The development of guidelines is one of the core activities of the European Society for Medical Oncology (ESMO) and European Society of Gynaecologial Oncology (ESGO), as part of the mission of both societies to improve the quality of care for patients with cancer across Europe. ESMO and ESGO jointly developed clinically relevant and evidence-based recommendations in several selected areas in order to improve the quality of care for women with ovarian cancer. The ESMO-ESGO consensus conference on ovarian cancer was held on April 12-14, 2018 in Milan, Italy, and comprised a multidisciplinary panel of 40 leading experts in the management of ovarian cancer. Before the conference, the expert panel worked on five clinically relevant questions regarding ovarian cancer relating to each of the following four areas: pathology and molecular biology, early-stage and borderline tumours, advanced stage disease and recurrent disease. Relevant scientific literature, as identified using a systematic search, was reviewed in advance. During the consensus conference, the panel developed recommendations for each specific question and a consensus was reached. The recommendations presented here are thus based on the best available evidence and expert agreement. This article presents the recommendations of this ESMO-ESGO consensus conference, together with a summary of evidence supporting each recommendation.

AIM:We sought to determine the epidemiology of mucinous ovarian tumors, the correlation between serum biomarkers and tumor status, and the outcomes of the management in different subtypes of mucinous ovarian tumors in a Chinese surgical cohort. METHODS:A total of 513 patients were enrolled from January 2009 to May 2017. The number of patients who had benign mucinous ovarian tumor, borderline mucinous ovarian tumor, or malignant mucinous ovarian tumor was pathologically quantified. All patients underwent surgery with or without postoperative adjuvant therapy. Prognosis was analyzed based on clinicopathological characteristics and the type of treatment received. Hyperthermic intraperitoneal chemotherapy efficacy and adverse effects in patients were also explored. RESULTS:In all, 383 (75%) patients were diagnosed as having benign mucinous ovarian tumor, 76 (14%) patients with borderline mucinous ovarian tumor, and 54 (5%) patients with malignant mucinous ovarian tumor. Levels of serum biomarkers increased as the tumors became more malignant. Patients with stage IA and IC (unilateral) malignant mucinous ovarian tumor who underwent fertility conserving surgery did not experience poorer prognoses when compared to those who received non-fertility conserving surgery. Hyperthermic intraperitoneal chemotherapy followed by chemotherapy significantly influenced survival rates in patients with a ruptured malignant mucinous ovarian tumor. CONCLUSIONS:Levels of serum tumor markers, carbohydrate antigen 125, carbohydrate antigen 199, carbohydrate antigen 242, and carcinoembryonic antigen may be useful in monitoring for malignant transformation. Fertility conserving surgery might be a preferable surgical procedure for patients with malignant mucinous ovarian tumor at early stage (IA and IC [unilateral]). Hyperthermic intraperitoneal chemotherapy appears to be a well-tolerated and promising postoperative adjuvant.

Ovarian mucinous tumors are subclassified in multiple categories. Recent studies have highlighted issues in interobserver reproducibility. This review will focus on some new developments including criteria and ancillary tests that may help to improve interobserver reproducibility at clinically important thresholds. These issues include proposals for a separate terminology of teratoma-associated ovarian mucinous neoplasms, the role of TP53 immunohistochemistry in distinction of crowded mucinous borderline tumors and expansile mucinous carcinomas as well as the assignment of the infiltrative pattern of invasion, which recently has been validated as important prognostic factor even in low stage mucinous ovarian carcinoma.

Globally, ovarian cancer is the eighth most common cancer in women, accounting for an estimated 3.7% of cases and 4.7% of cancer deaths in 2020. Until the early 2000s, age-standardized incidence was highest in northern Europe and North America, but this trend has changed; incidence is now declining in these regions and increasing in parts of eastern Europe and Asia. Ovarian cancer is a very heterogeneous disease and, even among the most common type, namely epithelial ovarian cancer, five major clinically and genetically distinct histotypes exist. Most high-grade serous ovarian carcinomas are now recognized to originate in the fimbrial ends of the fallopian tube. This knowledge has led to more cancers being coded as fallopian tube in origin, which probably explains some of the apparent declines in ovarian cancer incidence, particularly in high-income countries; however, it also suggests that opportunistic salpingectomy offers an important opportunity for prevention. The five histotypes share several reproductive and hormonal risk factors, although differences also exist. In this Review, we summarize the epidemiology of this complex disease, comparing the different histotypes, and consider the potential for prevention. We also discuss how changes in the prevalence of risk and protective factors might have contributed to the observed changes in incidence and what this might mean for incidence in the future.

