Non-epithelial Ovarian Cancer Research Articles

Prediction of ovarian cancer prognosis using statistical radiomic features of ultrasound images

Objective. Ovarian cancer is the deadliest gynecologic malignancy worldwide. Ultrasound is the most useful non-invasive test for preoperative diagnosis of ovarian cancer. In this study, by leveraging multiple ultrasound images from the same patient to generate personalized, informative statistical radiomic features, we aimed to develop improved ultrasound image-based prognostic models for ovarian cancer. Approach. A total of 2057 ultrasound images from 514 ovarian cancer patients, including 355 patients with epithelial ovarian cancer, from two hospitals in China were collected for this study. The models were constructed using our recently developed Frequency Appearance in Multiple Univariate pre-Screening feature selection algorithm and Cox proportional hazards model. Main results. The models showed high predictive performance for overall survival (OS) and recurrence-free survival (RFS) in both epithelial and nonepithelial ovarian cancer, with concordance indices ranging from 0.773 to 0.794. Radiomic scores predicted 2 year OS and RFS risk groups with significant survival differences (log-rank test, P < 1.0 × 10−4 for both validation cohorts). OS and RFS hazard ratios between low- and high-risk groups were 15.994 and 30.692 (internal cohort) and 19.339 and 19.760 (external cohort), respectively. The improved performance of these newly developed prognostic models was mainly attributed to the use of multiple preoperative ultrasound images from the same patient to generate statistical radiomic features, rather than simply using the largest tumor region of interest among them. The models also revealed that the roundness of tumor lesion shape was positively correlated with prognosis for ovarian cancer. Significance. The newly developed prognostic models based on statistical radiomic features from ultrasound images were highly predictive of the risk of cancer-related death and possible recurrence not only for patients with epithelial ovarian cancer but also for those with nonepithelial ovarian cancer. They thereby provide reliable, non-invasive markers for individualized prognosis evaluation and clinical decision-making for patients with ovarian cancer.

Predictive value of the Adult Comorbidity Evaluation 27 on adverse surgical outcomes and survival in elderly with advanced epithelial ovarian cancer undergoing cytoreductive surgery.

We aimed to evaluate the ability of Adult Comorbidity Evaluation 27 (ACE-27) to predict perioperative outcomes and survival in elderly women with advanced epithelial ovarian cancer (AEOC) undergoing cytoreductive surgery. We collected patients with AEOC in our hospital between January 1, 2012 and January 1, 2021. Patients younger than 65years old or those with non-epithelial ovarian cancer were excluded. ACE-27 was applied retrospectively to assess comorbidities in the selected patients, who were then classified into two groups based on their ACE-27 scores: low ACE-27 score group (none to mild) and high ACE-27 score group (moderate to severe). A total of 222 elderly women with AEOC were included, of whom 164 patients accepted debulking surgery. Among those who have undergone surgery, Clavien-Dindo grade III + perioperative complications or unintended intensive care unit (ICU) admission occurred more often in patients of high ACE-27 score group, with statistically significant difference (odds ratio [OR]: 4.21, 95% confidence interval [CI], 1.28-14.35, p = 0.018). Further stratified analyses by age, BMI, FIGO stage and pathology also prove that OS of patients graded severe was shorter than patients graded none to moderate in cohort of age < 70, BMI < 25kg/m2, FIGO III stage and pathology of serous, respectively. Kaplan-Meier survival curves analyzed by log-rank test showed that the overall survival (OS) of patients with severe comorbidities were shorter than with none to moderate (HR 3.25, 95%CI 1.55-6.79, p = 0.002). Our findings demonstrate the ability of ACE-27 to predict grade III + perioperative complications or unintended ICU admission and survival in elderly patients with AEOC. This highlights the possibility for ACE-27 to play an instrumental role in identifying AEOC patients who are more susceptible to adverse surgical outcomes and have a poor survival rate and assisting in decisions regarding treatment.

