The aim of this study was to assess the disease-free survival (DFS) and overall survival (OS) of patients with grade 1-2 endometrioid ovarian carcinoma apparently confined to the ovary, according to surgical staging. Multicenter, retrospective, observational cohort study. Patients with endometrioid ovarian carcinoma, surgical procedure performed between May 1985 and December 2019, stage pT1 N0/N1/Nx, grade 1-2 were included. Patients were stratified according to lymphadenectomy (defined as removal of any lymph node versus no lymph node assessment), and subgroup analyses according to tumor grade were performed. Kaplan-Meier curves and cox regression analyses were used to perform survival analyses. 298 patients were included. 199 (66.8 %) patients underwent lymph node assessment. Of these, 166 (83.4 %) had unilateral/bilateral pelvic and para-aortic/caval lymphadenectomy. Eleven (5.5 %) patients of those who underwent lymph node assessment showed pathologic metastatic lymph nodes (FIGO stage IIIA1). Twenty-seven patients (9.1 %) had synchronous endometrioid endometrial cancer. After a median follow up of 45 months (95 %CI:37.5-52.5), 5-year DFS and OS of the entire cohort were 89.8 % and 96.2 %, respectively. Age ≤ 51 years (HR=0.24, 95 %CI:0.06-0.91; p = 0.036) and performance of lymphadenectomy (HR=0.25, 95 %CI: 0.07-0.82; p = 0.022) represented independent protective factors toward risk of death. Patients undergoing lymphadenectomy had better 5-year DFS and OS compared to those not receiving lymphadenectomy, 92.0 % versus 85.6 % (p = 0.016) and 97.7 % versus 92.8 % (p = 0.013), respectively. This result was confirmed after exclusion of node-positive patients. When stratifying according to tumor grade (node-positive excluded), patients with grade 2 who underwent lymphadenectomy had better 5-year DFS and OS than those without lymphadenectomy (93.0 % versus 83.1 %, p = 0.040 % and 96.5 % versus 90.6 %, p = 0.037, respectively). Staging lymphadenectomy in grade 2 endometrioid ovarian carcinoma patients was associated with improved DFS and OS. Grade 1 and grade 2 might be considered as two different entities, which could benefit from different approach in terms of surgical staging. Prospective studies, including molecular profiles are needed to confirm the survival drivers in this rare setting.

Background: Health-related quality of life (HRQoL) is an important patient-reported outcome for all cancer patients, including adult patients with rare solid cancers. However, current knowledge of HRQoL in this population is limited, which hinders the delivery of personalized care. This review aimed to explore the heterogeneity of HRQoL among adult patients with a solid rare cancer across the ten European Reference Network for Rare Adult Solid Cancers (EURACAN) domains and to summarize the HRQoL measures used in clinical research. Methods: A systematic literature search was conducted to identify all clinical studies assessing HRQoL in adult patients with a solid rare cancer. Four databases (MEDLINE, PubMed, PsycINFO, and Web of Science/Scopus) were searched (February 2023). Results: The search yielded 18,704 articles, of which 1416 articles were fully screened and 463 were eligible for analysis. Of these, 397 studies used generic tools to assess HRQoL, while 270 used tumor-specific instruments. Three EURACAN domains (sarcoma, endocrine tumors, and thoracic tumors) primarily assessed HRQoL using generic questionnaires. Additionally, the rare gynecological tumor, rare male genitourinary, and sarcoma EURACAN domains lacked specific HRQoL measures. Brain, head and neck, and uveal melanoma EURACAN domains used tumor- or domain-specific questionnaires in more than half of the studies. Conclusions: This state-of-the-art literature review shows that HRQoL assessment is gradually becoming more prevalent in adult solid rare cancer research. A combination of generic, tumor-specific, and domain-specific questionnaires across various rare cancer domains has proven effective in capturing a broad range of HRQoL issues. However, many EURACAN domains still lack specific strategies for assessing HRQoL, which limits the ability to fully understand and address patients’ experiences. Future research should prioritize developing comprehensive and robust HRQoL measurement strategies and tools to enable meaningful clinical research and to ensure that the patient voice is incorporated in their clinical care.

Background: Vulvar dermatofibrosarcoma protuberans is an extremely rare disease. Its rarity can hamper the quality of treatment; deeper knowledge is necessary to plan appropriate management. The purpose of this review is to analyse the data reported in the literature to obtain evidence regarding appropriate disease management. Methods: We made a systematic search of the literature, including the terms “dermatofibrosarcoma protuberans”, “vulva”, and “vulvar”, alone or in combination. We selected articles published in English from two electronic databases, PubMed and MEDLINE, and we analysed their reference lists to include other potentially relevant studies. Results: We selected 39 articles, with a total of 68 cases reported; they were retrospective case reports and case series. Dermatofibrosarcoma protuberans of the vulva tends towards local recurrence; an early and timely pathological diagnosis, together with an appropriate surgical approach, are of utmost importance to ensure free margins and maximise the curative potential. Conclusions: Even if this is an indolent disease and it generally shows a good prognosis, appropriate management may help in reducing the rate of local recurrences that may hamper patients’ quality of life. Management by a multidisciplinary team is highly recommended.

