is preferentially transperitoneal and lymphatic [
1] which can explain the higher incidence (in comparison to the ovary) of distant and retroperitoneal metastases [
10]. Staging, surgical treatment and adjuvant medical treatment follow the same principles of epithelial ovarian cancers [
11,
12]. Prognosis is highly dependent of stage at diagnosis. A 5-year survival can range between 50 and 60% (stage II) and 10 and 20% (stages III and IV) [
1]. Other series report different survival rates, but use more aggressive chemotherapeutic protocols. There are some unfavorable prognostic factors besides stage: age above 50-years old [
13], tubal muscular layer (>50%) and/or serosal invasion [
1], less optimal cytoreduction [
12] and histological poor differentiation [
14]. Surprisingly, primary tumor size does not affect prognosis [
1].