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Objectives: Ovarian granulosa cell tumors (GCT) are rare tumors with a late recurrence and a good prognosis. The current study investigated the fertility and obstetrics situation, survival, and the factors influencing the mortality of patients with these uncommon ovarian neoplasms. Materials and Methods: This is a retrospective study on ovarian GCT patients admitted to the Al-Zahra hospital oncology department, the tertiary referral hospital in Tabriz, between 2009 and 2022. Data were collected from medical records. Chi-square/Fisher exact tests and t tests were used to compare categorical and quantitative variables between the alive and dead patients, respectively. The Kaplan-Meier curve was used to present patients' survival. Results: The study involved 65 patients with ovarian GCT. The presence of ovarian cysts statistically increased the survival of GCT patients (P = 0.028). The advanced tumor stage (P = 0.023), fast tumor growth (P = 0.001), and tumor relapse (P = 0.001) are significantly correlated with mortality in the affected patients. However, age and adjuvant chemotherapy were not associated with survival. Conclusions: There was no evidence of increased survival with the use of adjuvant chemotherapy. Tumor staging is an important prognostic factor. Advanced stages were associated with inferior survival, and only prospective studies can ascertain their definite role. [ABSTRACT FROM AUTHOR]

Mucinous ovarian tumors (MOTs) include primary and secondary neoplasms, the latter of which contribute for 80% of all cases. The most common site of origin for secondary MOTs is the gastrointestinal tract. Proper differentiation between primary and metastatic lesions is essential for effective treatment. Currently, definitive diagnosis is made based on post-operative histopathological examination with the use of immunohistochemical markers. However, the final diagnosis presents a challenge because of the histopathological similarity between mucinous metastases and primary ovarian lesions. Generally, treatment consists of cytoreductive surgery and adjuvant chemotherapy, even though malignant tumors are found to be chemo-resistant. Prognosis depends on the type of the tumor, presence of metastases and patient's general condition. Further research on the genetic background of MOTs is necessary for the better understanding of their origin and more effective treatment. This review aims to summarize recent advances in the field of the molecular features of MOTs and their implications for the diagnostic pathways and potential adjuvant therapy options. The analysis of molecular alterations might not only be an important prognostic factor, but also a useful diagnostic tool in distinguishing between primary mucinous tumors and extra-ovarian metastases or other subtypes of epithelial ovarian neoplasms. Moreover, the examination of genetic mutations seems to increase the efficiency of targeted therapy. However, more research evaluating such therapies in pre-clinical models is needed to improve the results of the diagnostics and treatment of MOTs. [ABSTRACT FROM AUTHOR]

Background: Mucinous ovarian tumors are a rare subtype of epithelial ovarian tumors, accounting for approximately 3% of all cases. These tumors exhibit unique clinical and pathological characteristics, with a particularly poor prognosis in advanced stages due to low responsiveness to platinum-based chemotherapy. Objective: This study aims to analyze the relationships between clinicopathological characteristics in patients with mucinous ovarian tumors treated at a single center in Indonesia between 2019 and 2023. Materials and Methods: A correlational study from March to June 2024 analyzed patients with confirmed mucinous ovarian tumors in Indonesia, focusing on age, histopathological grade, FIGO stage, CA-125 levels, tumor size, and metastasis presence. Results: A study of 123 mucinous ovarian tumor patients found that 57.7% were over 40. Histopathological analysis revealed 21.1% were benign, 26% borderline, and 52.8% malignant. Patients over 40 had more malignant tumors, advanced-stage disease, and omental metastasis. A significant correlation was found between malignant histopathology and advanced FIGO stage, as well as between advanced FIGO stage and both lymph node and omental metastasis. Tumor size was associated with elevated CA-125 levels and bilateral tumors. Conclusion: Mucinous ovarian tumors in patients over 40 years old are more likely to be malignant, present at an advanced stage, and involve omental metastasis. Malignant histopathological results are associated with advanced FIGO stages, which in turn are linked to lymph node and omental metastasis. Tumors larger than 10 cm tend to have elevated CA-125 levels. [ABSTRACT FROM AUTHOR]

Simple Summary: Gynecological cancers affect many women of reproductive age, necessitating the development of fertility preservation methods to fulfill family planning. Consequently, providing treatment options that preserve fertility in women diagnosed with gynecological cancers has become a crucial component of care for survivors. This leads to improved quality of life, allowing survivors to become mothers even in the seemingly adverse circumstances posed by cancers. However, although there are guidelines on fertility preservation in the context of neoplasms, physicians do not routinely consider it and do not discuss these options with their patients. It is important for patients to be informed about the available options for fertility preservation and to be encouraged to make informed decisions in collaboration with their medical team. Standardized guideline recommendations for onco-fertility should be considered in the future. We explore possible methods that can be employed for obtaining a pregnancy after gynecologic cancer treatment, including more exotic ones like uterine transplant. (1) Background: Currently, an increasing number of women postpone pregnancy beyond the age of 35. Gynecological cancers affect a significant proportion of women of reproductive age, necessitating the development of fertility preservation methods to fulfill family planning. Consequently, providing treatment options that preserve fertility in women diagnosed with gynecological cancers has become a crucial component of care for survivors. (2) Methods: We conducted an extensive search of relevant scientific publications in PubMed and Embase databases and performed a narrative review, including high-quality peer-reviewed research on fertility after being treated for gynecologic cancers, reporting pregnancy rates, birth rates, and pregnancy outcomes in cancer survivors as well as therapeutic options which partially preserve fertility and methods for obtaining a pregnancy in survivors. (3) Discussion: The medicine practiced today is focused on both treating the neoplasm and preserving the quality of life of the patients, with fertility preservation being an important element of this quality. This leads to an improved quality of life, allowing these women to become mothers even in the seemingly adverse circumstances posed by such a pathology. However, although there are guidelines on female fertility preservation in the context of neoplasms, an analysis shows that physicians do not routinely consider it and do not discuss these options with their patients. (4) Conclusions: Advancements in medicine have led to a better understanding and management of gynecological neoplasms, resulting in increased survival rates. Once the battle against these neoplasms is won, the issue of preserving the quality of life for these women arises, with fertility preservation being an important aspect for women who have not yet fulfilled their family planning desires at the time of diagnosis. It is important for patients to be informed about the available options for fertility preservation and to be encouraged to make informed decisions in collaboration with their medical team. Standardized recommendations for onco-fertility into guidelines should be taken into consideration in the future. [ABSTRACT FROM AUTHOR]

Simple Summary: Ovarian cancer is the eighth most common cancer among women globally and 207,000 women die every year, whereas endometriosis affects around 10% of women of reproductive age. Endometriosis-associated ovarian cancers (EAOCs) frequently arise from ectopic endometrium (i.e., the presence of endometrial/stromal cells outside the uterine cavity) in the ovary. Over the past decades, there has been an increasing volume of evidence to suggest that signalling centred around the mechanistic target of rapamycin (mTOR) plays an important role in cellular functions such as proliferation, survival, and autophagy. This review summarizes the current landscape of mTOR signalling in these gynaecological malignancies and the emerging therapeutic options. Ovarian cancer is an umbrella term covering a number of distinct subtypes. Endometrioid and clear-cell ovarian carcinoma are endometriosis-associated ovarian cancers (EAOCs) frequently arising from ectopic endometrium in the ovary. The mechanistic target of rapamycin (mTOR) is a crucial regulator of cellular homeostasis and is dysregulated in both endometriosis and endometriosis-associated ovarian cancer, potentially favouring carcinogenesis across a spectrum from benign disease with cancer-like characteristics, through an atypical phase, to frank malignancy. In this review, we focus on mTOR dysregulation in endometriosis and EAOCs, investigating cancer driver gene mutations and their potential interaction with the mTOR pathway. Additionally, we explore the complex pathogenesis of transformation, considering environmental, hormonal, and epigenetic factors. We then discuss postmenopausal endometriosis pathogenesis and propensity for malignant transformation. Finally, we summarize the current advancements in mTOR-targeted therapeutics for endometriosis and EAOCs. [ABSTRACT FROM AUTHOR]

Objective: To define the clinical, histopathological features and the prognostic factors affecting survival in patients with adult granulosa cell tumors of the ovary (AGCT). Methods: A 322 patients whose final pathologic outcome was AGCT treated at nine tertiary oncology centers between 1988 and 2021 participated in the study. Results: The mean age of the patients was 51.3 ± 11.8 years and ranged from 21 to 82 years. According to the International Federation of Gynecology and Obstetrics 2014, 250 (77.6%) patients were stage I, 24 (7.5%) patients were stage II, 20 (6.2%) patients were stage III, and 3 (7.8%) were stage IV. Lymphadenectomy was added to the surgical procedure in 210 (65.2%) patients. Lymph node involvement was noted in seven (3.3%) patients. Peritoneal cytology was positive in 19 (5.9%) patients, and 13 (4%) had metastases in the omentum. Of 285 patients who underwent hysterectomy, 19 (6.7%) had complex hyperplasia with atypia/endometrial intraepithelial neoplasia, and 8 (2.8%) had grade 1 endometrioid endometrial carcinoma. It was found that 93 (28.9%) patients in the study group received adjuvant treatment. Bleomycin, etoposide, cisplatin was the most commonly used chemotherapy protocol. The median follow-up time of the study group was 41 months (range, 1-276 months). It was noted that 34 (10.6%) patients relapsed during this period, and 9 (2.8%) patients died because of the disease. The entire cohort had a 5-year disease-free survival (DFS) of 86% and a 5-year disease-specific survival of 98%. Recurrences were observed only in the pelvis in 13 patients and the extra-abdominal region in 7 patients. The recurrence rate increased 6.168-fold in patients with positive peritoneal cytology (95% confidence interval [CI]=1.914-19.878; p=0.002), 3.755-fold in stage II-IV (95% CI=1.275-11.063; p=0.016), and 2.517-fold in postmenopausal women (95% CI=1.017-6.233; p=0.046) increased. Conclusion: In this study, lymph node involvement was detected in 3.3% of patients with AGCT. Therefore, it was concluded that lymphadenectomy can be avoided in primary surgical treatment. Positive peritoneal cytology, stage, and menopausal status were independent prognostic predictors of DFS. [ABSTRACT FROM AUTHOR]

