Appearances Can Be Deceiving: A Case Report of Asymptomatic Appendiceal Goblet Cell Adenocarcinoma Presenting as an Ovarian Tumor.

Introduction: Goblet cell adenocarcinoma (GCA) of the appendix is an uncommon type of cancer that includes both epithelial and neuroendocrine features, with goblet cells present. These tumors have traditionally been viewed as one of the more aggressive forms of appendiceal cancer, frequently being diagnosed at a metastatic stage. For patients with stage III-IV disease, the 5-year overall survival rate ranges from 14% to 22%. Due to limited data, the diagnosis and management of GCA are challenging. Case Presentation: We present the case of a 55-year-old female who presented with elevated serum carcinoembryonic antigen levels and a left ovarian tumor. Preoperative imaging indicated a normal appendix. During surgery for suspected ovarian carcinoma, frozen section analysis revealed mucinous adenocarcinoma of the ovary, but distinguishing between primary and metastatic lesions was challenging. Meticulous exploration revealed a firm and thickened appendix, prompting appendectomy and subsequent diagnosis of appendiceal GCA with ovarian metastasis. Conclusion: Pathologies of the appendix, like GCA, can mimic ovarian tumors despite a normal-looking appendix, complicating diagnosis. Through this case, we underscored the need for careful intraoperative examination of the appendix in patients with mucinous ovarian neoplasms to avoid misdiagnosis and ensure appropriate treatment.

Multiparametric MRI-based radiomics nomogram for differentiation of primary mucinous ovarian cancer from metastatic ovarian cancer.

OBJECTIVE: To develop a multiparametric magnetic resonance imaging (mpMRI)-based radiomics nomogram and evaluate its performance in differentiating primary mucinous ovarian cancer (PMOC) from metastatic ovarian cancer (MOC). METHODS: A total of 194 patients with PMOC (n = 72) and MOC (n = 122) confirmed by histology were randomly divided into the primary cohort (n = 137) and validation cohort (n = 57). Radiomics features were extracted from axial fat-saturated T2-weighted imaging (FS-T2WI), diffusion-weighted imaging (DWI), and contrast-enhanced T1-weighted imaging (CE-T1WI) sequences of each lesion. The effective features were selected by minimum redundancy maximum relevance (mRMR) and least absolute shrinkage and selection operator (LASSO) regression to develop a radiomics model. Combined with clinical features, multivariate logistic regression analysis was employed to develop a radiomics nomogram. The efficiency of nomogram was evaluated using the receiver operating characteristic (ROC) curve analysis and compared using DeLong test. Finally, the goodness of fit and clinical benefit of nomogram were assessed by calibration curves and decision curve analysis, respectively. RESULTS: The radiomics nomogram, by combining the mpMRI radiomics features with clinical features, yielded area under the curve (AUC) values of 0.931 and 0.934 in the primary and validation cohorts, respectively. The predictive performance of the radiomics nomogram was significantly superior to the radiomics model (0.931 vs. 0.870, P = 0.004; 0.934 vs. 0.844, P = 0.032), the clinical model (0.931 vs. 0.858, P = 0.005; 0.934 vs. 0.847, P = 0.030), and radiologists (all P < 0.05) in the primary and validation cohorts, respectively. The decision curve analysis revealed that the nomogram could provide higher net benefit to patients. CONCLUSION: The mpMRI-based radiomics nomogram exhibited notable predictive performance in differentiating PMOC from MOC, emerging as a non-invasive preoperative imaging approach.

Mucinous ovarian carcinoma: A survey of practice in Australia and New Zealand.

