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Background: Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is increasingly being diagnosed incidentally on imaging. It has malignant potential, making it vital to establish the correct diagnosis, assess its malignant risk and follow a management strategy to prevent development of invasive carcinoma of the pancreas., Objective: This review focuses on the epidemiology, natural history, risk factors, diagnosis and management of IPMN of the pancreas, and will provide practical points for general practitioners., Discussion: IPMN of the pancreas can transform into invasive pancreatic carcinoma at a low rate of approximately 2%/year. Upon diagnosis of IPMN, it is risk stratified based on the presence of worrisome or high-risk stigmata, which guides further management. Management needs to be individualised based on IPMN and patient factors due to limitations with the current diagnostic tools.

Background: Low-grade appendiceal mucinous neoplasms (LAMNs) can progress to pseudomyxoma peritonei (PMP). The incidence and risk factors for recurrence are unclear, and there is a lack of consensus on the need and duration of surveillance imaging., Methods: Patients at the Mayo Clinic in Rochester, Minnesota, with a histologic diagnosis of LAMNs confined to the appendix and limited involvement of the right lower quadrant from 1992 to 2023 were included. Associations between recurrence and risk factors were assessed using Kaplan-Meier curves and Cox proportional hazards regression., Results: A total of 125 patients with LAMNs underwent abdominal imaging surveillance for a median of 51.2 months (IQR, 26-92). Of note, 5 patients (4%) recurred, all of which were PMP. Overall, the 5- and 10-year cumulative recurrence incidence rates were 3% and 6%, respectively. The median time to recurrence was 24 months (IQR, 23-87). Only LAMNs limited to the right lower quadrant and LAMN tumor size of <2 cm were associated with recurrence (P < .05). The 5- and 10-year cumulative recurrence risks were 12% and 30%, respectively, for the 21 patients with either risk factor. Only 1% of patients without these 2 risk factors developed a recurrence at 10 years., Conclusion: The overall recurrence risk of LAMN after resection is low. Appendectomy is sufficient for LAMN. Select patients with acellular mucin confined to the right lower quadrant and a tumor size of <2 cm are at higher risk of recurrence. Thus, long-term surveillance is recommended for high-risk individuals. Conversely, LAMNs without risk factors can be safely observed expectantly., Competing Interests: Declaration of competing interest The authors declare no competing interests., (Copyright © 2024 Society for Surgery of the Alimentary Tract. Published by Elsevier Inc. All rights reserved.)

Background: The natural history of branch-duct intraductal papillary mucinous cystic neoplasms (BD-IPMNs) in the pancreas remains unclear. This study aimed to answer this clinical question by focusing on the development of concomitant pancreatic ductal adenocarcinomas (cPDAC)., Methods: The Japan Pancreas Society conducted a prospective multicenter surveillance study of BD-IPMN every six months for five years. The primary endpoints were progression of BD-IPMN, progression to high-grade dysplasia/invasive carcinoma (HGD/IC), and cPDAC. Factors predicting the progression of BD-IPMN to HGD/IC and development of cPDAC were also assessed as secondary endpoints., Results: Among the 2104 non-operated patients, 348 (16.5 %) showed progression of primary BD-IPMN. Cumulative incidences of BD-IPMN with HGD/IC and cPDAC during the 5.17-year surveillance period were 1.90 % and 2.11 %, respectively, and standard incidence ratios of BD-IPMN with HGD/IC and cPDAC were 5.28 and 5.73, respectively. Of 38 cPDACs diagnosed during surveillance, 25 (65.8 %) were resectable. The significant predictive characteristics of BD-IPMN for progression to HGD/IC were larger cyst size (p = 0.03), larger main pancreatic duct size (p < 0.01), and mural nodules (p = 0.02). Significant predictive characteristics for the development of cPDAC were male sex (p = 0.03) and older age (p = 0.02), while the size of IPMN was not significant., Conclusion: Careful attention should be given to "dual carcinogenesis" during BD-IPMN surveillance, indicating the progression of BD-IPMN to HGD/IC and development of cPDAC distinct from BD-IPMN, although the establishment of risk factors that predict cPDAC development remains a challenge (UMIN000007349)., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Introduction: Epithelial ovarian cancer (EOC) is the most prevalent type of ovarian cancer, yet the impact of ovarian laterality has received limited attention., Materials and Methods: We conducted a comprehensive investigation into the impact of laterality (left-right and bilateral-unilateral) on EOC incidence and prognosis, focusing on distinct subtypes. Binomial tests and Pearson's χ2 tests were employed to compare occurrence rates among laterality groups. Cox regression analyses were used to create a proportional hazards model for tumor prognosis. Nomograms were developed and validated, including internal validation via bootstrapping., Results: Our study encompassed 20,790 EOC patients, revealing disparities in incidence and prognosis between unilateral and bilateral cases. Unilateral tumor development was notably predominant in clear cell, endometrioid, brenner, and mucinous subtypes, while bilateral involvement was more frequent in serous ovarian cancer. Laterality differences, reflecting disparities between the left and right sides, were chiefly evident in the incidence rates across various stages and in the prognosis of specific subtypes. Notably, mucinous ovarian cancer exhibited significantly better prognosis on the right side compared to the left (right tumors: HR = 0.745, p = 0.015, CI: 0.587-0.945)., Conclusion: These findings emphasize the importance of considering ovarian laterality -both left-right and bilateral-unilateral aspects -as a critical factor influencing EOC incidence and prognosis, necessitating attention in clinical practice., Competing Interests: Declaration of competing interest The authors declared no conflict of interest., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background: The appendix is a small organ with no particular known function. Primary appendiceal neoplasms (ANs) are rare. While the prevalence is increasing worldwide over the past two decades, no apparent increase in the prevalence of ANs has been reported in the Arabian Gulf States. Recently, a significant decline in the age at diagnosis of some types of ANs has been reported worldwide, with a female predominance., Objectives: Evaluate the prevalence and clinicopathological characteristics of ANs within our institution in Saudi Arabia and compare them to limited existing studies from different regions as well as the Arabian Gulf States., Design: Retrospective cohort., Setting: Tertiary care center in Riyadh., Patients and Methods: All patients who underwent appendectomy and had the appendix submitted for histopathological evaluation between May 2015 and June 2020 were included to allow for a follow-up of 5 years or more at the time of data collection., Main Outcome Measures: Demographics, clinical presentations, surgical interventions, histopathological findings, complications, and recurrence rates., Sample Size: 25 AN patients., Results: Of 1110 patients, 25 had ANs (13 female and 12 male participants) with a mean (standard deviation) age of 54.6 (14.1) years. Only 40% presented with acute appendicitis, 64% had comorbidities, and less than 50% underwent laparoscopic appendectomy. Histopathologically, 72% were low-grade appendiceal mucinous neoplasms (LAMNs). Complications were minimal grades (Clavien-Dindo classification), with 80% experiencing none. The mean hospital stay was 9.96 days. Local recurrence occurred in 8% of cases, and distant metastasis was documented in one adenocarcinoma case. However, the 5-year overall and disease-free survival rates were 88% and 80%, respectively., Conclusions: The incidence of ANs is increasing in Saudi Arabia with the higher prevalence of LAMNs. The pathological examination of the resected appendix played a pivotal role in the diagnosis of ANs., Limitations: Data collected retrospectively, a single institution, and a small population.

Purpose: Ovarian cancer can be categorized into distinct histologic subtypes with varying identifiable risk factors, molecular composition, clinical features, and treatment. The global incidence of ovarian cancer subtypes remains limited, especially in low- and middle-income countries (LMICs) without high-quality cancer registry systems., Materials and Methods: We used data from population-based cancer registries of the Cancer Incidence in Five Continents project to calculate the proportions of serous, mucinous, endometrioid, clear cell, and other histologic subtypes of ovarian cancer. Proportions were applied to the estimated numbers of patients with ovarian cancer from Global Cancer Observatory 2020. Age-standardized incidence rates were calculated., Results: Globally, an estimated 133,818 new patients of serous cancer, 35,712 new patients of mucinous cancer, 29,319 new patients of endometrioid cancer, and 17,894 new patients of clear cell cancer were identified in 2020. The distribution of ovarian cancer histologic subtypes exhibited regional variation. Eastern Europe had the highest rate of serous and mucinous carcinomas, whereas Northern Africa and Eastern Asia had the highest burden of endometrioid and clear cell carcinomas, respectively., Conclusion: This study provides a global incidence landscape of histologic subtypes of ovarian cancer, particularly in LMICs lacking comprehensive registry systems. Our analysis offers valuable insights into disease burden and guidance for tailored strategies for prevention of ovarian cancer.