Simple Summary: Ovarian cancer is the leading cause of death due to gynecologic malignancies. Peritoneal metastases represent the most common pathway for the spread of OC, both at the time of initial diagnosis and at recurrence. Accurate mapping of peritoneal metastases helps in planning the appropriate therapeutic strategy, predicting the likelihood of optimal cytoreduction, and identifying potentially unresectable or difficult disease sites that may require surgical technique modifications. Preoperative diagnostic work-up with multidetector CT (MDCT), MRI, including diffusion-weighted imaging (DWI), or FDG PET/CT plays a vital role in the accurate assessment of the extent of peritoneal carcinomatosis. In this article, the aim was to update the role of MDCT, MRI, including DWI, and FDG PET/CT in the detection of peritoneal metastases in ovarian cancer by conducting a systematic review and meta-analysis of the existing literature. This review aims to compare the diagnostic performance of multidetector CT (MDCT), MRI, including diffusion-weighted imaging, and FDG PET/CT in the detection of peritoneal metastases (PMs) in ovarian cancer (OC). A comprehensive search was performed for articles published from 2000 to February 2023. The inclusion criteria were the following: diagnosis/suspicion of PMs in patients with ovarian/fallopian/primary peritoneal cancer; initial staging or suspicion of recurrence; MDCT, MRI and/or FDG PET/CT performed for the detection of PMs; population of at least 10 patients; surgical results, histopathologic analysis, and/or radiologic follow-up, used as reference standard; and per-patient and per-region data and data for calculating sensitivity and specificity reported. In total, 33 studies were assessed, including 487 women with OC and PMs. On a per-patient basis, MRI (p = 0.03) and FDG PET/CT (p < 0.01) had higher sensitivity compared to MDCT. MRI and PET/CT had comparable sensitivities (p = 0.84). On a per-lesion analysis, no differences in sensitivity estimates were noted between MDCT and MRI (p = 0.25), MDCT and FDG PET/CT (p = 0.68), and MRI and FDG PET/CT (p = 0.35). Based on our results, FDG PET/CT and MRI are the preferred imaging modalities for the detection of PMs in OC. However, the value of FDG PET/CT and MRI compared to MDCT needs to be determined. Future research to address the limitations of the existing studies and the need for standardization and to explore the cost-effectiveness of the three imaging modalities is required. [ABSTRACT FROM AUTHOR]

Simple Summary: In the present review, we illustrate the interests of molecular pathology for establishing an integrated histomolecular diagnosis of ovarian sex cord–stromal tumors as well as its use for prognosis and treatment. We discuss the key morphological, immunohistochemical and molecular features of each entity, as well as their respective differential diagnoses. This review is organized from the predominant cell morphology to the molecular pathology, based on a practical point of view for the pathologist. Five groups are defined: (i) Group 1: predominance of fibromatous/thecomatous cells and/or stromal cells of unusual morphology; (ii) Group 2: predominance of steroid or luteinized cells; (iii) Group 3: predominance of follicular cells; (iv) Group 4: predominance of Sertoli cells; and (v) Group 5: predominance of sarcomatoid/unclassified/poorly differentiated cells. Diagnostic algorithms are proposed to differentiate each entity within the sex cord–stromal tumor category, and the contribution of molecular pathology for diagnostic purposes is discussed. Ovarian sex cord–stromal tumors (SCSTs) account for 8% of all primary ovarian neo-plasms. Accurate diagnosis is crucial since each subtype has a specific prognostic and treatment. Apart from fibrosarcomas, stromal tumors are benign while sex cord tumors may recur, sometimes with a significant time to relapse. Although the diagnosis based on morphology is straightforward, in some cases the distinction between stromal tumors and sex cord tumors may be tricky. Indeed, the immunophenotype is usually nonspecific between stromal tumors and sex cord tumors. Therefore, molecular pathology plays an important role in the diagnosis of such entities, with pathognomonic or recurrent alterations, such as FOXL2 variants in adult granulosa cell tumors. In addition, these neoplasms may be associated with genetic syndromes, such as Peutz–Jeghers syndrome for sex cord tumors with annular tubules, and DICER1 syndrome for Sertoli–Leydig cell tumors (SLCTs), for which the pathologist may be in the front line of syndromic suspicion. Molecular pathology of SCST is also relevant for patient prognosis and management. For instance, the DICER1 variant is associated with moderately to poorly differentiated SLCTS and a poorer prognosis. The present review summarizes the histomolecular criteria useful for the diagnosis of SCST, using recent molecular data from the literature. [ABSTRACT FROM AUTHOR]

Simple Summary: The prognosis of ovarian cancer patients is intricately shaped by a multitude of factors, and there exist several pivotal favorable prognostic factors that exert a discernible influence on survival. In this study, we analyzed factors influencing the survival of ovarian cancer patients in Cyprus. We examined data from 106 women with ovarian cancer and found that the FIGO stage, type of surgery, and performance status were significant factors associated with overall survival (OS). Progression-free survival (PFS) was influenced by FIGO stage and performance status. For patients with high-grade serous carcinoma, the performance status, FIGO stage, and type of surgery were initially considered prognostic factors, but in multivariate analysis, only performance status and FIGO stage remained statistically significant for OS. These findings contribute to our understanding of disease and could help improve treatment and patient outcomes. Objective: To analyze the factors associated with overall survival (OS) and progression-free survival (PFS) in patients with ovarian cancer in Cyprus. Methods: We retrospectively analyzed data from patients with histologically confirmed epithelial ovarian cancer (EOC) and primary peritoneal cancer (PPC). Results: A total of 106 women diagnosed with ovarian cancer were included, with a median age at diagnosis of 58 years. The Kaplan–Meier survival analysis showed a median OS of 41 months (95% C.I = 36.9, 45.1), and the FIGO stage (p < 0.001), type of surgery (p < 0.001) and performance status (p < 0.001) were identified as statistically significant prognostic factors for OS. PFS analysis revealed the FIGO stage (p = 0.006) and the performance status (p < 0.001) as significant prognostic factors. Additionally, a Cox regression analysis for median OS was performed for patients with high-grade serous carcinoma, identifying the performance status, FIGO stage, and type of surgery as prognostic factors in univariate analysis. However, in the subsequent multivariate analysis, the performance status and the FIGO stage were confirmed to be the only statistically significant prognostic factors for OS (p < 0.05). Conclusions: This study confirms that the FIGO stage, performance status, and surgery type were considered as prognostic factors for OS in ovarian cancer. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the clinicopathological characteristics of primary mucinous ovarian carcinoma (MOC) and define oncologic outcomes. Material and Methods: This retrospective study reviewed patients diagnosed with primary MOC at a single institution and underwent primary treatment between 1990 and 2019. The clinicopathological factors affecting oncological outcomes and treatment response were evaluated. The Kaplan-Meier method was used to evaluate survival outcomes. Survival curves were compared using the log-rank test. Results: The cohort's (n=92) median (range) age was 48 (15-82) years. Seventy-five (81.5%) patients were in the International Federation of Gynecology and Obstetrics stage I-II. Forty patients received platinum-based adjuvant chemotherapy. The 5-year progression-free survival was 98% in stage I-II and 17% for stage III-IV (p<0.001). In multivariate analysis, the only independent risk factor for disease failure was stage (hazard ratio: 6.838, 95% confidence interval: 1,358-34,415; p=0.020). Conclusion: Advanced stage was an independent poor prognostic factor for recurrence in patient with MOC. [ABSTRACT FROM AUTHOR]

Our study aimed to analyze the prognosis and reproductive outcomes of patients with advanced-stage serous borderline ovarian tumors (SBOTs) who underwent fertility-sparing surgery (FSS). This study included patients aged ≤ 45 years diagnosed with advanced-stage (International Federation of Gynecology and Obstetrics II and III) SBOTs who were treated with FSS. Conservative surgeries were performed in 65 patients with advanced-stage SBOT with a median age of 28 years (range, 16–44 years). Nine patients had invasive implants. The median follow-up was 81.7 months. Forty-six patients (70.8%) had a relapse (median time to first recurrence, 22.8 months). Thirteen patients subsequently developed recurrence as an invasive disease, and two died due to disease progression. After multivariate analysis, age < 30 years and incomplete cytoreduction were independent risk factors for recurrence. Invasive implants and postoperative residual tumors were significantly associated with shorter disease-free survival. Of 35 patients attempting to conceive, 12 underwent assisted reproductive technology. Additionally, 19 pregnancies, including 15 full-term births, were documented. FSS provides a good chance of reproductive success in women with advanced-stage SBOT who desire fertility preservation, but it has a high recurrence rate and risk of malignancy transformation. Patients with invasive implants should be strictly selected for FSS. [ABSTRACT FROM AUTHOR]

Ovarian cancer is the worst prognostic gynaecological cancer and represents a grave clinical and social problem. Therefore, the study aimed to assess female patients' emotional, cognitive, physical, and social quality of life. The study included 100 patients diagnosed with ovarian cancer and treated with chemotherapy in a day hospital setting at the Department of Radiotherapy and Gynaecological Oncology at the Wielkopolska Oncology Centre in Poznań. The patients were given a standard treatment regimen: paclitaxel 175 mg/m2 in a 3 h infusion and carboplatin at an AUC of 6 (5–7) following Calvert as a 1 h infusion for six cycles administered every 21 days. In addition, standardised questionnaires of the Polish version of the EORTC QLQ-C30 and QLQOV28 were used. The analysis of the collected material shows that the patients reported the highest level of general health and quality of life at the study's first stage, i.e., before chemotherapy (mean value of 59.67 points). In contrast, the patients' lowest level of general health and quality of life was observed in the fourth stage of the study (mean value of 45.04 points). The problem of side effects, such as nausea and vomiting, affected the entire study group and was more troublesome in the final stage of treatment for all patients. In the study's first stage, the mean score on the nausea and vomiting symptom scale was 16 points; in the fourth stage, the mean score was 40.07. Of the clinical factors, the symptom of fatigue was the most severe health problem for the subjects. The mean score of the fatigue scale in the study's first stage was 37.11 points, while a score of 70.33 was obtained in the fourth stage of the research. The multivariate linear regression model showed that the lack of professional activity lowers quality of life, especially combined with other side effects of chemotherapy, including hair loss in Stage IV of the study. This study shows that women with ovarian cancer undergoing chemotherapy need exceptional support from psychologists, nurses, dieticians, and physiotherapists. [ABSTRACT FROM AUTHOR]