BACKGROUND: Mucinous ovarian carcinoma (MOC) is a rare ovarian cancer with limited evidence to support clinical care. AIMS: We undertook a clinician survey to better understand current practice in treating MOC in Australia and New Zealand, and to determine any features associated with variation in care. In addition, we aimed to understand future research priorities. METHODS: A RedCap survey was distributed to clinician members of the Australia New Zealand Gynaecological Oncology Group (ANZGOG). Questions included respondent demographics, three case studies and future research priorities. Clinicians were asked questions specific to their speciality. RESULTS: Respondents (n = 47) were commonly experienced gynae-oncology specialists, most often surgical (38%) or medical (30%) oncologists. There was good consensus for surgical approaches for stage I disease; however, variation in practice was noted for advanced or recurrent MOC. Variation was also observed for medical oncologists; in early-stage disease there was no clear consensus on whether to offer chemotherapy, or which regimen to recommend. For advanced and recurrent disease a wide range of chemotherapy options was considered, with a trend away from an ovarian-type toward gastrointestinal (GI)-type regimens in advanced MOC. This practice was reflected in future research priorities, with 'Is a GI chemotherapy regimen better than an ovarian regimen?' the most highly ranked option, followed by 'Should stage 1C patients receive chemotherapy?' CONCLUSIONS: Although the number of respondents limited the analyses, it was clear that chemotherapy selection was a key point of divergence for medical oncologists. Future research is needed to establish well-evidenced guidelines for clinical care of MOC.

Is routine gastrointestinal endoscopy required in every woman with mucinous ovarian cancer? An analysis of survival rates and metastatic tumours in a cancer centre.

INTRODUCTION: Mucinous ovarian cancer (MOC) represents a rare entity of ovarian malignant neoplasms. The true incidence could be as low as 3% of all ovarian cancers. The aim of this study is to compare and understand the clinicopathological characteristics of patients with mucinous ovarian cancer, report on the survival rates and evaluate the role of gastrointestinal (GI) endoscopy as part of the peri-operative investigations and the impact it has on the survival rates. METHODOLOGY: This is a retrospective data collection on patients with MOC operated in Nottingham gynaecological oncology centre over a 10-year period. Data were analysed using SPSS software. RESULTS: 43 cases were included in the final analysis. The median maximal tumour diameter was 180 mm. 32 (74.5 %) and 11 (25.5 %) women presented with unilateral and bilateral tumours respectively. 30 patients (69.7 %) presented with stage 1 disease, 1 (2.3 %) presented with stage 2 disease, 7 women (16.4 %) had stage 3 disease and 1 woman (11.6 %) had stage 4 disease. 41 women had staging surgical procedures and 2 women had limited surgery due to poor performance status. After final histology, 5 cases found to have metastatic disease to the ovary rather than primary MOC. 14 women had GI endoscopy as part of their investigation. The total estimated cost of the endoscopies that have been performed is £5635. Primary GI cancer was diagnosed in 1 case during the endoscopy (1 case of gastric cancer). The 5-year overall survival of the women included in this study is 62.8 %. The 5-year overall survival of the women in the endoscopy and non-endoscopy groups was 60 % and 64.3 % respectively (p-value: 0.767). CONCLUSION: The findings of this study show that the survival rates of patients treated for mucinous ovarian cancer in our centre are similar to other published studies. Our findings do not support the routine use of GI endoscopy in the peri-operative investigations of every patient with MOC due to the non-statistically significant difference in the overall survival.

Synergy of the microRNA Ratio as a Promising Diagnosis Biomarker for Mucinous Borderline and Malignant Ovarian Tumors.