Background and Aim: Colitis-associated intestinal cancer (CAC) can develop in patients with inflammatory bowel disease; however, the malignant grade of CAC may differ from that of sporadic colorectal cancer (CRC). Therefore, we compared histological findings distinct from cancer stage between CAC and sporadic CRC to evaluate the features of CAC., Methods: We reviewed the clinical and histological data collected from a nationwide database in Japan between 1983 and 2020. Patient characteristics were compared to distinguish ulcerative colitis (UC), Crohn's disease (CD), and sporadic CRC. Comparisons were performed by using all collected data and propensity score-matched data., Results: A total of 1077 patients with UC-CAC, 297 with CD-CAC, and 136 927 with sporadic CRC were included. Although the prevalence of well or moderately differentiated adenocarcinoma (Tub1 and Tub2) decreased according to tumor progression for all diseases (P < 0.01), the prevalence of other histological findings, including signet ring cell carcinoma, mucinous carcinoma, poorly differentiated adenocarcinoma, or squamous cell carcinoma, was significantly higher in CAC than in sporadic CRC. Based on propensity score-matched data for 982 patients with UC and 268 with CD, the prevalence of histological findings other than Tub1 and Tub2 was also significantly higher in those with CAC. At pT4, mucinous carcinoma occurred at a significantly higher rate in patients with CD (45/86 [52.3%]) than in those with sporadic CRC (13/88 [14.8%]) (P < 0.01)., Conclusion: CAC, including early-stage CAC, has a higher malignant grade than sporadic CRC, and this difference increases in significance with tumor progression., (© 2024 The Authors. Journal of Gastroenterology and Hepatology published by Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.)

Objectives: Aging is associated with a high prevalence of pancreatic cysts and intraductal papillary mucinous neoplasms (IPMNs). Metabolic syndrome (MS) may increase the risk of neoplasms, including those that develop in the pancreas. However, the influence of factors associated with MS on the development of IPMN remains unclear., Methods: A total of 9363 patients who underwent abdominal ultrasound examinations between April 2012 and May 2013 were included in this study. Multivariate logistic regression analysis was performed to identify factors associated with the presence of IPMN by age., Results: Pancreatic cysts were detected in 198 of 9363 patients, of whom 129 were found to have IPMNs. The presence of IPMN significantly correlated with age (10-year increments; odds ratio, 2.73; 95% CI, 2.28-3.29; P < 0.001). High body mass index, history of smoking, hyperlipidemia, hypertension, and MS were associated with a higher prevalence of IPMN with advancing age. In multivariate analysis, the presence of IPMN was more frequent in elderly patients with MS (odds ratio, 3.14; 95% CI, 3.14-6.72; P = 0.003)., Conclusions: The present study suggests that the incidence of IPMN increases with age and is accelerated in the presence of MS., Competing Interests: Conflicts of Interest and Source of Funding: This work was supported by JSPS KAKENHI (Grants-in-Aid for Scientific Research), including grant numbers 19K08444 (A.M.) and 19H03698 (Y.K.). This work was also supported by funds from the Research Committee of Intractable Pancreatic Diseases provided by the Ministry of Health, Labor, and Welfare of Japan (A.M.). The authors have no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background and Aims: In high-risk individuals (HRIs), we aimed to assess the cumulative incidence of intraductal papillary mucinous neoplasms (IPMNs) and compare IPMN growth, neoplastic progression rate, and the value of growth as predictor for neoplastic progression to these in sporadic IPMNs., Methods: We performed annual surveillance of Dutch HRIs, involving carriers of germline pathogenic variants (PVs) and PV-negative familial pancreatic cancer kindreds. HRIs with IPMNs were compared with Italian individuals without familial risk under surveillance for sporadic IPMNs., Results: A total of 457 HRIs were followed for 48 (range 2-172) months; the estimated cumulative IPMN incidence was 46% (95% confidence interval, 28%-64%). In comparison with 442 control individuals, IPMNs in HRIs were more likely to grow ≥2.5 mm/y (31% vs 7%; P < .001) and develop worrisome features (32% vs 19%; P = .010). PV carriers with IPMNs more often displayed neoplastic progression (n = 3 [11%] vs n = 6 [1%]; P = .011), while familial pancreatic cancer kindreds did not (n = 0 [0%]; P = 1.000). The malignancy risk in a PV carrier with an IPMN was 23% for growth rates ≥2.5 mm/y (n = 13), 30% for ≥5 mm/y (n = 10), and 60% for ≥10 mm/y (n = 5)., Conclusions: The cumulative incidence of IPMNs in HRIs is higher than previously reported in the general population. Compared with sporadic IPMNs, they have an increased growth rate. PV carriers with IPMNs are suggested to be at a higher malignancy risk. Intensive follow-up should be considered for PV carriers with an IPMN growing ≥2.5 mm/y, and surgical resection for those growing ≥5 mm/y., (Copyright © 2024 AGA Institute. Published by Elsevier Inc. All rights reserved.)

Introduction: Anal mucinous adenocarcinoma (AMAC) is an extremely rare form of anal cancer. Our objective was to examine the incidence, management, and prognostic factors of AMAC., Methods: We analyzed age-adjusted incidence (AAI) rates over time and compared the prognosis of AMAC with anal squamous cell carcinoma (ASCC) and adenocarcinoma (AAC) using propensity score matching and Kaplan-Meier analysis. Patients were classified based on summary stage and treatments to determine cancer-specific survival., Results: AAI of AMAC fluctuated within a narrow range (0.082-0.237 per million person-years) from 2000 to 2018. AMAC had a slight non-significant trend of worse prognosis than ASCC (p = 0.348) and a better prognosis than AAC (p &lt; 0.01). Females made up a larger proportion of patients diagnosed with the distant disease (p &lt; 0.05) and unmarried (p &lt; 0.05) and somewhat less probably to need surgical removal (p &lt; 0.01) and radiotherapy (p &lt; 0.01). Elderly patients have lower rates of survival (p &lt; 0.05). Localized stage was associated with better prognosis (p &lt; 0.05). Surgery was associated with a tendency toward better survival (p = 0.095)., Conclusions: AMAC exhibits a low incidence yet favorable prognosis compared to typical AAC and slightly worse compared to ASCC. Elderly age is associated with poorer prognosis, while localized stage indicates better prognosis. Surgery demonstrates a trend toward improved survival., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

Background: The degree of dysplasia is the most important prognostic factor for patients with resected intraductal papillary mucinous neoplasms. Intraductal papillary mucinous neoplasms are predominantly premalignant conditions; in most cases, surveillance is an adequate treatment. If worrisome features are present, surgery should be considered. However, there is limited data on the long-term prognosis of resected intraductal papillary mucinous neoplasms. We aimed to ascertain the nationwide survival of patients with resected intraductal papillary mucinous neoplasms and identify factors associated with survival., Methods: This is a retrospective nationwide cohort study. All intraductal papillary mucinous neoplasms operated on in Finland between 2000 and 2008 were identified. Patient records were evaluated, and original radiologic data and histologic samples were re-evaluated. Survival data were collected after a 10-year follow-up period., Results: Out of 2,024 pancreatic resections, 88 were performed for intraductal papillary mucinous neoplasm. The median age of the patients was 65 years. Histologic diagnoses were main duct intraductal papillary mucinous neoplasm 47/88 (53,4%), mixed-type intraductal papillary mucinous neoplasm 27/88 (30.7%), and branchduct intraductal papillary mucinous neoplasm 14/88 (15.9%). Of the tumors, 40/88 (45.5%) were low-grade dysplasia, 9/88 (10.2%) high-grade, and 39/88 (44.3%) were invasive cancer. The median survival was 121 (range 0-252) months. Ten-year survival was 72.5%, 66.7%, and 23.1% in the low-grade dysplasia, high-grade dysplasia, invasive cancer groups, respectively. Ten-year mortality for pancreatic cancer was 5%, 9.1%, and 71.8% in the low-grade dysplasia, high-grade dysplasia, invasive cancer groups, respectively., Conclusion: Overall, 44.3% of the patients had a malignant tumor, and three-quarters (74.5%) of the main duct intraductal papillary mucinous neoplasms were malignant or high-grade dysplasia at the time of surgery. Ten-year survival was significantly better in patients operated on at the stage of a premalignant tumor (low-grade dysplasia + high-grade dysplasia) than in patients operated on at the stage of a malignant tumor., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: In this study, we aimed to put forth the factors that contribute to the recurrence of mucinous ovarian cancer., Materials and Methods: Forty-four mucinous ovarian cancer patients who have presented to our clinic between February 2006 and May 2018 took part in the study. In order to predict the factors that contribute to recurrence, the univariate and the multivariate logistic regressions were utilized. The Kaplan-Meier survival analysis was utilized for survival and the log-rank test was used for the discrepancies between the groups. In the analysis of the data, the Statistical Package for the Social Sciences 22 program was used. It was acknowledged to have statistical meaning when the P value in all the tests was lower than 0.05., Findings: Recurrence was detected in 20 out of 44 patients who participated in the study. The ages of the patients who did not experience recurrence were significantly lower ( P = 0.001). The patients were detected mostly in Stage 1 (36.4%). In the group of patients without recurrence, systemic lymphadenectomy (43.2%) was greater ( P = 0.019). Lymph node metastasis was three times higher in the group that experienced recurrence ( P = 0.047). When the two groups were compared, the platinum resistance was considerably greater in the group with recurrence ( P = 0.005). In terms of residual tumor, the rate of complete resection was (9%) better in the group that experienced recurrence compared to the group that did not experience recurrence, with a rate of 45.5%. While 12 patients who experienced recurrence died, 6 people died in the other group. From the factors that contribute to recurrence, in terms of residual tumor quantity, this was grouped as complete (R0) resection and optimal + suboptimal (R1 + R2) resection and the following were determined: odds ratio (OR) - 5.7 (95% confidence interval [CI]: 1.56-20.9) and P = 0.008 for R1 + R2. In univariate analysis, the OR was determined as 1.16 (95% CI: 1.06-1.27) for age. Possessing a Stage 2 and higher disease statistically contributed considerably to the recurrence compared to Stage 1 disease (OR: 6.33; 95% CI: 1.59-25.22; P = 0.009). Age was determined as an independent prognostic risk factor in the multivariate analysis (OR: 1.10 [95% CI: 1.04-1.25]), P = 0.018. Furthermore, the OR for the advanced-stage (Stage 2 or higher) patients in the multivariate analysis was determined as 7.88 (95% CI: 0.78-78.8) and was found to be statistically significant at limits ( P = 0.079)., Results: We have put forth that the genetic, biological, and clinical characteristics of mucinous ovarian cancers differ from that of other epithelial ovarian cancers, and that age, advanced stage, and residual tumor quantity are prognostic risk factors in terms of recurrence, and that age is an independent prognostic risk factor., Conclusion: Biological and clinical characteristics of mucinous ovarian cancers differ from those of other epithelial ovarian cancers, and we observed that the age, advanced stage, and the amount of residual tumor regarding recurrence are prognostic risk factors, while age was determined as an independent prognostic risk factor., Competing Interests: None