Clinicopathological Features and Survival Trends of Non-Epithelial Ovarian Cancer: Analysis of the Surveillance, Epidemiology, and End Results (SEER) Database

Introduction: Owing to their low incidence, no reliable statistics about prognostication derived from large sample sizes have been reported of malignant ovarian germ cell tumors (MOGCTs) and sex cord-stromal tumors (SCSTs). The present study aimed to investigate the clinicopathological prognostic factors and the survival trends of MOGCTs and SCSTs. Materials and Methods: Patients with MOGCTs and SCSTs were recorded in the Surveillance, Epidemiology, and End Results (SEER) database diagnosed between 2000 and 2019. Clinical, demographic, and treatment characteristics were compared between groups of MOGCTs and SCSTs. Cox risk regression analysis and Kaplan-Meier survival curves were used to compare overall survival (OS) and cancer-specific survival (CSS) and to assess the prognostic factors. Results: Information about 2,506 patients with MOGCTs and 1,556 patients with SCSTs was extracted from the SEER database, respectively. Aged <40 years and single were more common in patients with MOGCTs than in those with SCSTs. The vast majority of patients with MOGCTs and SCSTs underwent surgery (98.1% vs. 94.5%; p < 0.001), and women with MOGCTs were more likely to receive chemotherapy than women with SCSTs (56.1% vs. 32.2%; p < 0.001). For both patients before and after propensity-score matching, the 5-year OS rates of patients with SCSTs were lower than those of patients with MOGCTs (p < 0.05). In multivariate Cox regression analysis, both age and surgery were independent predictors of OS in patients with MOGCTs and SCSTs. FIGO staging was an independent predictor of CSS in MOGCT patients. Tumor size and chemotherapy were also independent predictors of CSS in patients with SCSTs. Conclusion: Compared to patients with SCSTs, those with MOGCTs tended to be younger and had a higher OS and CSS. Adjuvant chemotherapy after surgery did not prolong OS and CSS in patients with SCSTs.

Obstetric Results after Fertility-Sparing Management of Non-Epithelial Ovarian Cancer.

To assess the recurrence and birth rates among patients with non-epithelial ovarian cancer. The study included 146 patients with germ cell (GCT, n = 84) and sex cord-stromal tumors (SCST, n = 62), who underwent fertility-sparing surgery. Adjuvant chemotherapy was administered to 86 (58.9%) patients. Most cases (133 out of 146) were staged FIGO I. The 5- and 10-year disease-free survival rates were 91% and 83%, respectively. The recurrence risk was not associated with tumor histology, stage or age. Twenty-four months after the treatment, the rate of recurrence was higher than the rate of childbearing. The childbearing rates kept rising after the treatment and exceeded the rate of recurrence after 2 years. The cumulative incidence rates of birth 36, 60 and 120 months after treatment were 13.24%, 20.75%, and 42.37%, respectively. Chemotherapy was not related to childbearing. The patients' age was related to the chance of childbearing. The prognoses of GCT and SCST are similar. Close follow-ups along with contraception should be offered to women during the first two years after treatment due to the increased risk of recurrence. After this period, relapses are rare and women can safely become pregnant.

Clinical Challenges in the Management of Malignant Ovarian Germ Cell Tumours.

Nonepithelial ovarian cancers (NEOC) are a group of rare malignancies, including germ cell tumours (GCT) and sex cord-stromal tumours (SCST), along with small-cell carcinomas and sarcomas. GCTs represent 2-5% of ovarian cancers, with a yearly incidence of 4:100,000, and they usually affect young women and adolescents. Precursory germ cells of the ovary form the basis of GCT. They are histologically classified into primitive GCT, teratomas, and monodermal and somatic-type tumours associated with dermoid cysts. A primitive GCT can be either a yolk sac tumour (YST), dysgerminoma, or mixed germ cell neoplasm. Teratomas are either mature (benign) or immature (malignant). Given that malignant GCTs occur rarely compared to epithelial ovarian tumours (EOC), greater focus is required in their diagnosis and treatment. In this article, we review the epidemiology, clinical manifestations, diagnosis, and molecular biology, along with the management and therapeutic challenges.

Reproductive and oncologic outcomes in women with non-epithelial ovarian cancer: Single center experience over 25 years.