Epithelial ovarian cancers (EOCs) are a heterogeneous collection of malignancies, each with their own developmental origin, clinical behavior and molecular profile. With less than 5% of EOC cases, mucinous ovarian carcinoma is a rare form with a poor prognosis and a 5-year survival of 11% for advanced stages (III/IV). At the early stages, these malignant forms are clinically difficult to distinguish from borderline (15%) and benign (80%) forms with a better prognosis due to the large size and heterogeneity of mucinous tumors. Improving their diagnosis is therefore a challenge with regard to the risk of under-treating a malignant form or of unnecessarily undertaking radical surgical excision. The involvement of microRNAs (miRNAs) in tumor progression and their potential as biomarkers of diagnosis are becoming increasingly recognized. In this study, the comparison of miRNA microarray expression profiles between malignant and borderline tumor FFPE samples identified 10 down-regulated and 5 up-regulated malignant miRNAs, which were validated by individual RT-qPCR. To overcome normalization issues and to improve the accuracy of the results, a ratio analysis combining paired up-regulated and down-regulated miRNAs was performed. Although 21/50 miRNA expression ratios were significantly different between malignant and borderline tumor samples, any ratio could perfectly discriminate the two groups. However, a combination of 14 pairs of miRNA ratios (double ratio) showed high discriminatory potential, with 100% of accuracy in distinguishing malignant and borderline ovarian tumors, which suggests that miRNAs may hold significant clinical potential as a diagnostic tool. In summary, these ratio miRNA-based signatures may help to improve the precision of histological diagnosis, likely to provide a preoperative diagnosis in order to adapt surgical procedures.

Clinical implications of histologic subtypes on survival outcomes in primary mucinous ovarian carcinoma.

OBJECTIVE: In 2014, the World Health Organization introduced a new histologic classification by dividing primary mucinous ovarian carcinoma (PMOC) into two: expansile (ES) or infiltrative subtypes (IS). This study investigated the clinical implications of these histological subtypes on survival outcomes. METHODS: Data from 131 patients with PMOC who underwent primary surgery between 2003 and 2021 were analyzed. The patients baseline characteristics, surgical and pathological information were collected. Survival outcomes were calculated, while factors affecting them were also investigated. RESULTS: During 55.9 months of median follow-up, 27 (20.6%) patients experienced recurrence and 20 (15.3%) died. Among 131 patients, 113 patients were classified into 87 (77%) ES and 26 (23%) IS after a slide review. Advanced stage, lymph node involvement, and residual tumors after surgery were more common in the IS, showing poorer prognosis. In multivariate analyses, advanced stage and residual tumors after surgery were associated with worse survival, while the IS showed no statistical significance. In subgroup analysis for stage I disease, survival did not vary between subtypes. Nevertheless, patients in the IS group who underwent fertility-sparing surgeries demonstrated a 5-year progression-free survival (PFS) rate of 83.3%, significantly lower than patients without fertility preservation, irrespective of histologic subtypes (5-year PFS rate: 97.9%; P = 0.002 for the ES, 5-year PFS rate: 100%; P = 0.001 for the IS). CONCLUSIONS: The IS of PMOC had poorer survival outcomes and a higher proportion of advanced-stage tumors. Although its independent prognostic significance remains uncertain, adjuvant chemotherapy should be considered for patients with fertility preservation in the IS group.

Durable remission in a patient with ERBB2-amplified recurrent mucinous ovarian carcinoma treated with Trastuzumab-Carboplatin-Paclitaxel.

Patients with advanced stage or recurrent mucinous ovarian carcinoma exhibit poor response to standard platinum- and taxane-based chemotherapy and poor prognosis. We report a 29-year-old patient with recurrent ERBB2-amplified mucinous ovarian carcinoma (with expansile growth pattern at initial diagnosis and previously treated with adjuvant capecitabine/oxaliplatin) who underwent optimal secondary cytoreduction followed by 6 cycles of carboplatin/paclitaxel/trastuzumab and 1-year maintenance trastuzumab. This patient remains without radiologic or biochemical evidence of disease for more than 3 years after secondary cytoreduction. This case supports routine assessment of HER2 status in patients with advanced or recurrent mucinous ovarian carcinoma and highlights the potential of HER2-targeted therapy with trastuzumab in combination with standard carboplatin and paclitaxel in this disease. This case also raises the possibility that expansile mucinous ovarian carcinomas with ERBB2 amplification and p53 mutant immunohistochemical staining pattern (as this patient had) may be associated with a more aggressive behavior and higher risk of relapse.

Development and validation of a nomogram to predict cancer-specific survival of mucinous epithelial ovarian cancer after cytoreductive surgery.

