tbuckley/cabot-search huggingface dataset:
Progress in the management of primary and metastatic cardiac sarcoma: Advancing multidisciplinary care in an academic sarcoma center.
Authors: Alejandra Cardona Perez, Suparna Chandra Clasen, Bharathi Muthusamy, Anna Roshal, Samantha Ann Armstrong
Journal: Journal of Clinical Oncology (2025)

e23568 Background: Cardiac sarcomas are rare tumors that are usually aggressive and associated with poor prognosis. While the optimal treatment strategy for cardiac sarcomas has yet to be established, a multidisciplinary approach including surgery, radiation and systemic therapies are utilized. We aimed to investigate the clinical characteristics, prognosis of cardiac sarcomas and the evolving treatment paradigms in this cohort. Methods: Pts diagnosed with primary and metastatic cardiac sarcomas were identified between January 2016 and 2025 at the Indiana University Cancer Center. Data was extracted from electronic medical records, including demographics, pathology, molecular testing, treatment and outcomes. Results: Eleven pts (5 female) were identified, with a median age at diagnosis of 38 years (range: 27-74). Dyspnea and chest pain were the most common presenting symptoms. The predominant histological subtypes were angiosarcomas of the right atrium and intimal sarcomas located in the left atrium or the left pulmonary artery. Other histopathologies included rhabdomyosarcoma, undifferentiated pleomorphic sarcoma, myxoid liposarcoma and chordoma. Six pts underwent upfront surgical resection of cardiac sarcomas: 4 with local disease underwent attempted curative surgery, but all had recurrence at a median time of 23.5 months (range: 5-80 months). Two pts with metastatic disease underwent definitive resection of both the cardiac tumor and all macroscopic disease, with 1 patient recurring at 52 months and the second remaining with no evidence of disease. Most pts (n = 5) required subsequent resection of primary cardiac recurrence and/or macroscopic disease, with 1 patient undergoing three sternotomies. Of the cohort, 10 pts received systemic therapy. Six pts received historical standard adjuvant doxorubicin based-chemotherapy, of which 5 subsequently received additional systemic treatment with an average of 3 lines of therapy (range: 1-7), including novel combinations of immunotherapy (n = 5) and targeted therapy (n = 4). Two pts achieved a complete response with surgery and adjuvant chemotherapy. One patient with metastatic primary cardiac intimal sarcoma had a complete response to immunotherapy. The median follow-up was 24 months (range: 3-120). Three pts died due to disease progression while four pts are alive with stable disease, three pts are alive with no evidence of disease, and one patient is alive with disease progression. Conclusions: Better outcomes were observed in those who underwent surgery and systemic therapy, findings that are consistent with previous studies. These findings underscore the complexity of managing cardiac sarcomas and the critical role of multidisciplinary care. Multi-institutional studies are needed to further elucidate comprehensive treatment strategies and biology-driven therapeutic regimens for these rare tumors.
Survival of malignant peripheral nerve sheath tumor of the trachea, mediastinum, and other respiratory organs: Analysis of SEER database.
Authors: Nora Sun, Jerry Wu, Faye Yin
Journal: Journal of Clinical Oncology (2025)

e23550 Background: Malignant peripheral nerve sheath tumor (MPNST) is a rare form of sarcoma arising from Schwann cells with an incidence rate of about 1 case per 100,000 people. Most MPNSTs occur in the extremities; MPNST of respiratory organs is exceedingly rare and has only been reported in case studies previously, with little being known about its incidence and treatment outcomes. Methods: Patients with MPNST of the “trachea, mediastinum and other respiratory organs” were identified from the Surveillance, Epidemiology, and End Results (SEER) database (2000-2020) using SEER*Stat. Patient demographics, tumor stage, treatment, and survival data were extracted. Survival outcomes were compared by stage, chemotherapy (any chemotherapy vs. none/unknown), radiation (beam radiation vs. no/unknown), and surgery (any surgery vs. none). Results: 43 patients (mean age: 44.7 ± 18.7) with MPNST of the trachea, mediastinum and other respiratory organs were extracted. Most patients (27; 63%) were male. Most patients were white (34; 79%), followed by black (5; 12%) and other races (4; 9.3%). Earlier tumor stages (p=0.04) and receiving surgery (p=0.02) were significantly associated with longer median survival (Table). Not receiving chemotherapy and receiving beam radiation also appeared to be associated with longer median survival, although these were not statistically significant. Conclusions: This study identified that tumor stage and receiving surgery are associated with improved survival outcomes among patients with MPNST of the trachea, mediastinum and other respiratory organs. The poor survival associated with distant metastasis and lack of surgical intervention highlight the need for novel systemic therapeutics and alternative local intervention options to surgery given the location of the disease which is often unresectable. Median survival of MPNST based on stage and treatment. Number of cases (%) Median survival (months) p-value Stage (n=34) LocalizedN=15 (44%) 30 p=0.04 RegionalN=10 (29%) 10.5 DistantN=9 (26%) 4 Chemotherapy (n=37) YesN=15 (41%) 10 p=0.2 None/unknownN=22 (59%) 20 Radiation (n=38) Beam radiationN=20 (53%) 13.5 p=0.3 None/unknownN=18 (47%) 12 Surgery (n=38) YesN=26 (68%) 20 p=0.02 NoneN=12 (32%) 4.5
Hot flashes and night sweats in women with breast cancer: Prevalence and severity of symptoms.
Authors: Long Lin, Liang Xu, Jie Liu, Ying Wang, Hongyu Zhuo, Qing Qin, Jie Zhang, Ye Chen, Hongmei Xiao, Wuning Zhong, Yu Jiang, Yaotiao Deng
Journal: Journal of Clinical Oncology (2025)