This study aimed to present our single-center clinical experience regarding tumor clinicopathologic features, treatment modalities, and reproductive and oncologic outcomes in patients with non-epithelial ovarian cancer (NEOC) over 25 years. A total of 100 patients with clinicopathological diagnosis of NEOC who were treated at our tertiary care center between 1996 and 2022 were included in this retrospective cohort analysis study. Data on demographic, clinical and obstetric characteristics of patients at the time of initial diagnosis as well as tumor clinicopathologic features, treatment modalities, and oncological and reproductive outcomes were recorded. NEOCs involved germ cell tumors (GCTs) in 46 (46%) patients and sex cordstromal tumors (SCSTs) in 54 (54%) patients. Thirty patients with GCTs and thirty-four patients with SCSTs possessed histological subtypes with malignant features. Most patients with GCTs (37%) and SCSTs (55.6%) had FIGO Stage 1 disease at the time of initial diagnosis. Overall, 76.6% of patients in the GCT group (n=23) underwent fertility-sparing surgery (FSS), while 76.5% of the patients in the SCST group (n=26) were treated with non-fertility-sparing surgical procedures. All patients who underwent FSS and had a recurrence in their follow-up (n=4) was stage 3 patients. Seven out of 10 patients (2 patients at stage 3 and 5 patients at stage 1) who desired pregnancy delivered between 38 and 40 gestational weeks without any congenital anomaly. The prognosis was excellent in both groups, with 5-year overall survival (OS) rates of 93.5% in GCTs and 96.3% in SCST groups. The 5-year disease-free survival was 89.1% in GCTs and 94.4% in SCSTs. FSS was not associated with worse oncologic outcomes. NEOCs usually have a good prognosis because they are detected at an early stage. FSS may be indicated for women of reproductive age with early-stage NEOCs.

Clinical utility of reporting research genetic predisposition testing results to Total Cancer Care participants.

10602 Background: The American College of Medical Genomics and Genetics (ACMG) has identified genes with highly penetrant phenotypes with treatment, prevention, or early disease identification strategies called secondary findings (SF) genes. ACMG recommends clinical laboratories report pathogenic/likely pathogenic (P/LP) SF variants identified during genomic testing. Cancer patients may undergo germline sequencing as part of research studies that can identify valuable SF variants. The objective of this institutional sub-study is to evaluate the feasibility of using ACMG SF genes to assess hereditary disease risk in a cancer population undergoing research sequencing. Methods: Between June 2017 and January 2021, patients unselected for family history, disease site, or stage enrolled in the prospective, observational Total Cancer Care study. Patients underwent research-grade germline whole exome sequencing, with bioinformatic analysis in a CLIA certified laboratory to identify P/LP variants in any ACMG SF v2.0 genes. P/LP variants were reported to treating oncologists with recommendations for genetic counseling referral. We assessed if patients with P/LP variants met NCCN guideline-directed germline testing criteria to quantify benefit from reporting research results. Results: We enrolled 786 patients, which included those with gynecologic (182, 23%), colorectal (124, 16%), lung/bronchus (83, 11%), and head/neck (82, 10%) cancers. The median age was 61 (IQR 52-68) years, 57% were female, 96% were non-Hispanic White, 51% had stage III/IV disease, and 64% resided in an Appalachian community. We identified 57 patients (7%) with cancer predisposition and 14 (2%) with other hereditary disease variants. 2021 NCCN guideline-directed testing would have missed 59% (42/71) of these patients. Incremental high penetrance cancer gene variants included: BRCA1 – endometrial cancer (n = 1); BRCA2– cholangiocarcinoma (n = 1), colon neuroendocrine tumor (n = 1), and lung (n = 2), endometrial (n = 1), and non-epithelial ovarian (n = 1) cancers; MSH6 – duodenal cancer (n = 1); SDHAF2 – non-melanoma skin cancer (n = 1); SDHB – head/neck cancer (n = 1); and TP53 – CNS tumor (n = 1) and head/neck cancer (n = 1). We identified patients at risk for other hereditary diseases including hypertrophic/dilated cardiomyopathy (n = 1), malignant hyperthermia (n = 3), arrhythmogenic right ventricular cardiomyopathy (n = 1), Romano-Ward long QT / Brugada syndromes (n = 4), familial hypercholesterolemia (n = 4), and ornithine transcarbamylase deficiency (n = 1). Conclusions: Reporting germline variants identified during research sequencing is feasible and can benefit cancer patients and their relatives. Though not traditionally assessed, non-cancer predisposition genetic variants may have important intervention opportunities and unexpected patterns may have oncologic implications that require further study.

Characterization of ovarian cancer survival by histotype and stage: A nationwide study in Norway.