BACKGROUND: Mucinous epithelial ovarian cancer (mEOC) is a relatively uncommon subtype of ovarian cancer with special prognostic features, but there is insufficient research in this area. This study aimed to develop a nomogram for the cancer-specific survival (CSS) of mEOC based on Surveillance, Epidemiology, and End Results (SEER) database and externally validate it in National Union of Real World Gynecological Oncology Research and Patient Management (NUWA) platform from China. METHODS: Patients screened from SEER database were allocated into training and internal validation cohort in a ratio of 7: 3, with those from NUWA platform as an external validation cohort. Significant factors selected by Cox proportional hazard regression were applied to establish a nomogram for 3-year and 5-year CSS. The performance of nomogram was assessed by concordance index, calibration curves and Kaplan-Meier (K-M) curves. RESULTS: The training cohort (n = 572) and internal validation cohort (n = 246) were filtered out from SEER database. The external validation cohort contained 186 patients. Baseline age, tumor stage, histopathological grade, lymph node metastasis and residual disease after primary surgery were significant risk factors (p < 0.05) and were included to develop the nomogram. The C-index of nomogram in training, internal validation and external validation cohort were 0.869 (95% confidence interval [CI], 0.838-0.900), 0.839 (95% CI, 0.787-0.891) and 0.800 (95% CI, 0.738-0.862), respectively. The calibration curves of 3-year and 5-year CSS in each cohort showed favorable agreement between prediction and observation. K-M curves of different risk groups displayed great discrimination. CONCLUSION: The discrimination and goodness of fit of the nomogram indicated its satisfactory predictive value for the CSS of mEOC in SEER database and external validation in China, which implies its potential application in different populations.

Entrectinib use in a platinum-refractory mucinous ovarian cancer harboring a NTRK3 gene fusion.

Ovarian cancer is difficult to treat, and the mucinous epithelial subtype has a particularly poor response to traditional chemotherapy regimens. Entrectinib is a tumor-agnostic tyrosine kinase inhibitor with limited data regarding its use in ovarian cancers, though it demonstrates significant tumor response and patient tolerability in other settings. Here we outline what we believe to be the first case in which Entrectinib was successfully utilized to treat a patient with mucinous ovarian cancer. A 51-year-old woman with stage IVB mucinous ovarian cancer possessing a KANK1-NTRK3 gene fusion experienced tumor progression and clinical deterioration with conventional chemotherapeutics. Upon initiation of Entrectinib treatment she experienced rapid clinical improvement, with significant partial response and sustained decrease in tumor markers.

Safety of fertility sparing management in invasive mucinous ovarian carcinoma.

BACKGROUND: The aim of the study was to evaluate the safety of fertility-sparing surgery in invasive mucinous ovarian carcinomas (MOC). METHODS: Retrospective review was performed of MOCs diagnosed between 1999 and 2019 at two tertiary cancer centers. Pathology was reviewed to rule out metastasis from gastrointestinal tract. The demographics and survival outcomes were compared between women who underwent fertility-sparing surgery and those who underwent radical surgery (at least hysterectomy, bilateral salpingo-oophorectomy +/- staging). Cox proportional hazard models were constructed to evaluate the effect of fertility sparing surgery on survival. RESULTS: Of 134 with stage I disease, 42 (31%) underwent fertility-sparing surgery with unilateral salpingo-oophorectomy. Compared to women who underwent radical surgery, these women were younger with low grade, early-stage disease. Two patients (5%) in the fertility-sparing cohort experienced a recurrence and 1 of these 2 patients died due to disease progression. There was no difference in either OS or RFS between those that underwent fertility-sparing surgery and radical surgery. In a multivariable analysis adjusting for age and use of adjuvant chemotherapy, fertility-sparing surgery was not significantly associated with OS (HR 0.18; 95% CI 0.01-2.78) or RFS (HR 0.19; 95% CI 0.03-1.45). There were 4 patients (9%) with documented full-term delivery with median interval to conception of 11 months. CONCLUSIONS: Fertility-sparing surgery in stage I MOC is not associated with worse outcomes compared to radical surgery and is reasonable to offer to those with early stage disease who wish to retain fertility.