Modern series report a prevalence of pancreatic cysts in the general population of up to 50% in prospective studies. Of these, about half will be pancreatic cystic neoplasms (PCNs) that have varying degrees of malignant potential. Intraductal papillary mucinous neoplasms (IPMNs) of the pancreas are the most common PCNs and are known predecessors of pancreatic adenocarcinoma. Critically, they are one of the only radiographically identifiable precursors of pancreatic cancer and thus provide an opportunity for early cancer detection and surgical resection with curative intent. The combination of high prevalence and potential for malignant degeneration underscore the relevance of discussing the best management of IPMNs and improving the existing standard of care. Landmark data on IPMN prevalence, guidelines, surveillance, biomarkers, and immune landscape are highlighted., (© 2023. The Author(s).)

Introduction: Low-grade appendiceal mucinous neoplasms (LAMN) are semi-malignant tumors of the appendix which are incidentally found in up to 1% of appendectomy specimen. To this day, no valid descriptive analysis on LAMN is available for the German population., Methods: Data of LAMN (ICD-10: D37.3) were collected from the population-based cancer registries in Germany, provided by the German Center for Cancer Registry Data (Zentrum für Krebsregisterdaten-ZfKD). Data was anonymized and included gender, age at diagnosis, tumor staging according to the TNM-classification, state of residence, information on the performed therapy, and survival data., Results: A total of 612 cases were reported to the ZfKD between 2011 and 2018. A total of 63.07% were female and 36.93% were male. Great inhomogeneity in reporting cases was seen in the federal states of Germany including the fact that some federal states did not report any cases at all. Age distribution showed a mean age of 62.03 years (SD 16.15) at diagnosis. However, data on tumor stage was only available in 24.86% of cases (n = 152). A total of 49.34% of these patients presented with a T4-stage. Likewise, information regarding performed therapy was available in the minority of patients: 269 patients received surgery, 22 did not and for 312 cases no information was available. Twenty-four patients received chemotherapy, 188 did not, and for 400 cases, no information was available. Overall 5-year survival was estimated at 79.52%. Patients below the age of 55 years at time of diagnosis had a significantly higher 5-year survival rate compared to patients above the age of 55 years (85.77% vs. 73.27%)., Discussion: In this study, we observed an incidence of LAMN in 0.13% of all appendectomy specimen in 2018. It seems likely that not all cases were reported to the ZfKD; therefore, case numbers may be considered underestimated. Age and gender distribution goes in line with international studies with females being predominantly affected. Especially regarding tumor stage and therapy in depth information cannot be provided through the ZfKD-database. This data analysis emphasizes the need for further studies and the need for setting up a specialized registry for this unique tumor entity to develop guidelines for the appropriate treatment and follow-up., (© 2022. The Author(s).)

Objectives: Pancreatic calcifications (PC) are considered specific for chronic pancreatitis (CP), but PC may also be present in non-CP diseases. The aims are to understand the pattern of calcifications in different diseases and to determine they were related to malignant diseases., Methods: A retrospective study was performed including patients with PC or CP undergoing surgery in the Department of General Surgery of Ruijin Hospital from January 2003 to June 2018., Results: PC were observed in 168 (4.5%) of the 3755 patients with pancreatic lesions treated during the study period. The majority of patients with PC had three kinds of CP (73.2%) while 26.8% had other five kinds of non-CP diseases. In patients with non-CP diseases, the incidence of PC in malignant intraductal papillary mucinous neoplasm (IPMN) was significantly higher than benign IPMN (8.3% vs. 0.7%, p = 0.004). In patients of CP with pancreatic mass (n = 81), PC (Odds ratio = 28.6, p = 0.000), advanced age (> 55 years) and parenchymal atrophy were independent predictors for malignancy. In patients of CP without pancreatic mass (n = 110), there were 82 cases (74.5%) with PC and 5 cases (4.5%) with pancreatic ductal adenocarcinoma. The regression model of risk factors was not successful., Conclusions: The disease spectrum with PC was very diverse. PC may be related to malignant IPMN in non-CP diseases and is related to malignancy in the patients of CP with pancreatic mass and indications for resection., (© 2022. The Author(s).)

Background: It is unclear whether patients with intraductal papillary mucinous neoplasia harbor a higher risk of developing extrapancreatic malignancies., Aims: We performed a pooled estimate of the incidence of extrapancreatic malignancies in patients with intraductal papillary mucinous neoplasia, with a particular focus on the comparison to the general population., Methods: Computerized bibliographic search of main databases was performed through February 2021. The primary endpoint was the pooled incidence of extrapancreatic malignancies in patients with intraductal papillary mucinous neoplasms. Additional outcome was the comparison between intraductal papillary mucinous neoplasia patients and the general population, expressed in terms of standardized incidence ratio along with 95% confidence intervals., Results: Eighteen studies with 8709 patients were included. The pooled rate of metachronous extrapancreatic malignancies was 10 (6-13)/1000 persons-year. No difference was observed according to intraductal papillary mucinous neoplasia histology and sex, whereas a significantly superior incidence of extrapancreatic malignancies was observed in patients with main-duct (36.7%, 25.4%-48%) as compared to branch-duct intraductal papillary mucinous neoplasia (26.2%, 17.6%-34.8%; p = 0.03). Pooled standardized incidence ratio comparing expected rates in the general population was 1.01 (0.79-1.29); no difference was observed concerning rates of metachronous gastric cancer (standardized incidence ratio 1.60, 0.72-3.54) and colorectal cancer (1.29, 0.92-1.18), whereas biliary cancer was observed more frequently in intraductal papillary mucinous neoplasia patients (2.29, 1.07-4.93)., Conclusion: Patients with intraductal papillary mucinous neoplasia harbor an overall rate of extrapancreatic malignancies as high as 27.3%. The rate of metachronous extrapancreatic malignancies is not superior to the general population., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Objective: The present study aimed to identify epidemiological factors associated with the development of intraductal papillary mucinous neoplasms (IPMN) of the pancreas comparing patients after IPMN resection with population-based controls., Methods: Preoperative data of 811 patients undergoing pancreatic resection for IPMN were matched in a 1:1 ratio with a random sample of volunteers from the Study of Health in Pomerania, which showed no pancreatic cyst greater than 2 mm in magnetic resonance cholangiopancreaticography., Results: A total of 811 controls with a mean age of 61.9 years (standard deviation, 8.4 years) were matched to cases with a mean age of 66.1 years (standard deviation, 9.3 years). A previous history of pancreatitis, endocrine pancreatic insufficiency was significantly more frequent in IPMN patients compared with controls (P = 0.001). Moreover, adjusted data revealed that urogenital cancer (P = 0.034), colorectal cancer (P = 0.021), as well as first-degree family history of colorectal cancer (P = 0.001) were significantly more frequent in IPMN patients., Conclusions: A history of urogenital and colorectal cancer often coincides with IPMN, which have an indication for surgery and are associated with preoperative episodes of pancreatitis and with endocrine insufficiency. Prospective studies are needed to investigate the role of these factors in IPMN development., Competing Interests: The authors declare no conflict of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

The incidence of uterine corpus cancer has been increasing globally due to increase in obesity. However, a detailed analysis of long-term epidemiological trends of corpus cancer in Japan, where obesity is relatively minimal, has not been conducted. In this retrospective, population-based study using the Osaka Cancer Registry, we analyzed 15 255 cases of corpus neoplasia registered between 1977 and 2016. We determined the age-standardized incidence, mortality, relative survival and conditional survival rates, and the treatment trends for corpus cancer over the last 40 years in Japan. The age-standardized incidence rate of corpus neoplasia increased sharply in 2000-2011 (APC = 9.9, 95% CI: 8.4-11.3), whereas the mortality rate trended to a much more modest increase (APC = 3.3, 95% CI: 2.7-3.8). Compared to 1977-2000, 10-year survival rates for post-2000 cases of localized and regional corpus cancers significantly improved (from 87.7% [95% CI: 85.8-89.4] to 94.2% [95% CI: 92.7-95.7] and from 47.5% [95% CI: 43.3-51.6] to 64.4% [95% CI: 61.0-67.6], respectively). This was largely associated with the significant increase in the percentage of localized and regional patients who received chemotherapy instead of radiation as an adjuvant therapy combined to surgery (P < .001 for both). We found that each histological type (endometrioid carcinoma, serous carcinoma, clear cell carcinoma and carcinosarcoma) has different characteristics of trend of age-standardized incidence rate, relative survival and distribution of extent of disease. In endometrioid carcinoma, the age-standardized incidence rate increased consistently after 1990, but the rate of increase was decreasing after 1997., (© 2021 The Authors. International Journal of Cancer published by John Wiley & Sons Ltd on behalf of UICC.)