Contemporary population-based data on ovarian cancer survival using current subtype classifications and by surgical status are sparse. We evaluated 1-, 3-, 5- and 7-year relative (and overall) survival, and excess hazards in patients with borderline tumors or invasive epithelial ovarian cancer diagnosed 2012 to 2021 in a nationwide registry-based cohort in Norway. Outcomes were evaluated by histotype, FIGO stage, cytoreduction surgery and residual disease. Overall survival was evaluated for non-epithelial ovarian cancer. Survival of women with borderline ovarian tumors was excellent (≥98.0% 7-year relative survival). Across all evaluated invasive epithelial ovarian cancer histotypes, 7-year relative survival for cases diagnosed with stages I or II disease was ≥78.3% (stage II high-grade serous). Survival for ovarian cancers diagnosed at stage ≥III differed substantially by histotype and time since diagnosis (eg, stage III, 5-year relative survival from 27.7% [carcinosarcomas] to 76.2% [endometrioid]). Overall survival for non-epithelial cases was good (91.8% 5-year overall survival). Women diagnosed with stage III or IV invasive epithelial ovarian cancer and with residual disease following cytoreduction surgery had substantially better survival than women not operated. These findings were robust to restriction to women with high reported functional status scores. Patterns for overall survival were similar to those for relative survival. We observed relatively good survival with early stage at diagnosis even for the high grade serous histotype. Survival for patients diagnosed at stage ≥III invasive epithelial ovarian cancer was poor for all but endometrioid disease. There remains an urgent need for strategies for risk reduction and earlier detection, together with effective targeted treatments.

Abstract CT161: A phase II basket trial of dual anti-CTLA-4 and anti-PD-1 blockade in rare tumors (DART) SWOG S1609: the non-epithelial ovarian tumor cohort

Abstract Background: Dual checkpoint inhibition with Anti-PD-1 and anti-CTLA4 checkpoint inhibitors have proven to be effective in several malignancies but their potential role in numerous rare solid cancers is yet to be established. This study presents the first results of ipilimumab and nivolumab in the non-epithelial ovarian tumor cohort (#13) of the SWOG S1609 Dual Anti-CTLA-4 &amp; Anti-PD-1 blockade in Rare Tumors (DART) trial. Methods: DART is a prospective, open-label, multicenter/multi-cohort phase 2 clinical trial of ipilimumab (1mg/kg intravenously every 6 weeks) plus nivolumab (240mg intravenously every 2 weeks). This cohort included several histologies grouped for statistical analysis. The primary endpoint was objective response rate (ORR) (RECIST v1.1) (confirmed complete (CR) and partial responses (PR)); progression-free survival (PFS), overall survival (OS), stable disease (SD) &amp;gt;6 months, and toxicity are secondary endpoints. Results: Seventeen evaluable patients (median age, 64 years) were analyzed. The subtypes of non-epithelial ovarian cancer were: granulosa cell (47%, n=8), carcinosarcoma or malignant mixed Mullerian tumor (MMMT; 35%, n=6), one each for Wolffian duct, yolk sac, and Sertoli-Leydig cell. There were 2 responses in the 8 patients with granulosa cell (ORR of 25% in the granulosa cell tumors): 1CR (79% regression [due to lymph node &amp;lt; 1.0cm], 59 month PFS, juvenile type) and 1 PR (79% regression, 51+ month PFS, adult type). 6/8 patients remain alive (PFS 52-1774 days). In contrast, carcinosarcomas showed no responses. One patient with Sertoli-Leydig cell tumor had a 22% response and 341 day PFS. Overall ORR was 12% (2/17), clinical benefit rate (CBR; no progression &amp;gt; 6months) of 29.4%. The median PFS was 3.5 months, median OS was 42.5 months. The most common adverse events were fatigue (52.9%, n=9) and hypothyroidism (35.3%, n=6). Grade 3-4 adverse events occurred in 47.1% of patients (n=8). There were 3 adverse events (17.6%) that led to discontinuation, of which 2 (11.8%) were grade 3-4. There were no grade 5 adverse events. Conclusion: Ipilimumab plus nivolumab in non-epithelial ovarian cancer resulted in an ORR of 12% (with 2 of 8 patients with granulosa ovarian tumors showing a durable CR and PR [both, &amp;gt;4 years)) and CBR of 29.4%, with durable responses seen. In contrast there were no responses in the carcinosarcoma group. One patient with Sertoli Leydig cell tumor suggested a possible benefit. Correlative studies to determine response and resistance markers are ongoing. Expanded prospective studies in granulosa tumor but not carcinosarcoma are warranted. Citation Format: Young Kwang Chae, Megan Othus, Sandip Pravin Patel, Kelly J. Wilkinson, Emily M. Whitman-Purves, Jayanthi Lea, John M. Schallenkamp, Nabil Adra, Leonard J. Appleman, Mitchell Alden, Jessica Thomes Pepin, John A. Ellerton, Andrew Poklepovic, Adam Walter, Murtuza M. Rampurwala, William R. Robinson, Liam Il-Young Chung, Christine M. McLeod, Helen X. Chen, Elad Sharon, Howard Streicher, Christopher W. Ryan, Charles D. Blanke, Razelle Kurzrock. A phase II basket trial of dual anti-CTLA-4 and anti-PD-1 blockade in rare tumors (DART) SWOG S1609: the non-epithelial ovarian tumor cohort [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2023; Part 2 (Clinical Trials and Late-Breaking Research); 2023 Apr 14-19; Orlando, FL. Philadelphia (PA): AACR; Cancer Res 2023;83(8_Suppl):Abstract nr CT161.