Characterization of Primary Mucinous Ovarian Cancer by Diffusion-Weighted and Dynamic Contrast Enhancement MRI in Comparison with Serous Ovarian Cancer.

(1) Background. The purpose of this study is to evaluate the diagnostic accuracy of a quantitative analysis of diffusion-weighted imaging (DWI) and dynamic contrast enhanced (DCE) MRI of mucinous ovarian cancer (MOC). It also aims to differentiate between low grade serous carcinoma (LGSC), high-grade serous carcinoma (HGSC) and MOC in primary tumors. (2) Materials and Methods. Sixty-six patients with histologically confirmed primary epithelial ovarian cancer (EOC) were included in the study. Patients were divided into three groups: MOC, LGSC and HGSC. In the preoperative DWI and DCE MRI, selected parameters were measured: apparent diffusion coefficients (ADC), time to peak (TTP), and perfusion maximum enhancement (Perf. Max. En.). ROI comprised a small circle placed in the solid part of the primary tumor. The Shapiro-Wilk test was used to test whether the variable had a normal distribution. The Kruskal-Wallis ANOVA test was used to determine the p-value needed to compare the median values of interval variables. (3) Results. The highest median ADC values were found in MOC, followed by LGSC, and the lowest in HGSC. All differences were statistically significant (p < 0.000001). This was also confirmed by the ROC curve analysis for MOC and HGSC, showing that ADC had excellent diagnostic accuracy in differentiating between MOC and HGSC (p < 0.001). In the type I EOCs, i.e., MOC and LGSC, ADC has less differential value (p = 0.032), and TTP can be considered the most valuable parameter for diagnostic accuracy (p < 0.001). (4) Conclusions. DWI and DCE appear to be very good diagnostic tools in differentiating between serous carcinomas (LGSC, HGSC) and MOC. Significant differences in median ADC values between MOC and LGSC compared with those between MOC and HGSC indicate the usefulness of DWI in differentiating between less and more aggressive types of EOC, not only among the most common serous carcinomas. ROC curve analysis showed that ADC had excellent diagnostic accuracy in differentiating between MOC and HGSC. In contrast, TTP showed the greatest value for differentiating between LGSC and MOC.

Evaluation of Outcomes of Mucinous Ovarian Cancer Treated at a Tertiary Care Cancer Hospital in Pakistan.

Syed Abdul Mannan HamdaniObjective To evaluate the clinicopathological features and survival outcomes of mucinous ovarian cancer (MOC) patients in an Asian population. Study Design Descriptive observational study. Place and Duration of Study Shaukat Khanum Memorial Cancer Hospital, Lahore, Pakistan, from January 2001 to December 2016. Methods Data of MOC were evaluated for demographics, tumor stage, clinical characteristics, tumor markers, treatment modalities, and outcomes from electronic Hospital Information System. Results Nine-hundred patients with primary ovarian cancer were reviewed, out of which 94 patients (10.4%) had MOC. The median age was 36 ± 12.4 years. The most common presentation was abdominal distension 51 (54.3%), while the rest presented with abdominal pain and irregular menstruation. Using FIGO (The International Federation of Gynecology and Obstetrics) staging, 72 (76.6%) had stage I, 3 (3.2%) stage II, stage III in 12 (12.8%), and 7 (7.4%) had stage IV disease. The majority of patients 75 (79.8%) had early-stage (stage I/II), while 19 (20.2%) presented with advanced-stage (III & IV). The median follow-up duration was 52 months (range 1-199 months). Among patients with early-stage (I&II), 3- and 5-year progression-free survival (PFS) was 95%, while for advanced stage (III&IV), PFS was 16% and 8%, respectively. The overall survival (OS) in early-stage I&II was 97%, while for advanced stages III & IV, the OS was 26%. Conclusion MOC is a challenging and rare subtype of ovarian cancer requiring special attention and recognition. Most patients treated at our center presented with early stages and had excellent outcomes, while advanced-stage disease had dismal results.