Purpose: To investigate the importance of mucinous histopathology on the assessment of tumor response in patients with metastatic colorectal cancer (mCRC) receiving regorafenib., Materials and Method: All patients diagnosed with histologically confirmed mCRC in 2 oncology centers between 2013 and 2018 were retrospectively analyzed. Among 678 patients diagnosed with mCRC, 103 patients were treated with regorafenib. Ninety-four of these patients who had used at least 2 cycles of regorafenib and evaluable for treatment response were included in the analysis. Histopathologically, 18 patients with mucinous adenocarcinoma and 76 patients with nonmucinous adenocarcinoma were compared in terms of response rate and survival durations., Results: Median follow-up duration of 6 months, median age of the patients was 61 (34-77) years. While 19.1% of the patients had mucinous histology, 80.9% had nonmucinous histology. The overall response rate was significantly lower in the mucinous subgroup than the nonmucinous subgroup (5.6% vs 43.4%, respectively, P = 0.003). Similarly, both progression-free survival (3.0 vs 4.0 months, respectively, P = 0.011) and overall survival duration were shorter in the mucinous subgroup (3.0 vs 7.0 months, P = 0.016, respectively) compared with the nonmucinous subgroup., Conclusion: The histological subgroup may predict tumor response in mCRC patients receiving regorafenib. Its efficacy on nonmucinous histology had significantly more favorable than mucinous subtype., (Copyright © 2020. Published by Elsevier Inc.)

Background: Clinically, locoregional recurrences following mucinous tumor resection are often experienced. However, it remains unclear whether mucinous tumors directly affect local recurrence or not, and if so, the mechanism is not known. Therefore, we investigated whether mucinous tumors are associated with locoregional recurrence after pulmonary resection and whether mucus extension is a risk factor for locoregional recurrence., Methods: The data of 152 patients who underwent pulmonary resection for metastases were reviewed. When mucus was partially or wholly present in the tumor based on macro- or microscopic identification, we assigned the tumor as mucinous. In mucinous tumors, when mucus was identified within the air spaces in the normal lung parenchyma, beyond the edge of the tumor, we assigned the tumor as positive for "mucus extension.", Results: The 5-year cumulative incidence of locoregional recurrence in patients with mucinous tumors was 48.1%, which was significantly higher than that observed in those with non-mucinous tumors (14.9%). Within the mucinous tumor, the presence of mucus extension beyond the tumor edge was an independent risk factor for locoregional recurrence after pulmonary resection (hazard ratio, 5.52; P = 0.019)., Conclusions: During the resection of mucinous cancer, surgeons should maintain sufficient distance from the tumor edge to prevent locoregional recurrences.

Objectives: The exact prevalence for intraductal papillary mucinous neoplasm (IPMN) in patients with chronic kidney disease (CKD) remains unknown. In this single-center case-control study, we aimed to study the prevalence and risk factors for IPMN in patients with CKD., Methods: We performed a retrospective case-control study comparing patients with and without CKD who had magnetic resonance imaging of the abdomen performed between January 2018 and December 2018. Patient demographic, clinical, and imaging metrics were extracted from chart review. The prevalence of IPMN was compared between the 2 groups., Results: A total of 800 patient charts were reviewed. There were 400 patients with CKD compared with an age-matched control group of 400 patients without CKD. The total prevalence of IPMN in patients with CKD was 13.7% (55/400) compared with 7.8% (29/400; P = 0.002) in non-CKD patients. The prevalence of diabetes mellitus was significantly higher in the CKD group (41% vs 14%, P = 0.0001). The percentage of patients consuming alcohol was significantly higher in the non-CKD group (23% vs 35%, P = 0.002)., Conclusions: Patients with CKD have a significantly higher prevalence of IPMN compared with non-CKD patients. Larger population-based studies are needed to confirm these findings., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Lung cancer remains the leading cause of cancer death globally. In 2015, the cancer classification guidelines of the World Health Organization were updated. The term "invasive mucinous adenocarcinoma (IMA)" aroused people's attention, while the clinicopathological factors that may influence survival were unclear., Methods: Data of IMA patients was downloaded from SEER database. Kaplan-Meier methods and log-rank tests were used to compare the differences in OS and LCSS. The nomogram was developed based on the result of the multivariable analysis. The discrimination and accuracy were tested by Harrell's concordance index (C-index), receiver operating characteristic (ROC) curve, calibration curve and decision curve analyses (DCA). Integrated discrimination improvement (IDI) index was used to evaluate the clinical efficacy., Results: According to multivariate analysis, the prognosis of IMAs was associated with age, differentiation grade, TNM stage and treatments. Surgery might be the only way that would improve survival. Area under the curve (AUC) of the training cohort was 0.834and 0.830 for3-and 5-year OS, respectively. AUC for 3-and 5-year LCSS were separately 0.839 and 0.839. The new model was then evaluated by calibration curve, DCA and IDI index., Conclusion: Based on this study, prognosis of IMAs was systematically reviewed, and a new nomogram was developed and validated. This model helps us understand IMA in depth and provides new ideas for IMA treatment.

Background: Our current knowledge of diabetes mellitus in intraductal papillary mucinous neoplasm is very limited and its prevalence and predictive value for malignant transformation are not clear. This study sought to systematically review the literature to define the prevalence of diabetes mellitus in intraductal papillary mucinous neoplasm and to evaluate the association of diabetes mellitus with the progression to high-grade dysplasia or invasive cancer., Methods: A PubMed/Medline systematic search was performed to identify studies reporting data on preoperative diabetes mellitus in intraductal papillary mucinous neoplasm. Articles meeting the predefined inclusion criteria were analyzed and a meta-analysis was performed. The study was preregistered (PROSPERO ID: CRD42020153581)., Results: From the initially detected 827 studies, 27 studies including resected patients with histologically confirmed intraductal papillary mucinous neoplasm were included. The global prevalence of preoperative diabetes mellitus was 25% (1,112 of 4,412); whereas new-onset/worsening diabetes mellitus was reported in 6% of patients (68 of 1,202). The meta-analysis revealed that patients with pre-existing diabetes mellitus had an increased risk of harboring a main pancreatic duct involvement (risk ratio 1.43, 95% confidence interval: 1.21-1.69, P < .001), high-grade dysplasia (risk ratio 1.27, 95% confidence interval: 1.01-1.59, P = .04), and invasive cancer (risk ratio 1.61, 95% confidence interval: 1.33-1.95, P < .001)., Conclusion: The prevalence of diabetes mellitus in intraductal papillary mucinous neoplasm is high, and diabetic patients demonstrate an increased risk of a more aggressive disease. Therefore, diabetes mellitus should be increasingly considered in the stratification of patients with intraductal papillary mucinous neoplasm. Further investigations to determine the mechanisms behind the association with progression should be carried out., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background & Aims: The role of diabetes in intraductal papillary mucinous neoplasms (IPMNs) is not known. We investigated the prevalence of diabetes among patients with resected IPMNs and the association between diabetes, clinical and morphological features, and high-grade dysplasia or invasive cancer., Methods: We collected clinical, pathology, laboratory, and demographic data from 134 patients who underwent pancreatic resection for IPMN from a referral center in Germany. We identified 50 patients with diabetes (37%)., Results: Higher proportions of patients with diabetes were male and older, but did not have increased body mass index, compared to patients without diabetes. Diabetes was significantly associated with main-duct involvement (odds ratio [OR], 2.827; 95% CI, 1.059-7.546; P = .038) and high-grade dysplasia or invasive carcinoma (OR, 2.692; 95% CI, 1.283-5.651; P = .009). Risk of high-grade dysplasia or invasive cancer was even higher in patients with new-onset or worsening diabetes (OR, 4.615; 95% CI, 1.423-14.698; P = .011). Fifty-eight percent of patients (18/31) with weight loss at diagnosis had diabetes vs 32% of patients (31/97) without weight loss (P = .009). However, when the analysis was restricted to IPMNs with low-grade dysplasia, weight loss and diabetes were no longer associated (42% [5/12] vs 21% [9/44]; P = .133)., Conclusions: In patients with IPMNs, diabetes is associated with increased risk of main duct involvement and high-grade dysplasia or invasive carcinoma. Studies are needed to determine the relationship between diabetes and progression of IPMNs, which might lead to strategies for early detection and prevention of invasive cancer. Findings from this study should be considered in the guidelines for management of IPMN., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Importance: The incidence of appendiceal cancer (AC) is rising, particularly among individuals younger than 50 years (early-onset AC), with unexplained etiologies. The unique spectrum of somatic cancer gene variations among patients with early-onset AC is largely undetermined., Objective: To characterize the frequency of somatic variations and genomic patterns among patients with early-onset (age <50 years) vs late-onset (age ≥50 years) AC., Design, Setting, and Participants: This cohort study included individuals aged 18 years and older diagnosed with pathologically verified AC. Cases with clinical-grade targeted sequencing data from January 1, 2011, to December 31, 2019, were identified from the international clinicogenomic data-sharing consortium American Association for Cancer Research Project Genomics Evidence Neoplasia Information Exchange (GENIE). Data analysis was conducted from May to September 2020., Exposures: Age at disease onset., Main Outcomes and Measures: Somatic variation prevalence and spectrum in AC patients was determined. Variation comparisons between early-onset and late-onset AC were evaluated using multivariable logistic regression with adjustment for sex, race/ethnicity, histological subtype, sequencing center, and sample type., Results: In total 385 individuals (mean [SD] age at diagnosis, 56.0 [12.4] years; 187 [48.6%] men; 306 [79.5%] non-Hispanic White individuals) with AC were included in this study, and 109 patients (28.3%) were diagnosed with early-onset AC. Race/ethnicity differed by age at disease onset; non-Hispanic Black individuals accounted for a larger proportion of early-onset vs late-onset cases (9 of 109 [8.3%] vs 11 of 276 [4.0%]; P = 0.04). Compared with patients aged 50 years or older at diagnosis, patients with early-onset AC had significantly higher odds of presenting with nonsilent variations in PIK3CA, SMAD3, and TSC2 (PIK3CA: odds ratio [OR], 4.58; 95% CI, 1.72-12.21; P = .002; SMAD3: OR, 7.37; 95% CI, 1.24-43.87; P = .03; TSC2: OR, 12.43; 95% CI, 1.03-149.59; P = .047). In contrast, patients with early-onset AC had a 60% decreased odds of presenting with GNAS nonsilent variations compared with patients with late-onset AC (OR, 0.40; 95% CI, 0.21-0.76, P = .006). By histological subtype, young patients with mucinous adenocarcinomas of the appendix had 65% decreased odds of variations in GNAS compared with late-onset cases in adjusted models (OR, 0.35; 95% CI, 0.15-0.79; P = .01). Similarly, patients with early-onset nonmucinous appendiceal adenocarcinomas had 72% decreased odds of presenting with GNAS variations vs late-onset cases, although these findings did not reach significance (OR, 0.28; 95% CI, 0.07-1.14; P = .08). GNAS and TP53 variations were mutually exclusive in ACs among early-onset and late-onset cases (P < .05)., Conclusions and Relevance: In the study, AC diagnosed among younger individuals harbored a distinct genomic landscape compared with AC diagnosed among older individuals. Development of therapeutic modalities that target these unique molecular features may yield clinical implications specifically for younger patients.