Simple Summary: Malignant bowel obstruction (MBO) requires adequate palliation in progressive ovarian cancer. Percutaneous endoscopic gastrostomy has limitations in 32.7% of patients with advanced/recurrent ovarian cancer due to anatomical constraints and a lack of transillumination. CT-guided gastrostomy is a safe and effective procedure, enabling rapid recognition of the anatomy, particularly in complex patients with peritoneal carcinomatosis and previous multivisceral surgeries. This technique should be particularly emphasized within the palliative management of MBO. A clinical registry should be implemented to evaluate the effectiveness of various treatment strategies in MBOs associated with gynaecological cancers. Peritoneal carcinomatosis-associated malignant bowel obstruction is a common feature that merits more attention in advanced and recurrent ovarian cancer. Decompressive gastrostomy is one of the most preferred methods to palliate distressing symptoms and maintain patients' quality of life. We retrospectively identified 31 patients with ovarian cancer-associated MBO, who underwent decompressive CT fluoroscopy-guided percutaneous gastrostomy (CT-PG) between September 2015 and April 2023 at our institution. A systematic literature review was conducted for CT-guided gastrostomy in ovarian cancer. Prior to CT-PG, 27 (87%) patients underwent unsuccessful attempts at endoscopic gastrostomy or surgery due to bowel obstruction; a total of 55% had received ≥3 lines of chemotherapy. CT-PG could be successfully inserted in 25 of 31 (81%) patients without grade 4–5 complications. CT-PG insertion was feasible in 76% of patients with previous unsuccessful attempts of endoscopic gastrostomy. A total of 80% of patients with a successful insertion had considerable symptom relief and could tolerate fluid intake. Mean survival after the procedure was 44.4 days. Chemotherapy could be administered in 7 of 25 (28%) patients following the CT-PG insertion. CT-guided percutaneous gastrostomy is a safe procedure that effectively manages intractable symptoms of bowel obstruction in ovarian cancer. This minimally invasive technique should be emphasised as a routine instrument within the palliative management of MBO. [ABSTRACT FROM AUTHOR]

Simple Summary: In this commentary, I delve into the complexities and potential of the recently proposed 2023 FIGO staging system for endometrial cancer, with a focus on the incorporation of molecular classifications. I aim to evaluate the predictive capacity of histology and molecular testing, emphasizing the challenge of utilizing the POLE mutation test in determining prognosis. By addressing challenges like discerning synchronous independent tumors from metastatic ones when both endometrium and ovary are involved, and considering new elements such as lymph node metastasis size, this work may inform future treatment approaches, reinforcing the indispensable role of pathologists in this evolving landscape. This commentary explores the complexities of the FIGO 2023 staging system and the inclusion of The Cancer Genome Atlas's (TCGA) molecular classification in the management of endometrial cancer. It highlights the importance of histology as a prognostic tool, while scrutinizing the merits and demerits of its application to aggressive endometrial cancers. The commentary review sheds light on the recent introductions of lymphovascular space invasion (LVSI) and lymph node metastasis size in cancer staging. It outlines the difficulties in differentiating between synchronous and metastatic endometrial and ovarian cancers, underlining their implications on treatment strategies. Furthermore, the commentary discusses the integration of molecular classifications within the FIGO 2023 framework, emphasizing the pivotal yet challenging implementation of the pathogenic POLE mutation test. The commentary concludes by reaffirming the vital role of pathologists in executing the FIGO 2023 staging system. [ABSTRACT FROM AUTHOR]

Introduction: This study aimed to describe the clinicopathological characteristics of recurrent adult granulosa cell tumor and identify the risk factors for recurrence. Material and methods: Seventy recurrent adult granulosa cell tumor patients treated in Peking Union Medical College Hospital between 2000 and 2020 were retrospectively reviewed. The primary outcomes were progression-free survival after first recurrence (PFS-R), overall survival after first recurrence (OS-R) and recurrence frequency. The Kaplan-Meier analysis, univariate and multivariate Cox proportional hazard analysis, and the Prentice, Williams and Peterson counting process (PWP-CP) model were adopted. Results: There were 70 patients included in the study, and recurrence occurred twice in more than 71% of patients, and 49.9% of patients relapsed = three times. The recurrence pattern in over half of the patients at first recurrence was multifocal and distant disease, and abdominal or pelvic mass and liver metastasis were the most common. The 5-year PFS-R was 29.3%, and the 10-year PFS-R was 11.3%; the 5-year OS-R was 94.9%, and the 10-year OS-R was 87.9%. Kaplan-Meier analysis demonstrated that patients with distant recurrence and PFS1 (PFS when first recurrence occurred) =60 months had worse PFS-R (p = 0.017, 0.018), and patients with PFS-R = 34 months had worse OS-R (p = 0.023). It demonstrated that PFS1 = 60 months (hazard ratio, HR 1.9, 95% confidence interval [CI]: 1.1-3.4, p = 0.028) was an independent risk factor for PFS-R, and local lesion at recurrence (HR 0.488, 95% CI: 0.3-0.9, p = 0.027) was an independent protective factor for PFS-R. In addition, it demonstrated that PFS-R = 33 months (HR 5.5, 95% CI: 1.2-25.3, p = 0.028) was an independent risk factor for OS-R. The PWP-CP analysis showed that laparoscopic operation (at each operation) could significantly increase recurrence times (p = 0.002, HR = 3.4), and no existence of gross residual lesion (R0) at each recurrence operation could significantly decrease recurrence frequency (p < 0.001, HR <0.001). Conclusions: The recurrence pattern in patients with recurrent adult granulosa cell tumor was characterized as late and repeated, multifocal, and distant relapse. It has been demonstrated that PFS1 = 60 months and distant lesion at recurrence are independent risk factors for PFS-R, and PFS-R = 33 months is an independent risk factor for OS-R. The PWP-CP model showed that the transabdominal approach and surgery reaching R0 could significantly decrease the recurrence frequency. [ABSTRACT FROM AUTHOR]

Simple Summary: Because ovarian germ cell tumors (OGCTs) are rare tumors, our current understanding of them is sparse; this is because few studies have investigated the molecular basis of pediatric and adult cancers. In this paper, we aim to provide an overview of the etiopathogenesis of OGCTs in children and adults, and we address the molecular landscape of these tumors. Ovarian germ cell tumors (OGCTs) are rare in adults; indeed, they occur predominantly in children, adolescents, and young adults, and they account for approximately 11% of cancer diagnoses in these groups. Because OGCTs are rare tumors, our current understanding of them is sparse; this is because few studies have investigated the molecular basis of pediatric and adult cancers. Here, we review the etiopathogenesis of OGCTs in children and adults, and we address the molecular landscape of these tumors, including integrated genomic analysis, microRNAs, DNA methylation, the molecular implications of treatment resistance, and the development of in vitro and in vivo models. An elucidation of potential molecular alterations may provide a novel field for understanding the pathogenesis, tumorigenesis, diagnostic markers, and genetic peculiarity of the rarity and complexity of OGCTs. [ABSTRACT FROM AUTHOR]

Extraskeletal Ewing sarcoma is a rare soft tissue tumor primarily affecting pediatric patients. The treatment is currently based on a multidisciplinary approach which allows, in cases of localized disease, good survival rates. We report the case of a 15-year-old female patient with a rapidly growing suspected pelvic mass misdiagnosed following the preliminary radiological exams, which assessed the findings as a mass of ovarian origin. The girl underwent surgery and, thanks to histopathological, immunohistochemical and real-time polymerase chain reaction (RT-PCR) examinations, it was possible to make the right diagnosis and to administer the best treatment in terms of surgery, chemotherapy and radiotherapy, obtaining a long disease-free interval and no recurrence to date. [ABSTRACT FROM AUTHOR]

Background and objectives: The routine application of lymphadenectomy remains a controversial part of surgical staging in malignant ovarian germ-cell tumor (MOGCT). Thus, studies are needed to explore the prognostic significance of lymphadenectomy in MOGCT. The goal of this retrospective study was to report the clinical outcomes of lymph node dissection (LND) and non-LND in MOGCT surgeries. Measurements and main results: A total of 340 cases of MOGCTs were included: 143 patients (42.1%) had LND and 197 (57.9%) had no LND. The OS rates at 5 years in the LND and non-LND group were 99.3% vs. 100%, respectively. The DFS rates at 5 years in the LND and non-LND group were 88.8% vs. 88.3%. Forty-three patients (12.6%) were successfully pregnant during the postoperative follow-up. There were 44 recurrences (12.9%) and six deaths (1.8%). Stage was an independent prognostic factor for DFS in the multivariate analysis. Pathology was reported as an independent prognostic factor associated with OS in the multivariate analysis. Conclusion: Lymphadenectomy had no significant influence on the OS (P=0.621) or disease-free survival rate (P=0.332) of patients with MOGCT. [ABSTRACT FROM AUTHOR]