Update on Prognostic and Predictive Markers in Mucinous Ovarian Cancer.

This review includes state-of-the-art prognostic and predictive factors of mucinous ovarian cancer (MOC), a rare tumor. Clinical, pathological, and molecular features and treatment options according to prognosis are comprehensively discussed. Different clinical implications of MOC are described according to the The International Federation of Gynecology and Obstetrics (FIGO) stage: early MOC (stage I-II) and advanced MOC (stage III-IV). Early MOC is characterized by a good prognosis. Surgery is the mainstay of treatment. Fertility-sparing surgery could be performed in patients who wish to become pregnant and that present low recurrence risk of disease. Adjuvant chemotherapy is not recommended, except in patients with high-risk clinical and pathological features. Regarding the histological features, an infiltrative growth pattern is the major prognostic factor of MOC. Furthermore, novel molecular biomarkers are emerging for tailored management of early-stage MOC. In contrast, advanced MOC is characterized by poor survival. Radical surgery is the cornerstone of treatment and adjuvant chemotherapy is recommended, although the efficacy is limited by the intrinsic chemoresistance of these tumors. Several molecular hallmarks of advanced MOC have been described in recent years (e.g., HER2 amplification, distinct methylation profiles, peculiar immunological microenvironment), but target therapy for these rare tumors is not available yet.

Disparity in the era of personalized medicine for epithelial ovarian cancer.

The treatment of high-grade serous ovarian cancer and high-grade endometrioid ovarian cancer has seen significant improvements in recent years, with BRCA1/2 and homologous recombination status guiding a personalized approach which has resulted in improved patient outcomes. However, for other epithelial ovarian cancer subtypes, first-line treatment remains unchanged from the platinum-paclitaxel trials of the early 2000s. In this review, we explore novel therapeutic approaches being adopted in the treatment of clear cell, mucinous, carcinosarcoma and low-grade serous ovarian cancer and the biological rational behind them. We discuss why such disparities exist, the challenges faced in conducting dedicated trials in these rarer histologies and look towards new approaches being adopted to overcome them.

Molecular characteristics and clinical behaviour of epithelial ovarian cancers.

Ovarian carcinoma (OC) is an umbrella term for multiple distinct diseases (histotypes), each with their own developmental origins, clinical behaviour and molecular profile. Accordingly, OC management is progressing away from a one-size-fits all approach, toward more molecularly-driven, histotype-specific management strategies. Our knowledge of driver events in high grade serous OC, the most common histotype, has led to major advances in treatments, including PARP inhibitor use. However, these agents are not suitable for all patients, most notably for many of those with rare OC histotypes. Identification of additional targeted therapeutic strategies will require a detailed understanding of the molecular landscape in each OC histotype. Until recently, tumour profiling studies in rare histotypes were sparse; however, significant advances have been made over the last decade. In particular, reports of genomic characterisation in endometrioid, clear cell, mucinous and low grade serous OC have significantly expanded our understanding of mutational events in these tumour types. Nonetheless, substantial knowledge gaps remain. This review summarises our current understanding of each histotype, highlighting recent advances in these unique diseases and outlining immediate research priorities for accelerating progress toward improving patient outcomes.

The effect of chemotherapy in patients with stage I mucinous ovarian cancer undergoing fertility-sparing surgery.