Population-based evidence regarding risk of ovarian cancer after a borderline ovarian tumor (BOT) is sparse. We aimed to examine the incidence of specific types of ovarian cancer in women with serous or mucinous BOTs in a nationwide cohort study with up to 36 years of follow-up. Using the nationwide Danish Pathology Data Bank, we identified 4,281 women with a BOT (2,058 serous BOTs and 2,223 mucinous BOTs) in Denmark during 1978-2012. We computed standardized incidence ratios (SIRs) to compare the incidence of ovarian cancer among women with BOTs compared to general population rates. We found that a serous BOT was especially and strongly associated with subsequent serous ovarian cancer (SIR = 9.2; 95% CI: 6.8-12.2), and that a mucinous BOT was strongly related to mucinous ovarian cancer (SIR = 18.6; 95% CI: 10.8-29.8). The SIRs remained elevated ≥10 years after a serous BOT and up to 5-9 years after a mucinous BOT. The increased incidence of serous ovarian cancer in women with a serous BOT was mostly pronounced in women <50 years at the serous BOT diagnosis. In conclusion, women with a serous BOT experience long-term increased incidence of serous ovarian cancer, and women with a mucinous BOT have long-term elevated incidence of mucinous ovarian cancer compared to the general population. This is the first population-based study to show compelling evidence of the histo-specific increased risk of ovarian cancer following specific types of BOTs. Thus, these results are supportive of the hypothesis that BOTs may be precursor lesions to carcinomas of the corresponding histologic type., (© 2020 UICC.)

Background: Identification of germline and somatic BRCA1/2 mutations in ovarian cancer is important for genetic counseling and treatment decision making with poly ADP ribose polymerase inhibitors. Unfortunately, data on the frequency of BRCA1/2 mutations in Vietnamese patients are scare., Methods: We aim to explore the occurrence of BRCA1/2 mutations in 101 Vietnamese patients with ovarian cancer including serous (n = 58), endometrioid (n = 14), mucinous (n = 24), and clear cell (n = 5) carcinomas. BRCA1/2 mutations were detected from formalin-fixed parafin-embedded tumor samples using the OncomineTM BRCA Research Assay on Personal Genome Machine Platform with Ion Reporter Software for sequencing data analysis. The presence of pathogenic mutations was confirmed by Sanger sequencing., Results: We found no BRCA2 mutation in the entire cohort. Four types of pathogenic mutations in BRCA1 (Ser454Ter, Gln541Ter, Arg1751Ter, and Gln1779AsnfsTer14) were detected in 8 unrelated patients (7.9%) belonging to serous and endometrioid carcinoma groups. Except for the c.1360&#95;1361delAG (Ser454Ter) mutation in BRCA1 exon 11 that was somatic, the other mutations in exons 11, 20, and 22 were germline.  Interestingly, the recurrent Arg1751Ter mutation in BRCA1 exon 20 appeared in 4 patients, suggesting that this is a founder mutation in Vietnamese patients., Conclusion: Mutational analysis of tumor tissue using next generation sequencing allowed the detection of both germline and somatic BRCA1/2 mutations., .

Background: Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare low grade renal tumour exhibiting characteristic morphological features. We share our experience and discuss briefly, a review of the current literature., Methods: Electronic medical records were searched between January 2005 to January 2017. The histopathology and immunohistochemistry slides were retrieved and reviewed., Results: Eleven cases of MTSCC were identified. Mean age at presentation was 53.9 (age range 41 to 71) years with a slight female preponderance (F: M=6:5). Clinical stage at presentation was: Stage I (4 cases), Stage II (3 cases), Stage III (1 case), and Stage IV (3 cases). The average tumour size was 7.5cm (range 3.5 to 17cm). Microscopically, characteristic biphasic tumour with tubular and spindle cell morphology with variable mucinous stroma was noted in all. The prominent immunohistochemical profile revealed positivity for CK7 (7/8, 87.5%), AMACR (6/8, 75%), AE1/3 (4/4, 100%), CD10 (3/10, 27.3%), and Vimentin (3/3, 100%). Seven patients (Stage I and II) had been treated with nephrectomy, whereas only a diagnostic biopsy was available in four patients who presented with locally advanced disease (n=1) or distant metastasis (n=3) at presentation. The mean follow-up was 37.8 months (range 8 to 96 months), available in 10 out of 11 patients, without recurrence in nine while one died 8 months after diagnosis., Conclusion: MTSCC is an indolent renal cancer with characteristic morphology. However, presentation with locally advanced disease or distant metastasis may be seen in a subset of these patients. This warrants close follow-up in even localized tumors., Competing Interests: None

Objective: The aim of this nationwide study was to provide insight in the incidence, risk factors, treatment, and survival of patients with ovarian metastases from colorectal cancer (CRC)., Methods: Data from the Netherlands Cancer Registry were used. All newly diagnosed female CRC patients between 2008 and 2016 were included. Treatment was categorized as follows: cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy (CRS-HIPEC); resection of the primary tumor; palliative treatment; and no treatment. Overall survival (OS) was investigated using Kaplan-Meier and multivariable Cox regression analyses., Results: Of 53,883 female CRC patients, 11,343 (21.1%) had metastases at time of diagnosis. Among them, 471 (4.2%) had ovarian metastases. Within latter group, 27.2% received CRS-HIPEC; 38.4% underwent resection of the primary tumor; 25.3% received palliative treatment; and 9.1% received no treatment. Median OS of all patients with ovarian metastases was 17.5 months. In patients receiving CRS-HIPEC, OS was significantly longer than in patients undergoing resection only (median OS 34.1 vs. 17.5 months, adjusted HR 0.44 [0.33-0.66]). Five-year OS was 28.5% for patients having underwent CRS-HIPEC, 11.0% for patients having underwent resection of the primary tumor, 1.2% for patients having underwent palliative treatment, and 0.0% for patients without treatment., Conclusions: Synchronous ovarian metastases are diagnosed in 4.2% of female colorectal patients presenting with metastatic disease. Risk factors are young age, T4/N+ tumor and histology of signet ring cell carcinoma. Median OS of the entire cohort was 17.5 months, ranging from 3.1 months in patients without treatment to 34.1 months in patients undergoing CRS-HIPEC.