Evidence indicates that different pathways of malignant degeneration underlie the development of endometriosis-associated ovarian tumors of endometrioid and clear cell histotypes. The aim of this study was to compare data from patients affected by these two histotypes to investigate the hypothesis of a dichotomy in the histogenesis of these tumors. Clinical data and tumor characteristics of 48 patients who were diagnosed with either pure clear cell ovarian cancer and mixed endometrioid–clear cell ovarian cancer arising from endometriosis (ECC, n = 22) or endometriosis-associated endometrioid ovarian cancer (EAEOC, n = 26) were compared. A previous diagnosis of endometriosis was detected more frequently in the ECC group (32% vs. 4%, p = 0.01). The incidence of bilaterality was significantly higher in the EAOEC group (35% vs. 5%, p = 0.01) as well as a solid/cystic rate at gross pathology (57.7 ± 7.9% vs. 30.9 ± 7.5%, p = 0.02). Patients with ECC had a more advanced disease stage (41% vs. 15%; p = 0.04). A synchronous endometrial carcinoma was detected in 38% of EAEOC patients. A comparison of the International Federation of Gynecology and Obstetrics (FIGO) stage at diagnosis showed a significantly decreasing trend for ECC compared to EAEOC (p = 0.02). These findings support the hypothesis that the origin, clinical behavior and relationship with endometriosis might be different for these histotypes. ECC, unlike EAEOC, seems to develop within an endometriotic cyst, thus representing a window of possibility for ultrasound-based early diagnosis. [ABSTRACT FROM AUTHOR]

Simple Summary: Patients diagnosed with early-stage ovarian cancer (OC) are treated by surgery and, if appropriate, by adjuvant chemotherapy. However, in practice, there is disagreement about both the extent of surgical staging and the indication of adjuvant chemotherapy. Therefore, we sought to structurally assess clinically relevant parameters of treatment practice and aimed to identify individual patient factors that may influence decisions regarding the extent of treatment. This study confirmed that a relevant proportion (34.2%) of early-stage OC patients were not operated on in compliance with the current standard. This was independent of patient age and hospital healthcare level. The reasons for omitting complete surgical staging were balanced. Only a subset of cases underwent BRCA1/2 testing. Though mono-chemotherapy is recommended for early-stage OC, almost every second patient receives combination treatment. Our study underlines the high need for further prospective registries and educational programs to increase awareness for adequate clinical management of patients with early-stage OC. Background: Surgery is the backbone of early-stage ovarian cancer (OC) management. However, in practice, there is disagreement about the extent of surgical staging and whether additional adjuvant treatment should be provided. As omitting relevant diagnostic or therapeutic procedures might lead to undertreatment, we aimed to structurally investigate treatment practice in addition to prognostic factors in a multicentre series of patients (pts) diagnosed with early-stage OC. Patients: Within this retrospective, multicentre study, medical records of 379 pts who had undergone surgery for suspected early-stage OC between January 2014 and March 2020 were analysed. Results: Of the 379 patients, 292 had pT stage 1a-2a and had complete data on the extent of surgical staging. At least one surgical step was omitted in 100 pts (34.2%). Complete surgical staging (n = 192; (65.8%)) was more often performed in high-grade serous OC and was independent of the healthcare level of the hospital where the initial diagnosis was made. Missing to take peritoneal biopsies was associated with reduced relapse-free-survival in incompletely staged, pT1 cases (p = 0.03). About every second patient (46.7%) with a final stage lower than FIGO IIB and treated with adjuvant chemotherapy received combination chemotherapy. BRCA1 and BRCA2 testing was only performed in a subset of pts, and mutations were detected in 18% (14/79) and 9% (7/85) pts, respectively. Conclusions: This study helps to increase our understanding of early-stage OC treatment and prognosis. In addition to treating patients in compliance with current guidelines, the need for BRCA testing should also be considered for early-stage OC. [ABSTRACT FROM AUTHOR]

Background: Granulosa cell tumors (GCT) are rare malignant ovarian tumors. The two subtypes, adult and juvenile granulosa cell tumors, differ in clinical and molecular characteristics. GCT are low-malignant tumors and are generally associated with favorable prognosis. However, relapses are common even years and decades after diagnosis. Prognostic and predictive factors are difficult to assess in this rare tumor entity. The purpose of this review is to provide a comprehensive overview of the current state of knowledge on prognostic markers of GCT to identify patients with a high risk of recurrence. Methods: Systematic research for adult ovarian granulosa cell tumors and prognosis revealed n = 409 English full text results from 1965 to 2021. Of these articles, n = 35 were considered for this review after title and abstract screening and topic-specific matching. A specific search for pathologic markers with prognostic relevance for GCT identified n = 19 articles that were added to this review. Results: FOXL2 mutation and FOXL2 mRNA were inverse and immunohistochemical (IHC) expression of CD56, GATA-4 and SMAD3 was associated with reduced prognosis. IHC analysis of estrogen receptor, Anti-Mullerian hormone (AMH) and inhibin was not associated with prognosis for GCT. Analyses of mitotic rate, Ki-67, p53, β-catenin and HER2 revealed inconsistent results. [ABSTRACT FROM AUTHOR]

Simple Summary: Mucinous ovarian cancer (MOC) is a rare tumor that can be classified according to stage in early MOC and advanced MOC. Early MOC is characterized by a good prognosis and, in most cases, surgical treatment alone represents the mainstay of therapy. Despite the good prognosis, some early-stage MOCs can relapse and exhibit aggressive behavior, and new biomarkers are emerging to better stratify these patients. Advanced MOC, instead, is characterized by a poor prognosis. Radical surgery is still the mainstay of treatment since these tumors are chemo-resistant. New targeted therapies are being studied to improve the prognosis in this setting, but unfortunately, none of these have been approved yet. This review focuses on the most relevant clinical, pathological, and molecular features and treatment options according to prognosis. This review includes state-of-the-art prognostic and predictive factors of mucinous ovarian cancer (MOC), a rare tumor. Clinical, pathological, and molecular features and treatment options according to prognosis are comprehensively discussed. Different clinical implications of MOC are described according to the The International Federation of Gynecology and Obstetrics (FIGO) stage: early MOC (stage I-II) and advanced MOC (stage III-IV). Early MOC is characterized by a good prognosis. Surgery is the mainstay of treatment. Fertility-sparing surgery could be performed in patients who wish to become pregnant and that present low recurrence risk of disease. Adjuvant chemotherapy is not recommended, except in patients with high-risk clinical and pathological features. Regarding the histological features, an infiltrative growth pattern is the major prognostic factor of MOC. Furthermore, novel molecular biomarkers are emerging for tailored management of early-stage MOC. In contrast, advanced MOC is characterized by poor survival. Radical surgery is the cornerstone of treatment and adjuvant chemotherapy is recommended, although the efficacy is limited by the intrinsic chemoresistance of these tumors. Several molecular hallmarks of advanced MOC have been described in recent years (e.g., HER2 amplification, distinct methylation profiles, peculiar immunological microenvironment), but target therapy for these rare tumors is not available yet. [ABSTRACT FROM AUTHOR]

Simple Summary: The DNA damage response (DDR) is frequently altered in ovarian cancer (OC), which can be exploited for therapeutic purposes. Moreover, targeting DDR signaling pathways has become an attractive strategy for increasing the effect of DNA-damaging drugs and overcoming chemoresistance. Here, we review the main DDR pathways and their alterations in OC. We also recapitulate the preclinical and clinical studies that target the DDR for the treatment of the disease. The DNA damage response (DDR), a set of signaling pathways for DNA damage detection and repair, maintains genomic stability when cells are exposed to endogenous or exogenous DNA-damaging agents. Alterations in these pathways are strongly associated with cancer development, including ovarian cancer (OC), the most lethal gynecologic malignancy. In OC, failures in the DDR have been related not only to the onset but also to progression and chemoresistance. It is known that approximately half of the most frequent subtype, high-grade serous carcinoma (HGSC), exhibit defects in DNA double-strand break (DSB) repair by homologous recombination (HR), and current evidence indicates that probably all HGSCs harbor a defect in at least one DDR pathway. These defects are not restricted to HGSCs; mutations in ARID1A, which are present in 30% of endometrioid OCs and 50% of clear cell (CC) carcinomas, have also been found to confer deficiencies in DNA repair. Moreover, DDR alterations have been described in a variable percentage of the different OC subtypes. Here, we overview the main DNA repair pathways involved in the maintenance of genome stability and their deregulation in OC. We also recapitulate the preclinical and clinical data supporting the potential of targeting the DDR to fight the disease. [ABSTRACT FROM AUTHOR]

Introduction: A possible cause for acute abdomen is haemoperitoneum resulting from the rupture of an ovarian tumour. Here we discuss a case of spontaneous haemoperitoneum caused by granulosa cell tumour (GCT) rupture in a postmenopausal woman. Material and methods: We present a systematic review of the current literature to draw attention to this rare gynaecological complication and provide guidance about the most appropriate management. Results: Eight case reports and one retrospective study were identified. A total of 11 patients were analysed in this review including the present case report. The first case was described in 1948, while the last one was in 2019. The mean age of the patients was 60.8 years. All cases were treated with primary surgery. The mean diameter of the masses was 10.1 cm. Discussion: We found endometrial pathology in 45% of the cases, of which 4 (36%) were associated with postmenopausal bleeding. The presentation of GCT is not always in the form of overt endocrine disturbance but can onset (10-15%) with acute abdomen. Conclusions: Granulosa cell tumour should remain in the differential diagnosis of all patients presenting with acute abdomen and imaging suspicious for gynaecological malignancy originating from the ovary. [ABSTRACT FROM AUTHOR]

Simple Summary: Ovarian cancer is still the most lethal gynecologic malignancy with a median 5-year survival of less than 50%. Most cases are diagnosed at the advanced stages and only limited improvements in overall survival are achieved by standard treatments. Despite the paucity of randomized clinical trials, combined cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) show encouraging survival outcomes in primary ovarian cancer with peritoneal metastases. It gives rise to the definite need to redefine not only the best treatment option, but also the optimal treatment sequence based on the primary tumor and the extent of disease. Ovarian cancer is the eighth most common neoplasm in women with a high mortality rate mainly due to a marked propensity for peritoneal spread directly at diagnosis, as well as tumor recurrence after radical surgical treatment. Treatments for peritoneal metastases have to be designed from a patient's perspective and focus on meaningful measures of benefit. Hyperthermic intraperitoneal chemotherapy (HIPEC), a strategy combining maximal cytoreductive surgery with regional chemotherapy, has been proposed to treat advanced ovarian cancer. Preliminary results to date have shown promising results, with improved survival outcomes and tumor regression. As knowledge about the disease process increases, practice guidelines will continue to evolve. In this review, we have reported a broad overview of advanced ovarian cancer management, and an update of the current evidence. The future perspectives of the Italian Society of Surgical Oncology (SICO) are discussed conclusively. [ABSTRACT FROM AUTHOR]