Ovarian mucinous tumors range from benign cystadenomas to borderline tumors to frankly malignant adenocarcinomas, and may display either intestinal-type morphology or, less frequently, endocervical-type differentiation. The latter category has been the subject of recent controversy owing to its morphologic overlap with so-called "seromucinous" ovarian tumors, a group that shares more molecular features with endometrioid tumors than it does with either serous or mucinous ovarian neoplasias. Endocervical-type differentiation in ovarian mucinous tumors may also represent an endocervical metastasis. Distinction of primary ovarian mucinous tumors from gastrointestinal metastases can be difficult, as the morphology of intestinal-type ovarian mucinous primaries sometimes differs only subtly if at all from gastrointestinal metastases.

STUDY OBJECTIVE:To study the progression of benign ovarian lesions to mucinous borderline ovarian tumors (mBOTs); analyze the clinicopathologic features, diagnosis, and management of mBOTs in pediatric and adolescent girls; and provide a review of the literature on mBOTs in this population. DESIGN:Retrospective chart review of female adolescents younger than 18 years diagnosed with mBOTs between July 2017 and February 2021. SETTING:Yale New Haven Hospital, New Haven, Connecticut; and Yale New Haven Health Bridgeport Hospital, Bridgeport, Connecticut. PARTICIPANTS:Three female patients diagnosed with mBOTs between ages 12 and 17 years. INTERVENTIONS:None. MAIN OUTCOME MEASURES:Clinical presentation, preoperative characteristics, surgical technique, histology, tumor stage, treatment, progression, outcome, and rate of recurrence. RESULTS:Three adolescent patients were identified to have mBOTs. All three patients presented with a chief complaint of abdominal pain. One of the 3 patients was premenarchal at presentation. Two of the 3 patients were initially diagnosed with a mucinous cystadenoma and had recurrences of an ovarian cyst in the same ovary within 5 and 17 months, respectively. Pathology of the recurrent cyst was consistent with mBOT. Two of the 3 patients initially underwent cystectomy, and all ultimately had a unilateral salpingo-oophorectomy. Subsequent surveillance over 2 to 4 years found no evidence of disease recurrence. CONCLUSION:mBOTs are rare in the pediatric and adolescent population and could arise from benign ovarian tumors.

Mucinous neoplasms of the ovary account for 10%-15% of ovarian neoplasms. They may be benign, borderline, or malignant. The large majority are benign or borderline, accounting for 80% and 16%-17%, respectively. Mucinous neoplasms of the ovary most commonly affect women in their 20s to 40s. The clinical manifestation is nonspecific, but most mucinous ovarian neoplasms manifest as large unilateral pelvic masses. At gross pathologic analysis, mucinous ovarian neoplasms appear as large multiloculated cystic masses. The contents of the cyst loculi vary on the basis of differences in internal mucin content. At histologic analysis, mucinous ovarian neoplasms are composed of multiple cysts lined by mucinous epithelium, often resembling gastrointestinal-type epithelium. Imaging evaluation most commonly includes US and/or MRI. The imaging findings parallel the gross pathologic features and include a large, unilateral, multiloculated cystic mass. The cyst loculi vary in echogenicity, attenuation, and signal intensity depending on the mucin content. Mucinous neoplasms of the ovary are staged surgically using the FIGO (International Federation of Gynecology and Obstetrics) staging system. Primary treatment is surgical, with adjuvant chemotherapy considered in the uncommon case of mucinous carcinoma with extraovarian disease. Since most mucinous ovarian neoplasms are benign or borderline, the overall prognosis is excellent.