Objective: The purpose of the study was to analyse the role of prognostic factors on the risk of recurrence and overall survival of patients with uterine adenosarcoma.Methods: A retrospective international multicentre study involving 46 centres collected 32 cases of uterine adenosarcoma, and these cases were included in the present subanalysis. Clinical and demographic features and tumour characteristics were gathered, as well as information on treatment and relapse. Disease-free and overall survival were analysed.Results: The 5-year disease-free survival (DFS) was 85.3% and the 5-year overall survival (OS) rate was 89.5%. The risk factors significantly associated with overall survival were age (HR 1.09, 95% CI 1.03–1.15; p = 0.004) and FIGO stage II–III (HR 17.75, 95% CI 2.87–109.93; p = 0.002). Patients who experienced early relapse (within 12 months) had a tumour size >30 mm and advanced stage. The majority of recurred cases were treated with radiotherapy or surgery and obtained a good response rate.Conclusion: The most significant prognostic factors in uterine adenosarcoma were age and FIGO stage and, indirectly, tumour size at diagnosis. The use of secondary surgery and/or radiotherapy could help in prolonging the disease-free status of the patients.

Background: The aim of this study was to assess the impact of prognostic factors on the survival of patients diagnosed with uterine carcinosarcoma. Methods: A sub-analysis of the SARCUT study, a multicentric retrospective European study, was carried out. We selected 283 cases of diagnosed uterine carcinosarcoma for the present study. Prognosis factors influencing survival were analyzed. Results: Significant prognostic factors for overall survival were: incomplete cytoreduction (HR = 4.02; 95%CI = 2.68–6.18), FIGO stages III and IV (HR = 3.21; 95%CI = 1.83–5.61), tumor persistence after any treatment (HR = 2.90; 95%CI = 1.97–4.27), presence of extrauterine disease (HR = 2.62; 95%CI = 1.75–3.92), a positive resection margin (HR = 1.56; 95%CI = 1.05–2.34), age (HR = 1.02; 95%CI = 1.00–1.05), and tumor size (HR = 1.01; 95%CI = 1.00–1.01). Significant prognostic factors for disease-free survival were: incomplete cytoreduction (HR = 3.00; 95%CI = 1.67–5.37), tumor persistence after any treatment (HR = 2.64; 95%CI = 1.81–3.86), FIGO stages III and IV (HR = 2.33; 95%CI = 1.59–3.41), presence of extrauterine disease (HR = 2.13; 95%CI = 1.44–3.17), administration of adjuvant chemotherapy (HR = 1.84; 95%CI = 1.27–2.67), a positive resection margin (HR = 1.65; 95%CI = 1.11–2.44), presence of LVSI (HR = 1.61; 95%CI = 1.02–2.55), and tumor size (HR = 1.00; 95%CI = 1.00–1.01). Conclusions: Incomplete cytoreduction, presence of tumor residual after treatment, advanced FIGO stage, extrauterine disease, and tumor size are significant prognostic factors decreasing disease-free survival and overall survival of patients with uterine carcinosarcoma.

Objective: To develop machine‐learning models that incorporate clinical information and radiomics features extracted from ultrasound images to distinguish uterine sarcomas from leiomyomas. Methods: This retrospective, multicenter, pilot case–control study included 200 patients (100 with a uterine sarcoma and 100 with a usual‐type leiomyoma, i.e. including no benign leiomyoma variants) who underwent preoperative ultrasound examination between January 2010 and June 2022. The patient cohort was split (70:30) into training and validation sets, with the same proportion of leiomyomas and sarcomas in each subset. We extracted radiomics features belonging to different families: intensity‐based statistical features and textural features. The variables used in model building were patient age and the radiomics features that differed statistically significantly between sarcomas and leiomyomas and that were not redundant based on Spearman's correlation coefficient. Logistic regression, random forest, extreme gradient boosting (XGBoost) and support vector machine models were tested in the model development process. We evaluated the performance of the models in differentiating between sarcomas and leiomyomas using the area under the receiver‐operating‐characteristics curve (AUC), accuracy, sensitivity and specificity. We compared these results to those of subjective assessment by the original ultrasound examiner and to those of two independent expert ultrasound examiners who, blinded to clinical history, reviewed the same grayscale ultrasound images as those used for the radiomics analysis. Results: Sixty‐three radiomics features were extracted. Of these, eight differed statistically significantly between sarcomas and leiomyomas and were not correlated, so were selected for inclusion in model building. In the validation set, the model that performed best in differentiating between sarcomas and leiomyomas was an XGBoost model integrating patient age and radiomics features. In the validation set, this model had an AUC of 0.93, sensitivity of 0.93 and specificity of 0.83, at a risk‐of‐malignancy cut‐off of 47% (the cut‐off that yielded the highest number of correct classifications based on Youden's index in the training set). The corresponding results for the model integrating only the radiomics features were: AUC of 0.87, sensitivity of 0.87 and specificity of 0.83. Subjective assessment by the original ultrasound examiner had a sensitivity of 0.87 and specificity of 1 in the validation set, while retrospective review of grayscale ultrasound images by ultrasound experts had a sensitivity of 0.87 and specificity of 0.80 (same results for both reviewers). Conclusion: A model including eight radiomics features and patient age demonstrated reasonably good discriminative and classification performance for distinguishing uterine sarcomas from leiomyomas. Its classification ability was similar to that of subjective assessment by the original ultrasound examiner, being more sensitive but less specific. To confirm the role of radiomics for discriminating between uterine sarcomas and leiomyomas, large prospective studies including benign leiomyoma variants are needed. If good performance of radiomics models can be confirmed, integrating automated radiomics analysis into ultrasound machine software may help ultrasound examiners to discriminate between sarcomas and benign leiomyomas. © 2026 The Author(s). Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.