Discussion
Endometrioid carcinomas are the second most common epithelial ovarian
cancer. in contrast to the more common serous carcinomas, endometrioid
carcinomas present earlier, at a younger age and have better long-term
outcomes [5-7]. Several studies have suggested that endometrioid
carcinomas have higher rates of both 5-year overall survival (80.6%)
and progression-free survival (68%) compared to other ovarian cancer
subtype. Additionally, they have a lower recurrence rate, especially at
lower grades [1, 6]. Relapse patterns appear to further
differentiate endometrioid carcinomas from serous carcinomas, with
endometrioid carcinomas having a much higher proportion, at nearly 50%,
recurring solely in the pelvis, while serous carcinoma relapse tends to
be much more diffuse [6]. Here we discuss an interesting, rare
presentation of a pelvic recurrence of low grade endometrioid carcinoma.
Endometriosis is a risk factor for the development of ovarian cancer,
mostly specific subtypes including endometrioid and clear cell
carcinomas. Up to 42% of endometrioid adenocarcinomas are associated
with endometriosis. Patients with endometrioid ovarian adenocarcinoma
arising from endometriosis tend to present at a younger age, lower
stage, and lower grade than those without associated endometriosis (8).
The transformation from benign to atypical endometriosis to
endometriosis-associated ovarian cancer involves a combination of:
oxidative stress, inflammation, molecular genomic alterations,
hyperestrogenism (32). Some of the molecular abnormalities encountered
in endometriosis-associated ovarian cancer include: the activation of
oncogenic KRAS and PI3 K pathways and the inactivation of tumour
suppressor genes PTEN and ARID1A (3).ome of the key mutations involved
in the malignant transformation and progression, including
AT-Rich Interaction Domain 1A
(ARID1A) mutations, may have potential to be effective chemotherapy
targets [8, 9].
In a patient with endogenic hyperoestrogenism related to obesity, a new
pelvic mass diagnosed three years from the initial surgery may represent
as, rather than a recurrence, a de novo lesion progressing from benign
endometriosis to atypical endometriosis and then endometrioid ovarian
adenocarcinoma.
Other important pathologies have also been noted to be associated with
endometrioid carcinoma that should be understood and considered,
including endometrial cancer. The rare presentation of primary tumours
in both the endometrium and ovary in synchronous endometrial and ovarian
carcinoma, is recognised to be a separate entity to either pathology
with different prognoses and treatment implications [10-12]. These
tumours were previously thought to be synchronous independent tumours,
however molecular analysis has established that they have a common
clonal origin [13]. The 2023 FIGO staging of endometrial cancer,
which incorporates molecular findings, classifies these tumours as stage
IA3 when certain criteria are met: unilateral disease, no capsular
spread, less than 50% myometrial invasion, absence of
substantial/extensive
lympho-vascular space invasion
(LVSI). These tumours have a better prognosis and do not require
adjuvant chemotherapy [14].
The Carboplatin/Paclitaxel regimen as adjuvant treatment has not been
proven to result in survival benefit for low-grade endometrioid ovarian
cancer [15]. Current National Comprehensive Cancer Network (NCCN)
guidelines [16] for grade 1 endometroid ovarian carcinoma are
Carboplatin/Paclitaxel or hormonal treatment, such as aromatase
inhibitors, leuprolide acetate, tamoxifen. Novel, biomarker-driven
therapies, are currently being investigated for this histological
subtype: Bouquet (NCT04931342 GOG-3051) is a multicentre clinical trial
which is currently recruiting patients with persistent or recurrent
low-grade endometrioid ovarian cancer and other rare ovarian tumours
that are not amenable to curative surgery.
Until new treatments options are identified, surgery with maximal
cytoreductive effort remains the mainstay treatment for this
histological subtype of ovarian cancer [17-25]. Achieving
resection of all macroscopic
disease (R0) is the single independent factor for survival. In their
meta-analysis including 6885 patients with advanced ovarian cancer,
Bristow et al (2023) have shown there is a 5.5% increase in median
survival time with each 10% increase in maximal cytoreduction [2].
In the case we presented, a radical total exenteration was required to
achieve RO. A right nephrectomy was performed to remove the right
kidney, which was non-functioning. The Bricker technique for urinary
diversion involved spatulation of the remaining ureter and anastomosis
to a segment of descending colon used as a conduit. Total cystectomy
with urinary diversion is a standard procedure in the context of
anterior or total exenterations. The use of a segment of descending
colon should be considered in patients undergoing end colostomy, to
avoid the need for primary small bowel anastomosis.
In conclusion, ovarian
endometrioid carcinoma of the ovary is an uncommon histological subtype
of ovarian carcinoma for which the mainstay of treatment is surgery.
Cytoreductive effort should be maximized to achieve R0, especially in
young patients. The complexity of the operation should not be a
deterrent factor if surgery is carried out in a multidisciplinary
environment with robust clinical governance.