Prognostic Factors of Non-epithelial Ovarian Cancer in a Tertiary Hospital in Indonesia

Introduction: Non-epithelial is a rare type of ovarian cancer but the most common ovarian neoplasm in reproductive age. This study analyzed the correlation of clinical characteristics to disease-free survival (DFS) and 3-year survival in non-epithelial ovarian cancer. Methods: A cohort analysis of medical records of 30 patients with non-epithelial ovarian cancer from 2016 to 2017 at Dr. Soetomo General Academic Hospital. Survival analysis was performed using Kaplan–Meier test, log-rank test, and Cox regression to determine the correlation of characteristics including age, stage, tumor size, tumor residue, histopathology type and chemotherapy status as prognostic factors for recurrence and mortality. Results: DFS was significantly affected by stage (p=0.049), tumor residue (p&lt;0.0001), and chemotherapy (p=0.005). Stage I, no residual disease, and adequate chemotherapy had the highest DFS and mean DFS rates (94.1% and 35.6 months; 95.5% and 35.7 months; 75% and 31.94 months, respectively). Highest recurrence rates were found in patients with unstaged disease (hazard ratio [HR]=10.08), residue &gt;0 cm (HR=23.13), and inadequate chemotherapy (HR=6.55). Three-year survival was significantly affected by stage (p=0.001), tumor residue (p&lt;0.0001), and chemotherapy (p&lt;0.0001). Stage I, no residual disease, and adequate chemotherapy had the highest 3-year survival rate and mean survival time (94.1% and 35.47 months; 95.5% and 35.7 months; 87.5% and 33 months). The highest mortality were found in patients with unstaged disease (HR=19.99), residue &gt;0 cm (HR=11.33), and inadequate chemotherapy (HR=11.71). Conclusion: Stage, tumor residue, and chemotherapy status in patients with non-epithelial ovarian cancer are significant prognostic factors for DFS and 3-year survival.

14P Unravelling the immune landscape of non-epithelial ovarian cancer

14P Unravelling the immune landscape of non-epithelial ovarian cancer

Oncological outcomes among young women with non-epithelial ovarian cancer: the YOC-Care study (Young Ovarian Cancer – Care)

ObjectiveTo determine oncological outcomes and associated prognostic factors in women younger than 45 years diagnosed with non-epithelial ovarian cancer.MethodsA retrospective, multicenter Spanish study was performed including women with non-epithelial ovarian...