Simple Summary: Adult granulosa cell tumors (aGCTs) are a rare subtype of ovarian cancer. First choice of treatment is surgery; when this is not possible, chemotherapy and anti-hormonal therapy are often used. There is limited evidence on the effect of systemic therapy in aGCT. The aim of our systematic review is to provide an overview of the response to chemotherapy and anti-hormonal therapy in patients with aGCT. We found very few articles reporting the response to chemotherapy and anti-hormonal therapy in only aGCT. The available data showed a moderate response to chemotherapy and anti-hormonal therapy, but if patients who achieve stable disease are also taken into account, the response is higher. This may mean that surgery can be postponed for a longer period of time. For adult granulosa cell tumors (aGCTs), the preferred treatment modality is surgery. Chemotherapy and anti-hormonal therapy are also frequently used in patients with recurrent aGCT. We aimed to review the existing literature on the response to chemotherapy and anti-hormonal therapy in patients with aGCT. Embase and MEDLINE were searched from inception to November 2021 for eligible studies. Objective response rate (ORR) was calculated as the total number of cases with a complete response (CR) or a partial response (PR). Disease control rate (DCR) was defined as the sum of cases with CR, PR or stable disease (SD). A total of 10 studies were included that reported on chemotherapy and 13 studies were included that reported on anti-hormonal therapy. The response rates of the 56 chemotherapy regimens that could be evaluated resulted in an ORR of 30% and DCR of 58%. For anti-hormonal therapy, the results of 73 regimens led to an ORR of 11% and a DCR of 66%. Evidence on systemic therapy in aGCT only is limited. For both chemotherapy and anti-hormonal therapy, the ORR is limited, but the response is considerably higher when patients achieving SD are included. New approaches are needed to provide more evidence and standardize treatment in aGCT. [ABSTRACT FROM AUTHOR]

Objective: To report a single-center experience in non-epithelial malignant ovarian tumours (NEMOT), by presenting different clinical and pathological characteristics, management and outcomes. Methods: We retrospectively reviewed electronic files of all female patients who underwent surgery for NEMOT at the Gynecology Department of King Abdulaziz University Hospital, Jeddah, Saudi Arabia, from July 2003 to July 2019. We collected baseline demographic, anthropomorphic and clinical data; pathological characteristics; management and follow-up data; and outcomes including residual disease, recurrence and last follow-up status (deceased or alive). Results: Thirty-three women were included; mean (standard deviation) age = 33.24 (17.72) years, range = 4, 86 years. Granulosa cell tumor was the most frequent subtype diagnosed in 17 (51.5%) patients, followed by germ cell tumours 13 (39.4%). The majority of patients were diagnosed at FIGO Stage I (22, 66.7%) and with tumor Grade 1 (23, 69.7%), while 8 (24.2%) were diagnosed with Grade 3 tumors. Granulosa cell and Sertoli-Leydig cell tumours were diagnosed at an older age (mean age = 39.30 vs. 23.92 years) compared to germ cell tumours, respectively (P = 0.012). Two-third of the patients benefited from conservative surgery including oophorectomy + staging, and 16 (48.5%) benefited from chemotherapy with bleomycin, etoposide and platinum being the most common protocol (13, 39.4%) for germ cell tumours. Postoperatively, only 2 (6.1%) patients had residual disease. Recurrence and mortality were reported in one and four patients, respectively, resulting in recurrence rate = 3.0% (95% confidence interval [CI] = 0.01%, 15.8%) and mortality rate = 12.1% (95% CI = 3.4%, 28.2%). Conclusions: The present series of NEMOT was predominated by sex cord-stromal cell tumors, which were diagnosed in patients with older age, while germ cell tumours were underrepresented. Although survival rates were comparable to those reported internationally, more consideration should be given to following up patients regarding fertility outcomes to provide a more comprehensive evaluation of treatment success and quality of care. [ABSTRACT FROM AUTHOR]

Simple Summary: Fertility-sparing surgery (FSS) is now a widely acceptable treatment for the management of Borderline Ovarian Tumours (BOTs) in women of reproductive age. However, many clinicians face the dilemma of balancing the risks of disease recurrence with progression to lethal malignancy whilst preserving fertility, in the absence of clear standardized guidelines. The aim of this study was to evaluate the oncological outcomes in women who underwent FSS for the management of primary, or recurrent presentation of BOTs, to provide clinicians with further evidence of the safety and feasibility of FSS. Oncological outcomes following a novel method of FSS in the form of laparoscopic ultrasound guided ovarian wedge resection has also been introduced, which has the potential to change the way BOTs are managed in the future in women of reproductive age. To determine the oncological outcomes following fertility-sparing surgery (FSS) for the management of Borderline Ovarian Tumours (BOTs). A retrospective analysis of participants diagnosed with BOTs between January 2004 and December 2020 at the West London Gynaecological Oncology Centre was conducted. A total of 172 women were diagnosed; 52.3% (90/172) underwent FSS and 47.7% (82/172) non-FSS. The overall recurrence rate of disease was 16.9% (29/172), of which 79.3% (23/29) presented as the recurrence of serous or sero-mucinous BOTs and 20.7% (6/29) as low-grade serous carcinoma (LGSC). In the FSS group, the recurrence rate of BOTs was 25.6% (23/90) presenting a median 44.0 (interquartile range (IQR) 41.5) months, of which there were no episodes of recurrence presenting as LGSC reported. In the non-FSS group, all recurrences of disease presented as LGSC, with a rate of 7.7% (6/78), following a median of 47.5 months (IQR 47.8). A significant difference between the type of surgery performed (FSS v Non-FSS) and the association with recurrence of BOT was observed (Pearson Chi-Square: p = 0.000; x = 20.613). Twelve women underwent ultrasound-guided ovarian wedge resection (UGOWR) as a novel method of FSS. Recurrence of BOT was not significantly associated with the type of FSS performed (Pearson Chi- Square: x = 3.166, p = 0.379). Non-FSS is associated with negative oncological outcomes compared to FSS, as evidenced by the higher rate of recurrence of LGSC. This may be attributed to the indefinite long-term follow up with ultrasound surveillance all FSS women undergo, enabling earlier detection and treatment of recurrences. [ABSTRACT FROM AUTHOR]

Background: Pseudomyxoma peritonei is a rare disease condition mainly caused by primary mucinous tumors from the appendix and rarely from the ovary, such as when mucinous ovarian tumors arise from within a teratoma. Molecular analyses of pseudomyxoma from the appendix showed that KRAS and GNAS pathogenic variants are common genetic features of pseudomyxoma peritonei. However, the origin of the tumors is difficult to be identified via genetic variants alone. This study presents a case of pseudomyxoma peritonei of ovarian origin, which was diagnosed by comprehensive genomic profiling with ploidy analysis in a series of primary, recurrent, and autopsy tumor specimens. Case presentation: A 40-year-old woman was diagnosed with Stage IC2 mucinous ovarian tumor of borderline malignancy with mature cystic teratoma, upon clinical pathology. Immunohistochemical analysis suggested that the mucinous tumor was derived from the intestinal component of an ovarian teratoma. Three years later, intraperitoneal recurrence was detected, which subsequently progressed to pseudomyxoma peritonei. Genomic analysis detected KRAS (G12D), GNAS (R201C), and FBXW7 (R367*) variants in the primary tumor. In addition, the tumor showed aneuploidy with loss of heterozygosity (LOH) in all its chromosomes, which suggested that the primary ovarian tumor was derived from germ cells. Existence of one Barr body suggested the existence of uniparental disomy of the tumors throughout the genome, instead of a haploid genotype. All three pathogenic variants remained positive in the initial recurrent tumor, as well as in the paired DNA from the whole blood in pseudomyxoma peritonei. The pathogenic variant of KRAS (G12D) was also identified in the autopsy specimen of the appendix by droplet digital polymerase chain reaction. Conclusions: This study pathologically and genetically confirmed that the primary ovarian borderline tumor was derived from the intestinal component of an ovarian teratoma, and that the subsequent pseudomyxoma peritonei progressed from the primary ovarian tumor. Integrative genomic analysis was useful to identify cellular origin of tumors, as well as to precisely interpret the process of disease progression. [ABSTRACT FROM AUTHOR]