Objective: To assess the incidence and the risk factors of venous thromboembolism (VTE) in patients with epithelial ovarian carcinoma (EOC) during the perioperative period., Methods: A retrospective analysis was conducted on the patients with epithelial ovarian cancer treated in our hospital, between January 2017 and July 2019, and a comprehensive review of the medical documentation was performed to collect relevant data. We then analyzed the related factors of the thrombosis in the EOC patients, using univariate and multivariate analysis to identify significant risk factors for VTE, and bootstrap resampling method was used to verify the multivariate analysis results. The ROC curve methods were conducted to evaluate the diagnostic value for the prediction of VTE., Results: We analyzed 233 cases of patients with EOC, of whom the incidence of VTE was 11.16%. According to multivariate and 5000 bootstrap samples analysis, preoperative D-dimer levels (>4.215 μg/ml, p = 0.041 and p = 0.032) and comorbid of cerebral infarction (p < 0.001 and p < 0.001) had statistical significance in predicting VTE events; bootstrap analysis also found the Alb, CA125, OCCC had statistical significance. While According to multivariate and 5000 bootstrap samples analysis, age (>50.5 years old, p = 0.019 and p = 0.002) and nonoptimal debulking surgery (p = 0.007 and p = 0.002) showed significance in predicting VTE after surgery; bootstrap analysis also found the D-dimer levels (>4.215 μg/ml) and tuberculosis had statistical significance., Conclusion: More effective thromboprophylaxis and pre-test assessment is necessary for EOC patients. For prediction VTE events, D-dimer levels (>4.215 μg/ml) were the independent predictors before operation. Age and debulking surgery were the independent predictors post operation., Competing Interests: Declaration of competing interest The authors declare no potential conflicts of interest., (Copyright © 2020 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Background: Colorectal cancer (CRC) in Egypt is a relatively high young onset disease. As a form of heterogeneous cancer, there is interplay between genetic and environmental factors. We aimed at probing the association of life style factors and Microsatellite Instability (MSI) status that could provide more insights on carcinogenic process of CRC., Methods: One hundred incident sporadic CRC patients were involved. Information on risk factors of CRC was obtained and microsatellite instability status was predicted through evaluation of MMR protein expression via immunohistochemistry (IHC)., Results: Median age was 47.50 years, females represented 54.0% and 36% of patients were Microsatellite Instability High (MSI-H). Most patients with right sided colon cancer (78.3%) were MSI-H while mostly stable or low MSS/MSI-L for left-sided colon and rectum (78.6%, 74.3% respectively, p<0.001). Patients with low physical activity had higher risk of MSS/MSI-L than those with moderate or high activity p =0.026. Patients with BMI greater than 30 Kg/m2 had higher MSS/MSI-L (75.5%) than those with BMI between 25-30 Kg/m2 (60.6%) and those with normal BMI.

Mucinous carcinoma (MC) is a rare breast cancer characterized by the presence of large extracellular mucin amount. Two main subtypes can be distinguished: pure (PMC) and mixed (MMC). We conducted a retrospective MC analysis in our prospective maintained database, calculating disease-free survival (DFS) and 5-year overall survival (OS). We found a global 92.1% OS (higher in MMC group and statistically significative) and a DFS of 95.3% (higher in MMC group but not statistically significative)., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest in relation to this work., (Copyright © 2019 The Authors. Published by Elsevier Ltd.. All rights reserved.)

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.

Background & Aims: Long-term outcomes of patients with branch-duct intraductal papillary mucinous neoplasms (IPMNs), particularly those after 5 years of surveillance, have not been fully evaluated in large studies. We analyzed incidences of IPMN-derived carcinoma and concomitant ductal adenocarcinoma (pancreatic ductal adenocarcinoma [PDAC]) over 20 years in a large population of patients., Methods: We identified 1404 consecutive patients (52% women; mean age, 67.5 years) with a diagnosis of branch-duct IPMN, from 1994 through 2017, at the University of Tokyo in Japan. Using a competing risk analysis, we estimated cumulative incidence of pancreatic carcinoma, overall and by carcinoma type. We used competing risks proportional hazards models to estimate subdistribution hazard ratios (SHRs) for incidences of carcinomas. To differentiate IPMN-derived and concomitant carcinomas, we collected genomic DNA from available paired samples of IPMNs and carcinomas and detected mutations in GNAS and KRAS by polymerase chain reaction and pyrosequencing., Results: During 9231 person-years of follow-up, we identified 68 patients with pancreatic carcinomas (38 patients with IPMN-derived carcinomas and 30 patients with concomitant PDACs); the overall incidence rates were 3.3%, 6.6%, and 15.0% at 5, 10, and 15 years, respectively. Among 804 patients followed more than 5 years, overall cumulative incidence rates of pancreatic carcinoma were 3.5% at 10 years and 12.0% at 15 years from the initial diagnosis. The size of the IPMN and the diameter of the main pancreatic duct associated with incidence of IPMN-derived carcinoma (SHR 1.85; 95% confidence interval 1.38-2.48 for a 10-mm increase in the IPMN size and SHR 1.56; 95% confidence interval 1.33-1.83 for a 1-mm increase in the main pancreatic duct diameter) but not with incidence of concomitant PDAC., Conclusions: In a large long-term study of patients with branch-duct IPMNs, we found the 5-year incidence rate of pancreatic malignancy to be 3.3%, reaching 15.0% at 15 years after IPMN diagnosis. We observed heterogeneous risk factor profiles between IPMN-derived and concomitant carcinomas., (Copyright © 2020 AGA Institute. Published by Elsevier Inc. All rights reserved.)

Colorectal carcinoma (CRC) is the third most common malignancy in adults. Pediatric colorectal carcinoma (PCRC) is a rare non-embryonal tumor with a significantly lower incidence compared to adults. The clinical manifestations of PCRC are not typical, and pediatricians usually have no enough experience in diagnosis and treatment. Therefore, early diagnosis is extremely difficult, which would always lead to late clinical stages when diagnosis is made. At present, the pathogenesis of PCRC is still not clear, and many countries have started to carry out researches at the level of genes, molecules and cells. In both tumor primary tumors and distant metastases, PCRC has obvious difference in distribution from adults, and the proportion of pathological type of mucous adenocarcinoma (including the signet ring cell carcinoma) was significantly higher than that of adults. Although treated according to adult colorectal cancer guidelines, PCRC has been unable to achieve ideal efficacy with poor prognosis and lower long-term survival rate. The purpose of this paper is to summarize the epidemiological characteristics, pathogenesis, clinical symptoms, pathological types, treatment and prognosis of colorectal cancer in children by reviewing the latest literatures at home and abroad.

Background: Intraductal papillary mucinous neoplasm (IPMN) is premalignant pancreatic lesion. International guidelines offer limited predictors of individual risk. A nomogram to predict individual IPMN malignancy risk was released, with good diagnostic performance based on a large cohort of Asian patients with IPMN. The present study validated a nomogram to predict malignancy risk and invasiveness of IPMN using both Eastern and Western cohorts., Methods: Clinicopathological and radiological data from patients who underwent pancreatic resection for IPMN at four centres each in Eastern and Western countries were collected. After excluding patients with missing data for at least one malignancy predictor in the nomogram (main pancreatic duct diameter, cyst size, presence of mural nodule, serum carcinoembryonic antigen and carbohydrate antigen (CA) 19-9 levels, and age)., Results: In total, data from 393 patients who fit the criteria were analysed, of whom 265 were from Eastern and 128 from Western institutions. Although mean age, sex, log value of serum CA19-9 level, tumour location, main duct diameter, cyst size and presence of mural nodule differed between the Korean/Japanese, Eastern and Western cohorts, rates of malignancy and invasive cancer did not differ significantly. Areas under the receiver operating characteristic (ROC) curve values for the nomogram predicting malignancy were 0·745 for Eastern, 0·856 for Western and 0·776 for combined cohorts; respective values for the nomogram predicting invasiveness were 0·736, 0·891 and 0·788., Conclusions: External validation of the nomogram showed good performance in predicting cancer in both Eastern and Western patients with IPMN lesions., (© 2019 BJS Society Ltd Published by John Wiley & Sons Ltd.)

Aim: To characterize the epidemiology of appendiceal mucinous adenocarcinoma. Methods: Prognostic factors were evaluated with univariate and multivariate analyses. The results were used to generate a nomogram. Results: The incidence of appendiceal mucinous adenocarcinoma showed a significant upward trend. Multivariate Cox analysis identified 11 independent prognostic factors. The nomogram was based on independent risk factors that were significant in multivariate Cox analysis, and the concordance-index for overall survival and cancer-specific survival were 0.76 (95% CI: 0.71-0.79) and 0.74 (95% CI: 0.70-0.79), respectively. Conclusion: Advanced age, single relationship status, male sex, black race, the presence of distant and regional lymph node metastases, poor differentiation or lack of differentiation, advanced SEER extent of disease, cancer-directed surgery and chemotherapy were independently associated with prognosis.