Abstract A083: Higher incidence of rare ovarian tumors in West Africa

Abstract There is considerable regional variation in the incidences of ovarian cancer (OC); Europe and North America have the highest rate of 10.1 and 8.7 per 100,000 respectively whereas Japan and developing countries present with lowest incidences. Recent studies showed that West and East Africa women have high proportion of non-epithelial ovarian cancer, 30% and 39.5% from West and East Africa women respectively compared to 14%, 12%, 3% and 4.12% from US Born Black, Caribbean Born Black, US Born white and Alberta women, respectively. Africa women with epithelial ovarian cancer (EOC) presented at a lower age group; median age of 52.5 years compared with 59 years and above for the other group of women. The objective of the study was to evaluate a large cohort of OC tumors from West Africa, Nigeria to determine the patients’ demographics, the diagnostic accuracy and the tumor histotypes with the aim at identifying opportunities to improve the OC diagnosis in Africa and long-term goal of reduction in mortality and morbidity associated with the disease condition. This is a multi-centered retrospective study of OC involving 19 tertiary health institutions in West Africa, Nigeria over a period of 3 years (2017-2019). The histological records were accessed with the retrieval of patients’ demographics, original diagnosis and formalin fixed paraffin embedded (FFPE) tissue blocks of the corresponding patients. The FFPEs were sent to the collaborating institution, Division of Gynecological Oncology, Miller School of Medicine, University of Miami, Florida for further diagnostic appraisal. All FFPEs were sectioned, stained with hematoxylin and eosin (H&amp;E) and reviewed by two pathologists. Immunohistochemistry (IHC) was performed on cases in which the morphology features were not diagnostic. A total of 422 FFPE ovarian tissue blocks were reviewed, corresponding to 377 patients. 238 (63.1%) of the cases fulfilled criteria for inclusion such as documentation of original diagnosis, presence of tumor on the slides, and interpretable morphologic features. 95 (39.9%) cases had some changes in their diagnosis. 5 cases (2.1%) had a complete change from malignant tumor to benign or borderline. IHC was required to perform differential diagnosis in 42 (44.2%) while the remaining 55 (56.8%) cases were made after histological secondary review. Epithelial ovarian cancer was 48.3% (115) of cases while non-epithelial ovarian cancer cases were 41 (36.4%) and other malignancies such as sarcoma, metastasis and hematological neoplasm accounted for the remaining 60 (25.2%) with 35 (58.3%) of the cases being metastatic tumors to the ovary. Our findings confirmed that West African women have a lower percentage of EOC compared to East Africa and North America women. We further demonstrated that secondary review of surgical pathology cases in a sub-specialized academic center and the use of ancillary techniques such as IHC may improve the diagnosis accuracy of OC in a low resource country. There would be need to incorporate this into the management of OC in low resource countries. Citation Format: Ayodele Joshua Omotoso. Higher incidence of rare ovarian tumors in West Africa [abstract]. In: Proceedings of the 15th AACR Conference on the Science of Cancer Health Disparities in Racial/Ethnic Minorities and the Medically Underserved; 2022 Sep 16-19; Philadelphia, PA. Philadelphia (PA): AACR; Cancer Epidemiol Biomarkers Prev 2022;31(1 Suppl):Abstract nr A083.

Disgerminom u trudnoći - prikaz slučaja

Introduction: Malignant germ cell tumors (MGCTs), as a subtype of rare non-epithelial ovarian cancers (NOEC), are most commonly found in pregnancy. Of all MGCTs, 38% are dysgerminoma. Considering the rarity of these entities, the aim of this paper is to show a rare case of ovarian dysgerminoma presented in pregnancy and its influence on course and outcome of the pregnancy. Patient Review: Patient aged 26, gravida 2, para 1, with one vaginal delivery five years before, was admitted to the Clinic for Gynecology and obstetrics in term pregnancy because of uterine contractions accompanied by left thigh pain and tingling sensation in the left leg. Solid hypoechogenic mass with regular borders, 125x90 mm in diameter adjacent to the left side of the uterus was seen by ultrasound, without free fluid in pelvic cavity. Since regular uterine contractions started, the decision was made to terminate pregnancy by Caesarean section (CS) because of tumor previa. Histopathological examination confirmed ovarian dysgerminoma, but after staging operation which was performed two months after CS, following imaging diagnostics, ovarian dysgerminoma was confirmed with FIGO stage IA, meaning that patient's specific oncological treatment was finished. Conclusion: Diagnosis of ovarian dysgerminoma is in general challenging since up to 50% are asymptomatic or symptoms are non-specific. The management of ovarian cancer in pregnancy should be multidisciplinary and individualized in the best interest of the mother and the fetus. The overall five-year survival rate for ovarian dysgerminoma is favorable in more than 90% of cases. Women diagnosed with dysgerminoma in pregnancy are young and in general have good feto-maternal outcome.