Objective: To describe the clinical and ultrasound characteristics of three types of rare malignant ovarian germ cell tumor: embryonal carcinoma, non-gestational choriocarcinoma and malignant mixed germ cell tumor.Methods: This was a retrospective multicenter study. From the International Ovarian Tumor Analysis (IOTA) database, we identified patients with a histological diagnosis of ovarian embryonal carcinoma, non-gestational choriocarcinoma or malignant mixed germ cell tumor, who had undergone preoperative ultrasound examination by an experienced ultrasound examiner between 2000 and 2020. Additional patients with the same histology were identified from the databases of the departments of gynecological oncology in the participating centers. All tumors were described using IOTA terminology. Three examiners reviewed all available ultrasound images and described them using pattern recognition.Results: One patient with embryonal carcinoma, five patients with non-gestational ovarian choriocarcinoma and seven patients with ovarian malignant mixed germ cell tumor (six primary tumors and one recurrence) were identified. Seven patients were included in the IOTA studies and six patients were examined outside of the IOTA studies. The median age at diagnosis was 26 (range, 14-77) years. Beta-human chorionic gonadotropin levels were highest in non-gestational choriocarcinomas and alpha-fetoprotein levels were highest in malignant mixed germ cell tumors. Most tumors were International Federation of Gynecology and Obstetrics (FIGO) Stage I (9/12 (75.0%)). All tumors were unilateral, and the median largest diameter was 129 (range, 38-216) mm. Of the tumors, 11/13 (84.6%) were solid and 2/13 (15.4%) were multilocular-solid; 9/13 (69.2%) manifested abundant vascularization on color Doppler examination. Using pattern recognition, the typical ultrasound appearance was a large solid tumor with inhomogeneous echogenicity of the solid tissue and often dispersed cysts which, in most cases, were small and irregular. Some tumors had smooth contours while others had irregular contours.Conclusions: A unilateral, large solid tumor with inhomogeneous echogenicity of the solid tissue and with dispersed small cystic areas in a young woman should raise the suspicion of a rare malignant germ cell tumor. This suspicion can guide the clinician to test tumor markers specific for malignant germ cell tumors. © 2020 International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to estimate the overall survival (OS) and relapse free survival (RFS) of the non-epithelial ovarian tumors (NEOTs) of ovarian germ cell tumors (OGCT) and ovarian sex cord tumors (OSCT) in Iranian women; we also evaluated the relative prognostic factors. Method: In this retrospective study, we screened the documents of all the women diagnosed with OGCTs and OSCTs from 2012 to 2019. We further assessed the OS, RFS, and different prognostic factors. Results: A statistically significant association existed between RFS and stage of the disease at diagnosis in OSCTs group by univariate analysis and multivariate analysis (HR: 0.25 (95% CI (0.08-0.78), P=0.01) and multivariate analysis (HR: 0.27 (95% CI (0.08-0.97), P=0.04), respectively. The kaplan-meier analysis and the Log Rrank (Mantel-Cox) showed a statistically significant relationship between the stage at diagnosis and RFS in OGCT group (P=0.042). RFS was 96% for OGCT patients, and 92.7% for OSCT patients. During the follow-up, only one patient passed away in the dysgerminoma group; the OS rate was 98% for OGCT patients, and 100% for OSCT patients. Conclusion: The OS and RFS obtained in this study confirmed that the ovarian germ cell and sex cord malignancies were among the highly treatable solid tumors. Stage can be proposed as the main prognostic factor; also, larger series of studies are needed for detecting the prognostic significance of serum markers. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to explore the clinicopathological characteristics of recurrent adult-type granulosa cell tumor of the ovary (AGCOT) and evaluated the treatment results to define the prognostic parameters for survival after recurrence. Results: A retrospective review of 40 patients with recurrent AGCOT, who were treated in the Cancer Hospital at the Chinese Academy of Medical Sciences from 2000 to 2015 was conducted. The impact of clinical and pathological characteristics, progression-free survival (PFS), and post-recurrence therapeutic approaches on prognosis were analyzed. Among the 40 recurrent patients, there were 10 cases where the relapse was uncontrolled, 24 cases had second relapses, and 6 cases without further relapses at the time of our follow-up. The median PFS was 61 months (range, 7-408 months), and the median time interval between the first and the second relapses (R-PFS) was 25 months (range, 0–94 months). The median time interval between the first relapse and death (R-OS) was 90 months (range, 2–216 months). PFS ≥ 61 months (P = 0.004) and post-recurrence therapeutic approach (P < 0.001) were independent risk factors for repeated recurrences. The age at recurrence (P = 0.031) and post-recurrence therapeutic approach (P = 0.001) were independent risk factors for death after recurrence. Conclusion: Among patients with recurrent AGCOT, those with long PFS had good prognoses. Maximal cytoreductive effort should be made after recurrence. Complete resection and postoperative adjuvant chemotherapy may improve the prognosis of patients with recurrent AGCOT. [ABSTRACT FROM AUTHOR]

Study Question: What are the oncofertility outcomes of young women (≤40 years old) with bilateral serous borderline ovarian tumors (SBOTs) after fertility-sparing surgery?Summary Answer: Fertility preservation with the bilateral ovarian cystectomy procedure is feasible for bilateral SBOTs, with an acceptable oncological outcome and worthwhile pregnancy rates.What Is Known Already: Fertility-sparing approaches are becoming the standard management of young patients with unilateral SBOTs and other borderline histological subtypes. However, there is a paucity of evidence to dictate the best management in bilateral SBOTs.Study Design, Size, Duration: This was a retrospective observational study performed at the Peking Union Medical College Hospital in Beijing, China, between January 1999 and January 2019.Participants/materials, Setting, Methods: Ninety-four women (≤40 years old) with pathologically confirmed bilateral SBOTs were included. Following preoperative counseling, patients self-selected into one of three treatment modalities: bilateral ovarian cystectomy (n = 48), unilateral adnexectomy plus contralateral cystectomy (UAC; n = 31), and radical surgery (n = 15). Univariate and multivariate analyses were used to determine the clinical and pathological features associated with disease-free survival and reproductive outcomes.Main Results and the Role Of Chance: During the median follow-up of 64 months (range, 4-243 months), 61 patients (65%) developed relapse, including 3 (20%) in the radical group, 26 (84%) in the UAC group and 32 (67%) in the bilateral cystectomy group. In the multivariate analyses, preoperative CA-125>300 U/mL, fertility preservation and micropapillary pattern were independently associated with adverse disease-free survival (P = 0.001, 0.03 and 0.026, respectively). Fourteen patients (15%) experienced invasive recurrence, and three (3%) died of progressive disease. The micropapillary pattern was significantly associated with invasive evolution risk (P = 0.006). Of the 49 patients who attempted to conceive, 23 (47%) achieved 27 pregnancies (24 spontaneous and three after IVF-ET), resulting in 19 live births. There was no significant difference in disease-free survival (P = 0.13) or pregnancy rate (41 vs. 50%, P = 0.56) between the UAC and bilateral procedures.Limitations, Reasons For Caution: As a retrospective study conducted in a referral center, inherent biases exist. The nonrandom allocation to treatment groups and relatively small number of patients attempt to conceive might limit the statistical power of our findings. Only 41 patients (43.6%) received complete staging during their initial surgeries, so an underestimation bias in terms of the FIGO stage and extraovarian implants might have occurred.Wider Implications Of the Findings: The ultraconservative bilateral ovarian cystectomy procedure should be proposed in bilateral SBOTs when technically feasible. Invasive evolution occurs frequently in these women, and intense follow-up and oncofertility counseling are warranted, especially for those with micropapillary patterns.Study Funding/competing Interest(s): No external funding was used for this study. There are no conflicts of interest to declare.Trial Registration Number: N/A. [ABSTRACT FROM AUTHOR]

Borderline ovarian tumors (BOT) account for 10-20% of all ovarian epithelial tumors, with one-third of cases diagnosed under the age of 40 [[1]]. They describe two successful cases that underwent gonadotrophin administration with concomitant letrozole, apparently without an increase in tumor volume and conclude that "oocyte cryopreservation at the time of recurrence of BOT is feasible and may be considered in the decision-making process with the patients." Is oocyte cryopreservation really feasible when the ovary harbors a BOT relapse? In conclusion, the management of fertility issues in patients diagnosed with BOT recurrence after FSS is still controversial; decisions should be individualized considering safety, feasibility, and appropriateness of each procedure. [Extracted from the article]

Objective: To describe the clinical and ultrasound characteristics of ovarian pure clear cell carcinoma.Methods: This was a retrospective study involving data from 11 ultrasound centers. From the International Ovarian Tumor Analysis (IOTA) database, 105 patients who had undergone preoperative ultrasound examination by an experienced ultrasound examiner between 1999 and 2016 were identified with a histologically confirmed pure clear cell carcinoma of the ovary. An additional 47 patients diagnosed with pure clear cell carcinoma between 1999 and 2016 and with available complete preoperative ultrasound reports were identified retrospectively from the databases of the departments of gynecological oncology in the participating centers. The ultrasound images of all tumors were described using IOTA terminology. Clinical and ultrasound characteristics were analyzed for the whole group, and separately, for patients with and those without histologically confirmed endometriosis, and for patients with evidence of tumor developing from endometriosis.Results: Median age of the 152 patients was 53.5 (range, 28-92) years and 92/152 (60.5%) tumors were FIGO Stage I. Most tumors (128/152, 84.2%) were unilateral. On ultrasound examination, all tumors contained solid components and 36/152 (23.7%) were completely solid masses. The median largest diameter of the lesion was 117 (range, 25-310) mm. Papillary projections were present in 58/152 (38.2%) masses and, in most of these (51/56, 91.1%), vascularized papillary projections were seen. Information regarding the presence, site and type of pelvic endometriosis at histology was available for 130/152 patients. Endometriosis was noted in 54 (41.5%) of these. In 24/130 (18.6%) patients, the tumor was judged to have developed from endometriosis. Patients with, compared to those without, evidence of tumor developing from endometriosis were younger (median 47.5 vs 55.0 years, respectively), and ground-glass echogenicity of cyst fluid was more common in pure clear cell cancers developing from endometriosis (10/20 vs 13/79 (50.0% vs 16.5%), respectively).Conclusions: Ovarian pure clear cell carcinoma is usually diagnosed at an early stage and typically appears as a large unilateral mass with solid components. Patients with clear cell carcinoma developing from endometriosis are younger than other patients with clear cell carcinoma, and clear cell cancers developing from endometriosis more often manifest ground-glass echogenicity of cyst fluid. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd. [ABSTRACT FROM AUTHOR]