We explored the frequency of germline and somatic mutations in homologous recombination (HR)-associated genes in major histological types of ovarian cancer. We performed targeted sequencing to assess germline and somatic mutations of 16 HR-associated genes and 4 mismatch repair (MMR) genes among 207 ovarian cancer patients (50 high-grade serous carcinomas (HGSC), 99 clear cell carcinomas (CCC), 39 endometrioid carcinomas (EC), 13 mucinous carcinomas (MC), and 6 low-grade serous carcinomas (LGSC)). Germline or somatic mutations of HR-associated genes were detected in 44% of HGSC, 28% of CCC, 23% of EC, 16% of MC, and 17% of LGSC patients. The profile of HR-associated gene mutations was remarkably different among each histological type. Germline BRCA1/2 mutations were frequently detected in HGSC and were rarely observed in CCC, EC, and MC patients. ATM somatic mutation was more frequently detected in CCC (9%) and EC patients (18%) than in HGSC patients (4%). There was a positive correlation between MMR gene mutations and HR-associated gene mutations (p = 0.0072). Our findings might be useful in selection of ovarian cancer patients that should be treated with PARP inhibitors.

Introduction: breast cancer is the most common cancer affecting women worldwide. It is a heterogeneous disease with diverse histological types that are associated with different natural history and response to therapy. Invasive ductal and lobular carcinoma are the most common histological types. There are rare histological types with different biological behaviours from the common types, although treatment approaches are the same. Data on rare histological types of breast cancer in our population are scarce raising the need to identify these patients and document their treatment outcome. The objectives of this study are to determine the proportion and treatment outcomes of breast cancer patients with rare histological types., Methods: this was an observational retrospective study using records of patients treated for breast cancer at the University College Hospital Ibadan Nigeria from 2008 to 2012. Patients with rare histological types were selected for further analysis. Data on patient and tumour characteristics were extracted and five-year survival pattern was determined using Kaplan Meier method., Results: the total number of patients with breast cancer was 761. Thirty-two (4.2%) had rare histology that consisted of medullary carcinoma 14(1.9%), mucinous carcinoma 10(1.4%) and 2(0.3%) each for squamous cell carcinoma, stromal sarcoma, cribriform carcinoma and Paget's disease. The overall five-year survival was 50% with median survival of 52 months., Conclusion: the proportion of breast cancer patients with rare histology is low similar to other reports among Caucasians. Medullary adenocarcinoma was the most common subtype followed by mucinous adenocarcinoma., Competing Interests: The authors declare no competing interests., (© Atara Isaiah Ntekim et al.)

Background: The inherited predisposition to developing specific histologic subtypes of invasive breast carcinoma has been incompletely investigated. By using a large, population-based database, the authors sought to investigate familial clustering of breast cancer by histologic subtype., Methods: By using the Utah Population Database, which links genealogy records to the National Cancer Institute's statewide Surveillance, Epidemiology, and End Results cancer registry, the authors identified patients with breast cancer by histology and tested for evidence of shared genetic predisposition to histologic specific subtypes by examining pairwise relatedness and estimating the relative risk (RR) among first-degree, second-degree, and third-degree relatives., Results: The authors identified 23,629 individuals in the Utah Population Database who had at least 3 generations of genealogy and at least 1 primary breast cancer, 2883 (12.2%) of which were specific histologic subtypes other than invasive ductal carcinoma (including inflammatory [n = 178], lobular [n = 1688], and mucinous [n = 542]). Statistically significant excess distant relatedness was identified for the mucinous subtype (P = .011) as well as for inflammatory breast cancers (P = .024). The RR for breast cancer of any histology in second-degree relatives was significantly increased for patients with inflammatory (RR, 1.32; 95% CI, 1.02-1.68; P = .03), lobular (RR, 1.36; 95% CI, 1.25-1.47; P < .001), and mucinous (RR, 1.27; 95% CI, 1.12-1.44; P = .00021) subtypes., Conclusions: These findings provide evidence for significant familial clustering within histological subtypes for lobular, mucinous, and inflammatory breast carcinomas. Further research is required to identify the underlying genetic variants responsible for the increased risk. Studies of high-risk pedigrees segregating a specific histologic subtype could be a powerful design for predisposition gene identification., (© 2019 American Cancer Society.)

Due to insufficient quantitative evaluation of the clinic-pathological features and prognosis of young colorectal cancer (CRC) with mucinous adenocarcinoma (MAC) and signet-ring cell carcinoma (SRC), the aim of our study was to develop a nomogram to identify the prognostic predictors for overall survival (OS) in this patient population. We retrospectively evaluated the patient records of MAC and SRC patients aged ≤ 40 years. Kaplan-Meier analysis and log-rank testing were performed to estimate OS. A nomogram predicting OS was created for risk quantitation and decision tree analysis was performed for patient grouping. With a median follow-up of 36.5 months, we included a total of 90 young CRC patients for analysis. The overall cumulate 5-year OS rate was 57.7% (95% confidence interval (CI): 45.1-68.5%). The estimated 5-year OS was 62.9% (95% CI: 48.5-74.3%) for MAC and 37.3% (95% CI: 14.4-61.2%) for SRC ( P =0.021). The recurrence rate was significantly greater in the SRC group compared with the mucinous group (52.4 compared with 26.1%, P =0.047). In the multivariate Cox regression model, preoperative carcinoembryonic antigen (CEA) levels and cycles of adjuvant chemotherapy (CT) were found to be an independent prognostic factor for OS (hazard ratio (HR): 2.43; 95% CI: 1.13-5.62, P =0.024; HR: 0.21; 95% CI: 0.083-0.57, P =0.002, respectively). Nomograms predicting 3- and 5-year OS were established that performed well (concordance index (c-indexes) of 0.636, 95% CI: 0.549-723) for OS. For MAC and SRC disease, a greater proportion of young patients present with advanced disease, and the prognosis for young SRC patients is poorer than MAC. Furthermore, preoperative CEA levels and cycles of adjuvant CT seem to independently affect the OS in this patient population., (© 2019 The Author(s).)

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.

Background & Aims: It has been a challenge to select treatment for patients with pancreatic ductal adenocarcinomas (PDACs) based on genome alterations. We performed targeted genomic profile analyses of a large number of PDACs to assess the full spectrum of actionable genomic alterations., Methods: We performed targeted genomic profile analyses of 3594 PDAC samples from an international cohort, including capture-based targeted genomic profiling of as many as 315 cancer-associated genes and intron regions of 28 genes that are rearranged in cancer cells. Tumor mutation burden (TMB) and microsatellite instability (MSI) status were also assessed. TMB was calculated across a 1.14-megabase region; TMB-high was defined as ≥20 mutations/megabase. MSI-high status was assigned based on analysis of 114 intron homopolymer loci., Results: KRAS, TP53, CDKN2A, and SMAD4 were the most frequently altered genes in PDAC. We found KRAS mutations in 88% of samples. Among PDACs without mutations in KRAS, we found alterations in genes whose products are in the mitogen-activated protein kinase signaling pathway and are candidate drug targets (actionable targets, n = 132; 4%), as well as gene fusions (n = 51), gene amplifications (n = 35), genes with missense mutations (n = 30), and genes that contain deletions (n = 16). Many of these encode proteins in receptor tyrosine kinase, RAS, or mitogen-activated protein kinase signaling pathways. Aside from TP53, alterations in genes encoding DNA damage repair proteins (BRCA and FANC) were detected in 14% of PDACs. Among PDACs evaluated for MSI (n = 2563) and TMB (n = 1021), MSI-high and/or TMB-high phenotypes were detected in 0.5% of samples. Alterations in FGF23, CCND2, PIK3CA, and FGF6 were more commonly detected in intraductal papillary mucinous neoplasm-associated PDACs., Conclusions: In targeted genomic profile analyses of 3594 PDACs, we found 17% to contain genomic alterations that might make the tumor cells susceptible to currently used anticancer agents. We identified mutations in genes that could contribute to progression of intraductal papillary mucinous neoplasms into malignancies. These alterations might be used as biomarkers for early detection., (Copyright © 2019 AGA Institute. Published by Elsevier Inc. All rights reserved.)

Background: Polycystic ovary syndrome (PCOS) is a complex endocrine disorder with an estimated prevalence of 4-21% in reproductive aged women. Recently, the Ovarian Cancer Association Consortium (OCAC) reported a decreased risk of invasive ovarian cancer among women with self-reported PCOS. However, given the limitations of self-reported PCOS, the validity of these observed associations remains uncertain. Therefore, we sought to use Mendelian randomization with genetic markers as a proxy for PCOS, to examine the association between PCOS and ovarian cancer., Methods: Utilizing 14 single nucleotide polymorphisms (SNPs) previously associated with PCOS we assessed the association between genetically predicted PCOS and ovarian cancer risk, overall and by histotype, using summary statistics from a previously conducted genome-wide association study (GWAS) of ovarian cancer among European ancestry women within the OCAC (22 406 with invasive disease, 3103 with borderline disease and 40 941 controls)., Results: An inverse association was observed between genetically predicted PCOS and invasive ovarian cancer risk: odds ratio (OR)=0.92 [95% confidence interval (CI)=0.85-0.99; P = 0.03]. When results were examined by histotype, the strongest inverse association was observed between genetically predicted PCOS and endometrioid tumors (OR = 0.77; 95% CI = 0.65-0.92; P = 0.003). Adjustment for individual-level body mass index, oral contraceptive use and parity did not materially change the associations., Conclusion: Our study provides evidence for a relationship between PCOS and reduced ovarian cancer risk, overall and among specific histotypes of invasive ovarian cancer. These results lend support to our previous observational study results. Future studies are needed to understand mechanisms underlying this association., (© The Author(s) 2019; all rights reserved. Published by Oxford University Press on behalf of the International Epidemiological Association.)

