Clinical symptoms and pathological characteristics
A total of 57 patients with PFTC were enrolled in this retrospective study. As shown in Table 1, the mean age of PFTC at diagnosis was 57.35±9.01 years (range, 39–80 years). 42 patients (73.7%) were postmenopausal, only one (1.8%) patient was nulliparous, and the mean time of post-menopause was 8.18±8.12 years (range, 1–33 years).
The main clinical symptoms were palpable pelvic and/or abdominal mass (68.4%), abdominal pain (33.3%), abdominal distension (26.3%), abnormal vaginal bleeding (19.3%) and followed by discharge (10.5%). Only 4 patients (7%) experienced typical Latzko’s triad symptoms including abdominal pain, vaginal bleeding or discharge, and a palpable pelvic mass. Furthermore, 6 cases (10.5%) were exhibited atypical clinical manifestation containing irregular menstruation (2 cases, 3.5%), urinary frequency and urgency (1 case, 1.75%), pantalgia (1 case, 1.75%), fever (1case, 1.75%), and severe edema of the lower limbs (1 case, 1.75%) ( Table 1).
50 patients (87.7%) had unilateral fallopian tube tumor, while seven (12.3%) patients had bilateral fallopian tubes tumors. Preoperatively, only 6 patients (10.5%) were diagnosed as PFTC, 46 (80.7%) were misdiagnosed with ovarian cancer, and 5 (8.8%) were misdiagnosed as endometrial cancer. 44 patients (77.2%) had no preoperatively pathological results, only eight cases (14.0%) were shown adenocarcinoma cells in the ascites, and five cases (8.8%) were in the endometrial tissues ( Table 1).
A primary tumor diameter greater than or equal to 5 cm were in 42 patients(73.7%), and majority of patients were at stage III (n = 25, 43.8%), 15 (26.3%) patients at stage I, 12 (21.1%) at stage II, and the rest at stage IV (n = 5, 8.8%). Consistently, serum carbohydrate antigen 125(CA125) was enhanced (≥35 U/mL) in 43 patients (75.4%) preoperatively in these 57 patients. The accuracy of intraoperative frozen pathological diagnosis was 70% (35/50), expelled 7 patients who without frozen results. The main common histologic subtype was serous adenocarcinoma (n = 54, 94.7%) with high-grade (n = 51, 89.5%) ( Table 2).
Treatment Regimen
Surgery was the initial therapy for all patients, 30 (52.6%) patients were underwent complete tumor resection with residual lesion no larger than 1 cm. In these 57 patients, bilateral salpingo-oophorectomy was performed in all patients, accompanying with total hysterectomy (96.5%), omentectomy (77.2%), pelvic lymphadenectomy (40.4%, with metastasis detected in two patients), para-aortic lymphadenectomy (10.5%, with metastasis detected in two patient), or appendectomy (29.8%, with metastasis detected in two patients), respectively.
In this cohort of 57 patients, 56 were received postoperative chemotherapy with intravenous paclitaxel plus platinum(TP) regimen. Among them, 30 (53.5%) were received ≥ six cycles of chemotherapy, and 36(64.3%) patients received ≥ two cycles of intraperitoneal chemotherapy, which contained cisplatinum 40 milligram(40mg) and recombinant human interleukin–2 500 international units (500 IU) for perfusion on the first and fifth days after surgery. Whereas the one case who had not received chemotherapy because of intolerance to the adverse effects.
Recurrence
Five patients were lost to follow-up. Among the remaining 52 patients, 26 patients (50%) were relapsed at the median of 18.5 (3–83) months. The recurrent sites involved the pelvis (n = 7, 26.9%), upper abdomen (n = 2, 7.7%), retroperitoneal lymph nodes (n = 3, 11.5%), and distant metastasis (n = 7; 1 in the lung, 3 in the liver, 1 in the bone, and 2 in the brain). Seven (26.9%) patients had elevated CA125 with no obvious image evidence for metastasis. At the time of data collection, 12 patients (46.2%) were still alive, and 14 (53.8%) were died from recurrence.
Survival analysis
The 5-year overall survival (OS) rate was 15.4%, and the 5-year Disease-free survival (DFS) was 11.5%. Additionally, univariate analysis showed that the 5-year OS was related to tumor stage (III/IV vs I/II, P = 0.013) and residual tumor size (≥1 vs <1 cm, P = 0.001) ( Table 3). While DFS was significantly related to clinical tumor stage (III/IV vs I/II, P = 0.007), residual tumor size (≥1 vs <1 cm, P = 0.003), and pre-treatment CA125 level (<35 vs ≥35 U/mL, P = 0.047) ( Table 4). Variables (including tumor stage, size of residual tumor, pelvic lymphadenectomy, and pre-treatment CA125 level) which shown significant difference in univariate analysis were entered into the multivariate analysis. The Cox proportional hazards model and curves of OS or DFS were demonstrated that residual tumor size was the only independent prognostic factor both related to 5-year OS and DFS ( Table 5 & Fig. 1).
All other factors(such as age, postmenopausal period, histologic grade, primary tumor diameter, cycles for chemotherapy) did not have any significant influence on PFTC prognosis.