Background: Because of the rarity of endometrioid borderline ovarian tumours (EBOTs), there is a paucity of data concerning the natural history and prognosis of this condition. Thus, the objective of our study was to establish the feasibility of fertility preservation in young women with EBOTs, as well as their oncological and reproductive outcomes.Methods: Consecutive patients with EBOTs, treated at a tertiary referral centre during a span of 22 years, were retrospectively analysed. Recurrence-free interval, as well as its association with the type of surgery and with other clinical and pathological features, was assessed using the Kaplan-Meier and Cox proportional hazards methods.Results: Of the 59 patients studied, the median follow-up time was 30 months (range, 6-177 months). Nine (15.3%) patients developed 13 recurrences 6-137 months after the initial surgeries, including three patients (5.1%; n = 3/59) who developed six invasive recurrences 8, 18 and 68 months after their initial surgeries. Conservative surgery showed a tendency towards a high recurrence rate (17.2% versus 13.3%); however, this difference was not significant (p = 0.45). The 5-year recurrence-free survival rate was significantly higher in the oophorectomy group than in the cystectomy group (p = 0.001). Cox regression analysis showed that none of the variables assessed were associated with an increased hazard ratio for recurrence, except for a younger age at diagnosis (p = 0.021). Of 20 patients who attempted to conceive, three pregnancies among two patients (10.0%) resulted in two live births.Conclusions: Conservative surgery with unilateral adnexectomy can be proposed for young women with EBOTs with fertility desire; however, the reproductive result is not satisfactory. In addition, careful evaluations of the endometria should be offered during the initial surgery and follow-up period.Trial Registration: Retrospectively registered. [ABSTRACT FROM AUTHOR]

Objective: To describe the clinical and ultrasound characteristics of ovarian pure endometrioid carcinomas.Methods: This was a retrospective multicenter study of patients with a histological diagnosis of pure endometrioid carcinoma. We identified 161 patients from the International Ovarian Tumor Analysis (IOTA) database who had undergone preoperative ultrasound examination by an experienced ultrasound examiner between 1999 and 2016, and another 78 patients from the databases of the departments of gynecological oncology in the participating centers. All tumors were described using IOTA terminology. In addition, one author reviewed all available ultrasound images and described them using pattern recognition.Results: Median age of the 239 patients was 55 years (range, 19-88 years). On ultrasound examination, two (0.8%) endometrioid carcinomas were described as unilocular cysts, three (1.3%) as multilocular cysts, 37 (15.5%) as unilocular-solid cysts, 115 (48.1%) as multilocular-solid cysts and 82 (34.3%) as solid masses. Median largest tumor diameter was 102.5 mm (range, 20-300 mm) and median largest diameter of the largest solid component was 63 mm (range, 9-300 mm). Papillary projections were present in 70 (29.3%) masses. Most cancers (188 (78.7%)) were unilateral. In 49 (20.5%) cases, the cancer was judged by the pathologist to develop from endometriosis. These cancers, compared with those without evidence of tumor developing from endometriosis, more often manifested papillary projections on ultrasound (46.9% (23/49) vs 24.7% (47/190)), were less often bilateral (8.2% (4/49) vs 24.7% (47/190)) and less often associated with ascites (6.1% (3/49) vs 28.4% (54/190)) and fluid in the pouch of Douglas (24.5% (12/49) vs 48.9% (93/190)). Retrospective analysis of available ultrasound images using pattern recognition revealed that many tumors without evidence of tumor developing from endometriosis (36.3% (41/113)) had a large central solid component entrapped within locules, giving the tumor a cockade-like appearance.Conclusions: Endometrioid cancers are usually large, unilateral, multilocular-solid or solid tumors. The ultrasound characteristics of endometrioid carcinomas developing from endometriosis differ from those without evidence of tumor developing from endometriosis, the former being more often unilateral cysts with papillary projections and no ascites. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd. [ABSTRACT FROM AUTHOR]

Objective. Adult granulosa cell tumors (AGCTs) represent 2%–5% of all ovarian malignancies. The aim of this study was to analyze clinical and pathohistological parameters and their impact on recurrence, overall, and disease-free survival in FIGO stage I AGCT patients. Methods. The tumor specimens analyzed in this retrospective study were obtained from a total of 36 patients with diagnosis of ovarian AGCT surgically treated at the Department of Gynecology, Rijeka University Hospital Centre, between 1994 and 2012. Clinical, pathological, and follow-up data were collected. Results. The mean age at diagnosis was 54.5 years with a range of 24–84. The majority of the patients, 30 (83%), were in FIGO stage IA, 3 (8%) in stage IC1, 1 (3%) in stage IC2, and 2 (6%) in stage IC3. During follow-up period (median 117.5 months, range 26–276), recurrence occurred in 4 patients (12%) with 2 deaths of the disease recorded. In univariate analysis, the 5-year survival rates were significantly shorter in patients with FIGO substage IC (p=0.019), with positive LVSI (p=0.022), with presence of necrosis (p=0.040), and with hemorrhage (p=0.017). In univariate analysis, the 5-year disease-free survival rates were significantly shorter in patients treated with fertility surgery (p=0.004), with diffuse growth pattern (p=0.012), with moderate and severe nuclear atypia (p=0.032), and with presence of hemorrhage (p=0.022). FIGO substage IC proved to be independent predictor for recurrence (OR = 16.87, p=0.015, and OR = 23.49, p=0.023, resp.) and disease-free survival (p=0.0002; HR 20.84, p=0.02) at the uni- and multivariate analyses. Conclusions. FIGO substage IC is predictive of recurrence and disease-free survival in patients with early-stage AGCTs. LVSI, presence of necrosis and hemorrhage, diffuse growth pattern, and nuclear atypia in AGCTs seem to be associated with overall and disease-free survival, so these pathological features should be taken into consideration when managing patients with AGCT. [ABSTRACT FROM AUTHOR]

Adult-type granulosa cell tumors of the ovary (aGCTs) are rare gynecologic malignancies that exhibit a high frequency of somatic FOXL2 c.C402G (p.Cys134Trp) mutation. Treatment of relapsed aGCT remains a significant clinical challenge. Here we show, using whole-exome and cancer gene panel sequencing of 79 aGCTs from two independent cohorts, that truncating mutation of the histone lysine methyltransferase gene KMT2D (also known as MLL2) is a recurrent somatic event in aGCT. Mono-allelic KMT2D-truncating mutations are more frequent in recurrent (10/44, 23%) compared with primary (1/35, 3%) aGCTs (p = 0.02, two-sided Fisher’s exact test). IHC detects additional non-KMT2D-mutated aGCTs with loss of nuclear KMT2D expression, suggesting that non-genetic KMT2D inactivation may occur in this tumor type. These findings identify KMT2D inactivation as a novel driver event in aGCTs and suggest that mutation of this gene may increase the risk of disease recurrence. [ABSTRACT FROM AUTHOR]

Abstract: Adjuvant chemotherapy is recommended for patients with resected high‐risk adult granulosa cell tumors (GCT), although strong data to support this are lacking. The objective of this study was to assess the outcomes of GCT patients, with the specific focus on patients that received adjuvant chemotherapy with curative intent (stage I‐III), reported in a large national cancer registry. Data from the Surveillance, Epidemiology, and End Results (SEER) database between 2000 and 2013 were used for analysis. Patient and disease characteristics were extracted and analyzed for association with administration of chemotherapy. Impact on disease‐specific survival (DSS) was analyzed using log‐rank test. A total of 739 patients with surgically treated adult GCT were identified. Median age was 51 years. 570 (77%) patients were stage I, 87 (12%) were stage II, and 82 (11%) were stage III. Adjuvant chemotherapy was administered to 176 (24%) patients. Young age, higher stage, and hysterectomy were associated with chemotherapy administration. Higher disease stage was associated with decreased five‐year DSS (IA/B 98.5%, IC 95.1%, II 86.1%, III 83.5%, P < 0.01). Notably, administration of adjuvant chemotherapy was not associated with improved five‐year DSS (P = 0.45) regardless of disease stage (stage IA/B: 96% with chemotherapy vs. 99% without chemotherapy; P = 0.64), (stage IC: 97% with chemotherapy vs. 94% without chemotherapy; P = 0.49), (stage II: 89% with chemotherapy vs. 83% without chemotherapy; P = 0.56), (stage III: 73% with chemotherapy vs. 93% without chemotherapy; P = 0.18). In this analysis, chemotherapy was not found to be associated with improved DSS of patients with operable disease regardless of stage, questioning the role for adjuvant chemotherapy in GCT. [ABSTRACT FROM AUTHOR]

Study Question: Is transplantation of cryopreserved ovarian tissue from patients with borderline ovarian tumors (BOTs) a safe procedure?Summary Answer: BOT cells were found in frozen-thawed and xenografted ovarian tissue in 1 of 11 BOT patients.What Is Known Already: The risk of reintroducing malignant cells upon ovarian tissue transplantation has been subject of debate for many years. Reimplantation of cryopreserved ovarian tissue from leukemia patients is unsafe, while results from studies of cryopreserved ovarian tissue from other forms of cancer, such as Hodgkin's lymphoma, are reassuring.Study Design, Size, Duration: Prospective experimental study conducted in an academic research unit using ovarian tissue from 11 patients undergoing cryopreservation for BOTs.Participants/materials, Setting, Methods: Histology, immunohistochemistry (IHC) for mucin 1 (MUC1) and cytokeratin 7 (CK7) and molecular analysis by reverse transcription quantitative polymerase chain reaction (RT-qPCR) for CK7 and MUC1 were performed on frozen-thawed ovarian tissue from 11 patients. Long-term (5 months) xenografting of ovarian tissue in immunodeficient mice was performed. The xenografts were analyzed by histology, IHC and RT-qPCR, furthermore IHC for CD10, a marker of endometriosis, was performed on a selected sample.Main Results and the Role Of Chance: Analysis by histology, IHC and RT-qPCR indicated 10 of the ovarian tissue samples were negative. Analysis of the xenograft samples indicated nine were negative for malignant cells but in two xenografts glandular lesions were detected by histology. In these two xenografts, CK7 and MUC1 markers were demonstrated by IHC and CK7 expression also by RT-qPCR. A BOT was confirmed in the xenograft in which the original ovarian tissue was positive, while in the other case IHC demonstrated expression of endometriosis marker CD10.Limitations, Reasons For Caution: Cryopreserved ovarian fragments cannot be tested before transplantation, therefore the preimplantation analysis cannot guarantee that all cryopreserved fragments will be free of BOT cells.Wider Implications Of the Findings: BOT cells can be found in cryopreserved ovarian tissue from BOT patients, therefore preimplantation analysis is an absolute prerequisite. Endometriosis can also be detected in cryopreserved ovarian tissue and caution should also be exercised in this scenario.Study Funding/competing Interest(s): This study was supported by grants from the Fonds National de la Recherche Scientifique de Belgique (FNRS-PDR Convention T.0077.14, Télévie Grant 7.4590.16 awarded to Rossella Masciangelo, and Grant 5/4/150/5 awarded to Marie-Madeleine Dolmans), the Fonds Speciaux de Recherche, and the Foundation Against Cancer. None of the authors have any conflicting interests to declare. [ABSTRACT FROM AUTHOR]