Background: The prognostic value of tumor sidedness in metastatic colorectal cancer (CRC) has been established, but its impact on nonmetastatic disease remains unclear. Our study aimed to explore the prognostic effect of tumor sidedness by subgroup survival analyses, according to histology and tumor grade in stage I-IV CRCs., Methods: A retrospective population-based study was conducted based on Surveillance, Epidemiology and End Results (SEER) data. Population data in the SEER 9 registry (1975-2014) were used to determine survival trends of CRCs, and associated population data in the SEER 18 registry (2000 to 2014) were used to assess the prognostic impact of tumor sidedness on CRCs., Results: The 5-year cause-specific survival for all subgroups of CRCs improved from 1975 to 2014. Of 238,826 patients, 44.2% had right-sided cancer. Patients with right-sided cancer were more likely to be older, to be women, to have disease of mucinous or signet-ring cell histology, to have more poorly differentiated tumors, and to be diagnosed with a more advanced disease stage. Multivariate Cox regression showed stage I-II right-sided cancers had better cause-specific survival than the left-sided cancers (left colon: hazard ratio [HR] = 1.091, 95% confidence interval [CI], 1.052-1.132; rectum: HR = 1.363; 95% CI, 1.304-1.425; P < .001), while stage III and IV right-sided cancers had worse cause-specific survival. In subgroup analyses by histology and tumor grade within stage III CRCs, right-sided poorly differentiated mucinous adenocarcinoma showed significantly better survival (left colon: HR = 1.352; 95% CI, 1.145-1.596; rectum: HR = 1.125; 95% CI, 0.916-1.381; P = .002)., Conclusion: The relationship between sidedness and prognosis in CRCs depends on stage and histopathologic characteristics, especially for stage III disease., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Pseudomyxoma peritonei (PMP) is a rare disease, most commonly of appendiceal origin. Treatment consists of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC). The aim of this study was to identify prognostic factors for recurrence and survival., Methods: This was an observational study using a prospectively designed database containing consecutive patients with PMP originating from the appendix, undergoing CRS-HIPEC at a tertiary referral centre between 1996 and 2015. Histopathological slides were reassessed. Cox regression was used for multivariable analyses., Results: Of 225 patients identified, 36 (16·0 per cent) were diagnosed with acellular mucin, 149 (66·2 per cent) had disseminated peritoneal adenomucinosis (DPAM) and 40 (17·8 per cent) had peritoneal mucinous carcinomatosis (PMCA). The 5-year overall survival (OS) rates were 93, 69·8 and 55 per cent respectively. Recurrence was observed in 120 patients (53·3 per cent), 39 of whom (17·3 per cent) were treated with a second CRS-HIPEC procedure. Factors independently associated with poor disease-free survival were six or seven affected regions (hazard ratio (HR) 6·01, 95 per cent c.i. 2·04 to 17·73), incomplete cytoreduction (R2a resection: HR 1·67, 1·05 to 2·65; R2b resection: HR 2·00, 1·07 to 3·73), and more than threefold raised carcinoembryonic antigen (CEA) and/or carbohydrate antigen (CA) 19-9 level (HR 2·31, 1·30 to 4·11). Factors independently associated with poorer OS were male sex (HR 1·74, 1·09 to 2·77), incomplete cytoreduction (R2a resection: HR 1·87, 1·14 to 3·08; R2b resection: HR 2·28, 1·19 to 4·34), and more than threefold raised CEA and/or CA19-9 level (HR 2·89, 1·36 to 6·16)., Conclusion: CEA and CA19-9 levels raised more than threefold above the upper limit identify patients with PMP of appendiceal origin and poorer survival.

Objective: Histamine is suggested to play a role in ovarian carcinogenesis. We examined the association between antihistamine use and ovarian cancer risk in a nationwide case-control study., Study Design: Cases (n = 5 556) comprised all women in Denmark aged 30-84 years with a histologically verified first diagnosis of epithelial ovarian cancer during 2000-2015. Age-matched population controls (n = 83 340) were selected using risk-set sampling. Data on prescription use, patient and demographic characteristics were retrieved from nationwide registries., Main Outcome Measures: We used conditional logistic regression to estimate odds ratios (ORs) with 95% confidence intervals (CIs) for epithelial ovarian cancer associated with antihistamine use (≥2 prescriptions). The association was evaluated according to patterns of antihistamine use, menopausal status, and histological subtype of ovarian cancer., Results: Ever use of antihistamines was not associated with ovarian cancer overall (OR = 0.97, 95% CI = 0.90-1.05). The lack of association remained in subanalyses for patterns of antihistamine use. We observed an inverse association between antihistamine use and ovarian cancer among pre-menopausal women (<50 year: OR = 0.72, 95% CI = 0.57-0.90), but not post-menopausal women (≥50 year: OR = 1.02, 95%CI = 0.93-1.11). In analyses of histological subtypes, an inverse association emerged for mucinous ovarian cancer (OR = 0.74, 95% CI = 0.57-0.96), but not for other subtypes., Conclusion: Antihistamine use was not associated with overall ovarian cancer risk. Additional research is needed to confirm inverse associations between antihistamine use and mucinous ovarian cancer, and overall ovarian cancer among pre-menopausal women., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objective: Germline mutations in BRCA1/2 genes predict improved survival and sensitivity to treatment with poly(adenosine-diphosphate-ribose) polymerase inhibitors in epithelial ovarian carcinoma. The prognostic importance of other genetic alterations leading to homologous recombination deficiency, collectively BRCAness phenotype, is unresolved. The aim was to analyze the distribution of homologous recombination deficiency in epithelial ovarian carcinoma caused by mutations in a panel of homologous recombination genes (including BRCA1/2 ) or epigenetic alterations. A further aim was to investigate the prognostic importance of homologous recombination deficiency, the BRCAness phenotype., Methods: We assessed 380 patient specimens from a Danish population-based epithelial ovarian carcinoma cohort for germline and somatic mutations in 18 different homologous recombination genes, including BRCA1 and BRCA2 , using next generation sequencing. Epigenetic alteration due to BRCA1 hypermethylation was assessed by pyrosequencing and BRCA1 protein expression was evaluated by immunohistochemistry., Results: Seventeen percent of patients with epithelial ovarian carcinoma carried a germline (9.8%) and/or somatic (6.3%) mutation in 12 ( BRCA1, BRCA2 , CHEK2 , ATM , RAD51D , EMSY , PALB2 , BRIP1 , ERCC1 , RAD50 , ATR , RAD51C ) of 18 sequenced homologous recombination genes. The homologous recombination mutation rate was similar among the different histologic subtypes, however the type of mutation ( BRCA1/2 and other homologous recombination mutations) differed, p=4×10 -4 . BRCA1 hypermethylation was present in 7.4% of patient specimens for a total BRCAness phenotype of 23.9%. The BRCAness phenotype was associated with improved overall survival in the high-grade serous carcinoma subgroup with a median overall survival of 4.4 years (95% CI 3.0 to 5.3) versus 2.2 years (95% CI 1.9 to 2.4) in BRCAness wildtype, p=0.0002. Multivariate analysis confirmed an independent prognostic value of the BRCAness phenotype among the high-grade serous carcinoma subgroup, hazard ratio 0.65 (95% CI 0.47 to 0.92), p=0.014., Conclusions: The BRCAness phenotype is present in almost one-fourth of epithelial ovarian carcinoma and holds important prognostic information. The implications of our findings in relation to poly(adenosine-diphosphate-ribose) polymerase inhibitor treatment call for further investigation., Competing Interests: Competing interests: None declared., (© IGCS and ESGO 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: The purpose of this case-control study was to compare the prognoses of women with stage III mucinous ovarian carcinoma (MOC) who received maximal or optimal cytoreduction followed by paclitaxel plus carboplatin chemotherapy to those of women with stage III serous epithelial ovarian cancer (EOC) treated in the similar manner., Methods: We performed a multicenter, retrospective review to identify patients with stage III MOC at seven gynecologic oncology departments in Turkey. Eighty-one women with MOC were included. Each case was matched to two women with stage III serous EOC in terms of age, tumor grade, substage of disease, and extent of residual disease. Survival estimates were measured using Kaplan-Meier plots. Variables predictive of outcome were analyzed using Cox regression models., Results: With a median follow-up of 54 months, the median progression-free survival (PFS) for women with stage III MOC was 18.0 months (95% CI; 13.8-22.1, SE: 2.13) compared to 29.0 months (95% CI; 24.04-33.95, SE: 2.52) in the serous group (p = 0.19). The 5-year overall survival rate of the MOC group was significantly lower than that of the serous EOC group (44.9% vs. 66.3%, respectively; p < 0.001). For the entire cohort, presence of multiple peritoneal implants (Hazard ratio [HR] 2.39; 95% confidence interval [CI], 1.38-4.14, p = 0.002) and mucinous histology (HR 2.28; 95% CI, 1.53-3.40, p < 0.001) were identified as independent predictors of decreased OS., Conclusion: Patients with MOC seem to be 2.3 times more likely to die of their tumors when compared to women with serous EOC.