Hormone replacement in premenopausal women treated with bilateral oophorectomy for ovarian cancer – a nationwide population-based study

ObjectiveTo study the extent of hormone replacement therapy (HRT) dispensing in premenopausal women after being treated with bilateral salpingo-oophorectomy (BSOE) for ovarian cancer (OC). MethodsNationwide population- and register-based cohort study including women 18–50 years old, registered in The Swedish Quality Register for Gynecological Cancer (SQRGC), where BSOE was performed due to epithelial (EOC) and non-epithelial ovarian cancers (NEOC) or borderline ovarian tumor (BOT) between 2008 and 2014. Data on HRT dispensing was obtained from the National Prescribed Drug Register analyzed at semi-annual intervals from surgery until end of follow-up December 2015, including a logistic regression analysis. ResultsA cohort of 664 women were identified with OC, whereas 396 women had an EOC, 61 a NEOC and 207 a BOT. At surgery 49% of the women were ≤44 years. HRT dispensed to the total cohort varied between 32% and 41% the first five years after surgery. During follow-up at first 0.5–1 year 51% of the women <40 years were dispensed HRT compared to 25% of women ≥40 years. Of women with EOC, 21% dispensed HRT at first 0.5–1 year. In the multivariable regression analysis; age <40 (OR6.17, p < 0.001) and age 40–44 (OR2.95, p < 0.001) as well as BOT histology (OR3.84, p < 0.001) were found significant variables for dispensing of HRT. ConclusionA majority of premenopausal women undergoing BSOE for OC did not use HRT postoperatively. Our study shows that there is a need to address HRT use after OC treatment in young women to prevent from morbidity and a poorer quality of life.

535P Long term quality of life after chemotherapy among nonepithelial ovarian cancer survivors: The case-control vivrovaire rare tumours study

Treatments (ttt) of non-epithelial rare germ cell tumors (GCT) and sex cord stromal tumors (SCST) are associated with long survival. The standard treatment mainly includes conservative surgery combined with chemotherapy (CT) [bleomycin, etoposide and cisplatin (BEP)] depending on stage and prognostic factors. As reported in testicular cancer survivors, BEP may induce late side effects with negative impact on quality of life (QOL). The French Rare Malignant Gynecological Tumors (TMRG)/GINECO case-control VIVROVAIRE Rare tumors study assessed long term QOL among survivors treated with BEP as compared to age-matched healthy women (HW). Non-epithelial ovarian cancer survivors (nEOCS), cancer-free ≥2 years after end of treatment, were identified from the INCa French Network for TMRG. HW were issued from the ‘Seintinelles’ research platform. QOL (FACT-G and FACT-O), chronic fatigue (MFI), anxiety/depression (HADS), insomnia (ISI), neurotoxicity (FACT/GOG-NTX), cognition (FACT-COG) and sexuality items (from the FACT-O OCS) were compared between nEOCS and HW. A minimal 5% difference for the score between groups was considered as clinically relevant. 144 nEOCS were included with 144 age-matched HW (mean age at inclusion: 38 yrs; 60% aged below 40). Most nEOCS were treated for GCT (n=112). Median delay from the end of ttt to inclusion was 6 yrs. At inclusion, 42% of nEOCS were menopausal versus 17% of HW (p<0.001). General and ovarian QOL, fatigue, anxiety/depression and insomnia scores were similar between nEOCS and HW. nEOCS reported clinically significant (6%) better social functioning (p=0.006). However, nEOCS reported more perceived cognitive impairment than HW (31 vs 14%, p<0.001) and clinically significant (8%) neurotoxicity (p<0.001). In addition, nEOCS reported less interest in sex (35% vs 55%, p<0.001), felt less like a woman (67% vs 81%, p=0.012), and were more afraid of not having children (31% vs 13%, p=0.007) than HW, whatever the menopausal status. 6 yrs after BEP CT, most of nEOCS reported similar global QOL as HW, but experienced more risk of premature menopause, some late side effects on cognition, neurotoxicity and sexuality that may impact their daily life.

The rate of germ cell and sex cord stromal ovarian cancer is increasing at different rates in the US and in Asia: Why? (185)

<b>Objectives:</b> To study the incidence and trends of non-epithelial ovarian cancer (OC) in the United States as compared to Asia. <b>Methods:</b> Data were obtained from the United States Cancer Statistics (USCS) Public Use Databases and the Taiwan Cancer Registry of Taiwan Health and Welfare Data Center (HWDC) from 2001 to 2017. SEER*Stat 8.3.9, Joinpoint regression program 4.8.0.1, and Excel were used to calculate the incidence and trends. <b>Results:</b> From 2001 to 2017, the incidence of germ cell and sex cord-stromal tumors in the US was 0.42 and 0.26 per 100,000, while In Asia, the incidence was 0.74 and 0.33 per 100,000, respectively. While the average annual percent change (AAPC) has been increasing for non-epithelial OC in both the US and Asia, the increase is markedly greater in Asia. In Asia, the incidence is increasing at three times the rate for sex cord-stromal tumors (1.7 vs 5.7 AAPC) and at nearly 5.5 times the rate for germ cell tumors (0.3 vs 1.6 AAPC), compared to the US. For germ cell tumors, the peak age incidence is younger in the US versus Asia (20-24 years vs 25-29 years, respectively). While for sex cord-stromal tumors, the peak age incidence is older in the US versus Asia (65-69 years vs 50-54 years, respectively). <b>Conclusions:</b> The overall incidence of germ cell and sex cord-stromal OCs is increasing in both the US and Asia. However, it is approximately 50 percent higher in Asia as compared to the US. Further investigation should investigate the role that genetic and environmental factors play in explaining these trends.