Copyright of Turkish Journal of Obstetrics & Gynecology is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Sertoli-Leydig cell tumors (SLCTs) are an uncommon subtype of sex-cord stromal tumors of the ovary, which most commonly arise in women of reproductive age, creating an issue with regard to the preservation of fertility. The clinical manifestation of SLCTs varies widely, ranging from an asymptomatic clinical profile to extreme virilization. Correct diagnosis of SLCT is crucial and is primarily based on histopathological results. The current study presents the case of a 20-year-old woman who underwent unilateral salpingo-oophorectomy and adjuvant chemotherapy due to the diagnosis of an SLCT of the left ovary. Almost 2 years after the initial surgery, during the follow-up period, the patient conceived normally. Pregnancy was uneventful and the patient vaginally delivered a healthy infant at 38 weeks of gestation. A total of 1 year after delivery (3 years after the initial diagnosis), follow-up of the patient did not reveal any disease recurrence. In conclusion, SLCTs may be adequately treated by fertility-sparing surgery and chemotherapy in young women who wish to preserve their fertility. Natural conception, an uncomplicated pregnancy and a vaginal delivery are possible. [ABSTRACT FROM AUTHOR]

Cancer is one of the leading causes of mortality worldwide. Usually, the diagnosis of cancer at an early stage is important to facilitate proper treatment and survival. Nuclear medicine has been successfully used in the diagnosis, staging, therapy and monitoring of cancers. Single-photon emission computed tomography and PET-based companion imaging agents are in development for use as a companion diagnostic tool for patients with ovarian cancer. The present review discusses the basic and clinical studies related to the use of radiopharmaceuticals in the diagnosis and management of ovarian cancer, focusing on their utility and comparing them with other imaging techniques such as computed tomography and MRI. [ABSTRACT FROM AUTHOR]

Objective: We evaluated the efficacy of 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) in recurrent disease, response to therapy, and long-term follow-up of ovarian cancer (OC) patients in relation to cancer antigen-125 (CA125) levels and the prognostic meaning of this modality in this subset of subjects. Material and Methods: Between 2005 and 2015, we retrospectively evaluated 125 patients affected by OC who underwent FDG PET/CT imaging at our institution. The indications for PET/CT were recurrence of disease in 78 patients, therapy response assessment in 29, and follow-up in 18. The results of FDG PET/CT were compared with those of histopathology and clinical and radiological progression during follow-up for at least 6 months. The median long-term follow-up was 33 months. The diagnostic accuracies for the different clinical settings were evaluated. The relationships among global survival (GS), FDG PET/CT results, and CA125 levels were evaluated by both Kaplan-Meier and Cox regression analysis. Results: CA125 results were positive (>35 UI/mL) in 62 patients and negative in 63 (49% vs. 51%). The sensitivity and specificity of CA125 were 72% and 91%, respectively. PET/CT imaging showed a sensitivity of 98.6% and a specificity of 77.8% for the assessment of recurrent disease, and a sensitivity of 72.7% and a specificity of 88.9% for therapy evaluation. Meanwhile, in 18 patients evaluated during follow-up, the specificity was 82.3%. GS was significantly higher in case of negative CA125 values at the time of FDG PET/CT, of a negative PET/CT scan and when no evidence of peritoneum recurrence and distant metastases was determined by PET. Multivariate regression analysis showed that only age and peritoneum recurrence as determined by PET were identified as independent predictors of poor prognosis. Conclusion: Metabolic imaging with FDG PET/CT proved useful in patients where OC recurrence was suspected, even when the value of tumor marker CA125 was in a normal range. A positive PET/CT scan and the presence of peritoneum recurrence at PET were associated with a poor prognosis after approximately 30 months. [ABSTRACT FROM AUTHOR]

Background: Owing to the rarity of sex cord tumor with annular tubules (SCTAT), it is difficult to recognize SCTAT clinically and there is no standard treatment. The aim of our study was to investigate the treatment outcomes and prognosis of patients with ovarian SCTAT. Methods: A cohort of 13 patients with SCTAT diagnosed and treated in Peking Union Medical College Hospital was studied. Data on clinicopathological characteristics, treatment, and prognosis were retrospectively reviewed and analyzed. Results: SCTAT accounted for 1.4% of ovarian sex cord stromal tumors, with an average onset age of 22.6 years. All patients presented with menstrual disturbances or isosexual precocity at disease onset. Initial surgery was unilateral salpingo-oophorectomy in 11 cases. Recurrence rate was 46.2%, and 38.5% of patients experienced multiple recurrences. The disease free interval gradually shortened with increasing numbers of recurrences. Recurrent tumors were mostly ipsilateral to the primary tumor and located in retroperitoneum. Surgery remained the main treatment for recurrent cases. Serum estradiol and progesterone levels usually elevated at disease onset, decreased dramatically after operation, and they elevated again with the development of recurrence. The median progression-free survival (PFS) was 97.8 months, and the 1-year and 5-year PFS were 92% and 67%, respectively. Five-year overall survival (OS) was 100%. Conclusions: Unilateral salpingo-oophorectomy is a feasible treatment for primary SCTAT cases with intact capsules and without PJS. Complete tumor resection is suggested for recurrent cases and long-term follow-up is strongly recommended. Despite the high risk of recurrence, SCTAT prognosis is relatively favorable. [ABSTRACT FROM AUTHOR]

BACKGROUND Clinical data suggest that the presence of an ovarian endometrioma may cause per se damage to the surrounding otherwise healthy ovarian tissue. However, the basic research has so far done a limited job in trying to understand the potential detrimental effect of an endometrioma presence in the context of the ovarian physiology. We have reviewed the literature with the aim of characterizing the pathophysiology of the endometrioma focusing mostly on factors and mechanisms potentially affecting the surrounding, otherwise normal, ovarian tissue. METHODS Comprehensive searches of PUBMED were conducted to identify human studies published from 1991 to 2013 in the English language on the cellular and molecular characterization of the various endometrioma components. RESULTS An endometrioma contains free iron, reactive oxygen species (ROS), proteolytic enzymes and inflammatory molecules in concentrations from tens to hundreds of times higher than those present in peripheral blood or in other types of benign cysts. The cyst fluid causes substantial changes in the endometriotic cells that it baths from gene expression modifications to genetic mutations The physical barrier between the cyst contents and the normal ovarian tissue is a thin wall composed of the ovarian cortex itself or fibroreactive tissue. ROS potentially permeating the surrounding tissues and proteolytic substances degrading the adjacent areas are likely to cause the substitution of normal ovarian cortical tissue with fibrous tissue in which the cortex-specific stroma is reduced. The fibrosis is associated with smooth muscle metaplasia and followed by follicular loss and intraovarian vascular injury. Follicular density in tissue surrounding the endometriotic cyst was consistently shown to be significantly lower than in healthy ovaries but this pathological change does not appear to be caused by the stretching of surrounding tissues owing to the presence of a cyst. CONCLUSIONS There is sufficient molecular, histological and morphological evidence, in part deriving from knowledge of the pathophysiology, to support a deleterious effect of the endometrioma on the adjacent ovarian cortical tissue, independent of the mere mechanical stretching owing to its size. [ABSTRACT FROM AUTHOR]

Copyright of Ginecología y Obstetricia de México is the property of Federacion Mexicana de Ginecologia y Obstetricia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

BACKGROUND The aim of this systematic review was to evaluate the fertility outcome after borderline ovarian tumor (BOT) management and the results of conservative management, risk of recurrence and alternative options. METHODS The search strategy was based on relevant terms concerning BOT using Medline and the Central Cochrane Library. Both early and advanced stages of serous and mucinous BOT were included, but not rare entities such as endometrioid, Brenner or clear-cell BOT because of their low incidence. We considered all articles—case reports, original studies, meta-analyses and reviews—in English and French. RESULTS Overall, 230 articles were screened of which 120 were retained for review. Most pregnancies were spontaneous but some data were obtained from studies analyzing the contribution of assisted reproductive technology (ART). However, not all studies differentiated spontaneous pregnancy from those obtained after fertility treatment including ovulation induction, intrauterine insemination and IVF. Conservative management of early stage BOT resulted in a pooled estimate for spontaneous pregnancy rate of 54% with a low risk of lethal recurrence (pooled estimate: 0.5%). In patients with advanced stage BOT, the spontaneous pregnancy rates was lower (34% in the single series reporting pregnancy rate in this context) and the risk of lethal recurrence increased (pooled estimate: 2%). CONCLUSIONS This systematic review underlines that fertility subsequent to treatment of BOT depends mainly on histology and initial staging to distinguish early from advanced stages. In patients with advanced stage BOT, several alternative options to conservative management are available to allow patients to conceive without compromising their prognosis. [ABSTRACT FROM AUTHOR]

Sertoli-Leydig cell tumor of the ovary, also known as androblastoma, is a rare neoplasm from the group of sex cord-stromal tumors of the ovary. The tumor accounts for <0.5% of all primary ovarian neoplasms. The clinical signs and symptoms of Sertoli-Leydig cell tumors can be associated with either hormonal production or the presence of a mass-occupying lesion. In the current study, a 13-year-old female was diagnosed with a stage ?c ovarian Sertoli-Leydig cell tumor following abdominal pain and distension. One month after a right oophorectomy, the follow-up magnetic resonance imaging scan was negative for residual or recurrent tumor. The overall 5-year survival rate for moderately-differentiated (grade 2) and poorly-differentiated (grade 3) Sertoli-Leydig cell tumors is 80%, and long-term follow-up is therefore highly advised in this patient. [ABSTRACT FROM AUTHOR]