Objective: To determine the effect of adjuvant chemotherapy in patients with stage I mucinous ovarian cancer (MOC) undergoing fertility-preserving surgery. Patients and methods: The clinicopathological characteristics and survival information of young women with stage I MOC from SEER databases between 2004 and 2019 were collected. The relationship between chemotherapy and the characteristics was examined by univariate and multivariate logistic regression analyses. Univariable and multivariate Cox proportional hazards survival analysis were employed for cancer-specific survival. Cox analysis was performed to build a nomogram model. Results: All 901 eligible patients with stage I MOC were screened from the SEER database. There were 321(35.6%) patients aged 9-30 years, 580(64.4%) aged 31-45 years, 645 (71.6%) patients with stage IA/IB, 256 (28.4%) with stage IC disease, 411(45.6%) who underwent fertility-sparing surgery, and276(30.6%) who received postoperative adjuvant chemotherapy. Multivariate logistic regression analyses showed that postoperative chemotherapy was often used in patients aged 31-45 relative to aged 9-30 (HR: 2.215, 95%CI 1.443-3.401, P < 0.001) or with grade 3 compared to grade 1 tumors (HR: 7.382, 95%CI 4.054-13.443, P < 0.001) or with stage IC compared to stage IA/IB (HR: 6.436, 95%CI 4.515-9.175, P < 0.001) or with non-fertility sparing surgery relative to fertility-sparing (HR:2.226, 95%CI 1.490-3.327, P < 0.001). Multivariate analysis for the special population with fertility preservation indicated that patients with chemotherapy (HR: 2.905, 95% CI: 0.938-6.030, P=0.068) or with grade 3 (HR: 4.750, 95% CI: 1.419-15.896, P=0.011) had a greater risk of mortality. Significant CSS differences were observed between the non-chemotherapy and chemotherapy groups in MOC when patients were stage IA/IB-grade 2 (P=0.004) (10-year CSS rates of chemotherapy=84%, non-chemotherapy = 100%), but not when they were stage IA/IB-grade 1, stage IA/IB-grade 3 or stage IC (both P>0.05). A prognostic prediction nomogram model was built for stage I MOC patient who underwent fertility-sparing and the C-index was 0.709. Discussion: The patients aged 31-45 years, with grade 3, stage IC, and non-fertility-sparing surgery were more likely to receive adjuvant chemotherapy in the real world. For stage I MOC patient who underwent fertility-sparing surgery, the choice of chemotherapy may increase the risk of death, and it should be carefully selected in clinical practice.

A Nomogram Based on SEER Database for Predicting Prognosis in Patients with Mucinous Ovarian Cancer: A Real-World Study.

Purpose: Mucinous ovarian cancer (MOC) is a rare histological type of EOC. In order to guide the clinical diagnosis and management of MOC patients, we constructed and verified a nomogram for the estimation of overall survival in patients with MOC. Patients and Methods: We collected 494 patients with MOC diagnosed from 2010 to 2015 in SEER database, and the following main inclusion criteria were used: (1) patients whose MOC was confirmed by pathology; (2) patients without a history of primary other cancer. Subsequently, we performed randomized grouping (6:4) and Cox hazard regression analysis in the training group. Subsequently, the nomogram was established. A variety of indicators were used to validate the prognosis value of nomogram, including the C-index, area under the receiver operating characteristic curve, calibration curve, and decision curve analysis (DCA). Moreover, Kaplan-Meier analysis was used to compare the survival results among different risk subgroups. Results: Cox hazard regression analysis revealed that age, grade, FIGO stage and log odds of positive lymph nodes stage were independent risk factors for patients with MOC. In the training group, the C-index of the nomogram was 0.827 (95% CI: 0.791-0.863) and the areas under the curve (AUC) predicting the 1-, 3- and 5-year survival rate were 0.853 (95% CI: 0.791-0.915), 0.886 (95% CI: 0.852-0.920) and 0.815 (95% CI: 0.766-0.864), respectively. The calibration curve revealed that the nomogram of the 1-, 3- and 5-year survival rate was consistent with the actual fact. Patients with high risk had a poorer prognosis than those with low risk (P < 0.001). DCA revealed that the nomogram had the best clinical value than other classical prognostic markers. Similarly, nomogram had excellent prognostic ability in the testing group. Conclusion: The nomogram was constructed to predict overall survival in patients with MOC, which had the significance for clinical evaluation.