Nonepithelial ovarian cancer – the current clinical practice in the Nordic countries. Survey from the surgical subcommittee of the Nordic society of gynecological oncology (NSGO)

Background Nonepithelial ovarian cancer (NEOC) represents a wide variety of rare tumors. They are often diagnosed at an early stage and have a good prognosis compared to epithelial ovarian cancer. In the Nordic countries, the total annual number of patients diagnosed with ovarian cancer, Fallopian tube cancer or primary peritoneal carcinoma (hereafter ovarian cancer) was 2281 in 2014–2018, of which 3–10% were NEOC. International guidelines for diagnosis, treatment and follow-up have been developed. We present the results of a survey, aiming at clarifying current clinical practice in the Nordic countries. Material and Methods Between 09.2020 and 02.2021 a 33-question electronic survey was distributed to 22 hospitals in Finland, Sweden, Norway, Iceland and Denmark via the Nordic Society of Gynecological Oncology (NSGO) National Representatives. Data were collected in a secure web-based software platform. The questionnaire focused on demographics, diagnosis, treatment and follow-up programs. Results Twenty-one (95,4%) centers completed the survey. A total of 155 annual new NEOC cases treated in the Nordic countries were reported, corresponding to approximately 7% of all ovarian cancer cases. Most centers measured some or all of the recommended biomarkers routinely. Vaginal ultrasound and computed tomography (CT) were the preferred imaging modalities. The majority of centers conducted multidisciplinary team (MDT) meetings. The primary reported treatment was surgery. In 65% of centers, lymph node dissection was only performed in cases with suspicious lymph nodes. Surveillance was usually offered > four years. Discussion Despite, the presence of clinical European guidelines, variation in the current clinical practice amongst participating centers adhering to national guidelines was observed. Prospective clinical national research programs are sparse, and an enhanced cooperation in the Nordic countries toward development of a Nordic guideline and database is highly warranted and a prerequisite for future research, preferably in cooperation with the larger international groups.

Non-Epithelial Ovarian Cancers: How Much Do We Really Know?

Non-epithelial ovarian cancers (NEOC) are a group of uncommon malignancies that mainly includes germ cell tumours (GCT), sex cord-stromal tumours (SCST), and some extremely rare tumours, such as small cell carcinomas and sarcomas. Each of these classifications encompasses multiple histologic subtypes. The aetiology and molecular origins of each sub-group of NEOC require further investigation, and our understanding on the genetic changes should be optimised. In this article, we provide an update on the clinical presentation, pathology, genetics, treatment and survival of the main histological subtypes of the GCT and the SCST, as well as of ovarian small cell carcinomas. We also discuss miRNA expression profiles of NEOC and report the currently active clinical trials that include NEOC.

Adjuvant and post-surgical treatment in non-epithelial ovarian cancer.

Non-epithelial cancers arising from the ovary are uncommon malignancies. Germ cell tumors of the ovary arise from primordial germ cells, and sex cord-stromal tumors of the ovary represent a cluster of tumors arising from the sex cord and stromal compartment. Most patients diagnosed with germ cell tumors are young adults and adolescent females. In contrast, ovarian sex cord-stromal tumors more commonly occur in a mature age group. Advances in the adjuvant management of non-epithelial ovarian cancer following optimal surgical and pathological staging have improved patient survival outcomes. In addition, active surveillance is preferentially assigned to patients diagnosed with stage I germ cell tumor, stage 1A grade 1 immature teratoma, stage 1A yolk sac tumor, and stage 1AI sex cord-stromal tumors. This article discusses the importance of selecting the adjuvant treatment approach most suitable to the patients' surgical and pathological stages, thereby safeguarding patient outcomes.