We report the case of a young woman with an advanced stage primary peritoneal serous borderline tumor (PPsBT) who strongly desired to maintain her fertility and refused a radical surgery. After obtaining a full informed consent, we proceeded to a complete cytoreduction with preservation of the uterus and the adnexa. The borderline implants were diffusely observed in the peritoneal surfaces of the pelvis (Figure 1), but without any evidence of borderline cyst on the ovaries. All peritoneal implants were removed to obtain a complete cytoreduction (R0). All implants had histological characteristics of borderline tumor (Figure 2) and no invasive implants were documented. We therefore considered the diagnosis of a PPsBT and allocated a stage pTIIIAN0 or FIGO IIIA2. PPsBT are considered to have similar natural history, prognosis and oncologic outcomes than sBOT and are therefore managed accordingly (7).
Approximately one-third of sBOT affect young women during their reproductive age and the majority of sBOT are limited to the adnexa (FIGO stage I) (3,8). In this specific context, an uni/bilateral cystectomy or unilateral ovariectomy are adapted procedures to obtain a complete resection of the disease, allowing fertility preservation. If the overall prognosis of early stage sBOT is excellent, data showed that fertility preserving approach is associated with an increased risk of recurrence without affecting survival, since the recurrences mostly occur on the remaining ovaries and/or the peritoneum (3,9,10). Relapses can often be safely resected by conservative surgery (11).
In contrast to early stage BOT, the safety of fertility sparing surgery of advanced-stage sBOT is much less documented (11). When peritoneal implants are documented either in the pelvis (stage II) and/or in the abdomen (stage III), data concerning the efficacy and safety of fertility sparing management are limited to small retrospective series (all series reported in Table 1, 9, 10, 12, 13, 14, 15, 16, 17, 18, 19, 20).
The main publications on the topic are listed in the Table 1. The majority of the series reported on 20 patients or less, with only 2 series reporting on more than 50 patients. All studies have a retrospective design. The recurrence rates as borderline tumor range from 20 to 60% but the relapse rate as invasive disease range from 0 to 33.3% (Table 1).
The recent multi-institutional retrospective italian study is the largest series that reported on 91 patients with advanced-stage sBOT treated by fertility sparing surgery (19). The authors documented a recurrence rate of 53,8% but the disease-specific survival (DSS) does not seem impacted (19). The authors consider that the risk of recurrence is not related to the ovarian preservation per se, but to the natural history of the initial peritoneal spread (19).
The large uni-institutional series published by French group reported on 212 patients with advanced stage sBOT treated between 1971 and 2017 (20). Among these patients, 65 underwent conservative treatment, including 8 patients with invasive implants (20). Among patients treated conservatively,
58% experienced a recurrence (20). Again the authors documented that fertility sparing management is associated with a decreased disease free survival (DFS), but without affecting the overall survival (OS). It seems therefore essential to inform patients that the recurrence rate is high
and that some recurrences could not be salvaged leading to three deaths (20).
One meta-analysis was conduced by Huang et al. to assess the feasibility of fertility-sparing surgery in treating advanced-stage sBOT, pooling the results of four small retrospective series (21). The meta-analysis concluded that conservative surgery could be proposed to young patients who want to preserve their fertility. However, the validity of data is limited by the following characteristics: the small sizes of the cohorts, the retrospective design of the studies, the observational and nonrandomized natures of the trials (21).
The initial FIGO stage, the presence of invasive implants and the completeness of the surgery are considered as the most important prognostic factors for recurrence (22). The multivariate analysis from Wang et al. revealed that FIGO stage III is an independent risk factor for recurrence (6). Multivariate analysis focusing on patients under 40 years old identified advancedstage and fertility-sparing surgery as independent prognostic factors negatively affecting DFS (5, 8).
The up-dated series by Gouy et al. led to a change in their initial conclusions (20). The authors confirm that the risk of recurrence is increased after conservative treatment compared to radical surgery and that OS rates are similar in both surgical approaches. However, if they initially suggested that patients with invasive implants should not be managed conservatively, their conclusion has been amended since their recent data on oncological outcomes of patients with invasive implants suggest that the prognosis is probably related to the natural history of the peritoneal disease and not to the use of a fertility-sparing strategy itself. Therefore, the type of implants does not seem to be a selection factor to consider a conservative surgical approach in stage II or III disease, but this proposal should be treated with caution (20). The recent study by Falcone et al. confirmed that fertility-sparing treatment should be considered even in context of invasive implants (23). According to Wang et al., patients treated with ovarian cystectomy may be follow closely if post-operative imaging are negative (24).
After fertility-sparing surgery, the patients are advised to proceed with their fertility program as soon as possible (25). The pregnancy rate after fertility sparing treatment in advancedstage sBOT is much less known than its oncologic safety (19). Only a few studies have reported the fertility outcome of fertility-sparing management in women with advanced sBOT (21). Spontaneous fertility is favored since the impact of in vitro fecondation (IVF) in the natural history of borderline tumor remain unclear (10). Uzan et al. reported on fertility results about their series of 80 patients treated conservatively for Stage II or III BOT restricted to patients with non-invasive peritoneal implants. Their results confirm that spontaneous pregnancy occur after a conservative treatment of advanced disease (9). Song et al. reported their experience about 25 women treated for advancedstage BOT. Five underwent fertility-sparing surgery, four attempted to conceive and five pregnancies occurred (17). In a series of 59 patients treated for advanced-stage BOT, Helpman L et
Reported fertility sparing procedure on 33 patients, 34 pregnancies occurred on 21 patients who attempt conception, but the FIGO stage is not specified (26). A total 26 live births were documented among 21 patients who attempt to conceive (26). Encouraging fertility data were also reported by the two most recently published series by Falcone et Gouy (19, 20). In the series of 91 patients, reported by Zanetta et al., among the 29 patients (31.8%) who attempted to conceive, 20 patients achieved at least one pregnancy and 18 gave birth to a healthy child (12). In the French series, 24 pregnancies were observed in 20 women among the 29 patients who wanted to become pregnant. 13 pregnancies were spontaneous (20).
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