Nomograms for primary mucinous ovarian cancer: A SEER population-based study.

BACKGROUND: To develop predictive nomograms of overall survival (OS) and cancer-specific survival (CSS) in patients with primary mucinous ovarian cancer (PMOC). METHODS: Patients diagnosed with PMOC from 2010 to 2015 were obtained from the Surveillance, Epidemiology, and End Results (SEER) database, and randomly divided into a training cohort and a validation cohort. Univariate and multivariate Cox regression analyses were conducted to identify the independent risk factors. Nomograms were constructed and then verified by calibration plots, the concordance index (C-index), and the area under the receiver operating characteristic curve (AUC). RESULTS: A total of 991 patients with PMOC were enrolled and randomly divided into a training cohort (n=695) and a validation cohort (n=296) at a ratio of 7:3. Multivariate Cox regression analyses demonstrated that independent risk factors for OS included age, laterality, and American Joint Committee on Cancer (AJCC) stage. Independent risk factors for CSS included age, laterality, grade, and AJCC stage. Predictive nomograms for OS and CSS were developed with respective independent risk variables. In the training cohort, the C-index of the CSS and OS nomograms were 0.88 [95% confidence interval (CI): 0.84-0.92] and 0.85 (95% CI: 0.81-0.89), respectively. In the validation cohort, the C-index of the predictive CSS and OS nomograms were 0.86 (95% CI: 0.80-0.92) and 0.80 (95% CI: 0.74-0.87), respectively. The AUCs were higher in both cohorts. Furthermore, the calibration curves in both cohorts showed good consistency between the predicted results and the actual results. CONCLUSION: The nomograms demonstrated good predictability for the survival of patients with PMOC, and could serve as an applicable tool to help clinicians improve treatment plans.

A Rare Presentation of Dual Ovarian Pathologies: Small Cell Carcinoma of the Ovary and Mucinous Ovarian Cancer.

The small cell carcinoma of ovaries co-occurring with mucinous ovarian cancer is a rare event. We report a 21-years-old lady with a composite tumour comprising small cell carcinoma and mucinous carcinoma of ovaries. The incidental finding of the left ovarian cyst led to further workup and revealed a solid cystic mass in the left adnexal area pathologically proven to be mucinous ovarian carcinoma. The initial surgery was deferred upon the patient's request. After a few more cycles of chemotherapy, at the completion of surgery as per ovarian protocol, the pathological evaluation showed small cell carcinoma in the left ovary with a residual focus of mucinous carcinoma. In contrast, the right ovary also showed surface deposits of small cell carcinoma. The patient's clinical condition deteriorated very rapidly after that, and she passed away. Early recognition of small cell carcinoma in a composite tumour is critically essential for timely intervention.

The Protein Landscape of Mucinous Ovarian Cancer: Towards a Theranostic.

MOC is a rare histotype of epithelial ovarian cancer, and current management options are inadequate for the treatment of late stage or recurrent disease. A shift towards personalised medicines in ovarian cancer is being observed, with trials targeting specific molecular pathways, however, MOC lags due to its rarity. Theranostics is a rapidly evolving category of personalised medicine, encompassing both a diagnostic and therapeutic approach by recognising targets that are expressed highly in tumour tissue in order to deliver a therapeutic payload. The present review evaluates the protein landscape of MOC in recent immunohistochemical- and proteomic-based research, aiming to identify potential candidates for theranostic application. Fourteen proteins were selected based on cell membrane localisation: HER2, EGFR, FOLR1, RAC1, GPR158, CEACAM6, MUC16, PD-L1, NHE1, CEACAM5, MUC1, ACE2, GP2, and PTPRH. Optimal proteins to target using theranostic agents must exhibit high membrane expression on cancerous tissue with low expression on healthy tissue to afford improved disease outcomes with minimal off-target effects and toxicities. We provide guidelines to consider in the selection of a theranostic target for MOC and suggest future directions in evaluating the results of this review.