12053 Background: Hot flashes(HF) and night sweats(NS) are common endocrine-related symptoms experienced by breast cancer patients, which potentially arise from the tumor or treatment-related pharmacologic effects. They can significantly diminish patients' quality of life. However, there is a paucity of studies in China investigating the incidence and characteristics of HF and NS among patients with breast cancer. Methods: This study was a multi-center, cross-sectional survey that included women diagnosed with breast cancer from seven hospitals' oncology departments in China. Information was collected about demographic characteristics, psychological status (the Hospital Anxiety and Depression Scale and the Distress Thermometer), the frequency and severity of HF and NS(modified Hot Flash and Night Sweat scale), and patients' perceptions of these symptoms. Group differences based on the presence or absence of HF and NS were analyzed using t-tests or chi-square tests. Finally, univariate analyses was conducted to explore factors associated with the frequency of HF and NS. Results: Between October and November 2024, 960 questionnaires were distributed, and 767 Chinese female breast cancer patients(aged 25 to 79 ) replied and completed fully. Over 56% (n = 430) patients reported experiencing HF or NS.The average weekly frequency was 8.6 times for HF and 7.3 times for NS. Patients attributed HF and NS primarily to treatment (HF:36.8%;NS:32.7%) or uncertain causes (HF:15.8%;NS:17.4%). Compared to patients without HF and NS, those experiencing the symptoms had significantly higher levels of anxiety (p &lt; 0.001), depression (p = 0.019), and psychological distress (p &lt; 0.001). Notably, Patients reported severity, distress, and impact on life scores above 4, with HF at 47.90%, 37.46%, and 35.89%, and NS at 49.67%, 43.46%, and 40.85%, respectively. Among those affected, 59.24% of patients with HF and 63.7% of patients with NS adopted coping strategies, primarily resting/lying down (HF:27.7%;NS:36.3%). Regarding coping ability, HF group had a significant higher score than NS group (HF: 5.90; NS: 5.30, p = 0.012). Furthermore, univariate analysis revealed that HF and NS were associated with distress(HF:p = 0.032; NS:p = 0.030) and sleep quality(HF:p = 0.031; NS: p = 0.043). Additionally, for HF, single marital status (p = 0.017) was identified as a relevant factor, while stage IV tumor status (p = 0.044) was associated with NS. Conclusions: HF and NS affect over half of Chinese breast cancer patients, impacting their mental well-being and life quality, while low coping ability and limited coping strategies further underscore the need for healthcare professionals to pay closer attention to patients experiencing these symptoms, particularly single individuals and those with advanced-stage breast cancer.
Systemic therapy for advanced epithelioid sarcoma: A meta-analysis of effectiveness and safety study.
Authors: Manaswini Krishnakumar, Maha Zafar, Aswanth Reddy
Journal: Journal of Clinical Oncology (2025)

e23558 Background: Epithelioid sarcoma (ES) is a rare and aggressive type of soft tissue sarcoma (STS) with limited evidence available on systemic therapies. Due to the rarity of the disease, high-level evidence for guiding systemic therapy is minimal, and treatment approaches are often based on data from broader studies on STS. This meta-analysis examines the efficacy and safety of chemotherapy, targeted therapy, and immunotherapy in the management of ES. Methods: This meta-analysis adhered to PRISMA guidelines. A systematic review of PubMed, Embase, and Cochrane Library was conducted to identify studies reporting systemic therapy outcomes in ES with a search cutoff until December 2024. Data on overall response rate (ORR), progression-free survival (PFS), overall survival (OS), and adverse events (AEs) were analyzed and documented. Keywords included "epithelioid sarcoma," "systemic therapy," "chemotherapy," "targeted therapy," and "immunotherapy." Results: Twenty-two studies involving 340 ES patients were included. Doxorubicin-based regimens (gemcitabine, ifosfamide) showed an ORR of 10–20%, median PFS of 3–6 months, and median OS of 9–12 months. Combination regimens improved ORR (20–30%) but with increased toxicity. Grade 3/4 toxicities in 45% of patients, primarily hematologic (neutropenia, anemia). Tazemetostat (EZH2 inhibitor) demonstrated an ORR of approximately 15%, disease control rate of approximately 40%, median PFS of 5.5 months, and OS of 12–14 months in patients with SMARCB1/INI1 loss, with minimal grade 3–4 AEs (fatigue and nausea). Immunotherapy (anti-PD-1/PD-L1) yielded ORRs of approximately 15%, with durable responses in select patients with PDL1 &gt; 1%. Data on Pazopainb is limited with one study reporting PFS of 5.5 months without any documented OS. Conclusions: The results highlight the limited efficacy of conventional chemotherapy in epithelioid sarcoma, with modest response rates and survival outcomes. Targeted therapies, such as tazemetostat, offer a promising alternative for patients with specific molecular profiles. Immune checkpoint inhibitors are emerging as effective options, particularly in PD-L1-positive cases, underscoring the need for biomarker-driven treatment strategies. mTOR and EGFR inhibiting treatment are under investigation and other combination strategies are under investigation. Despite these advances, challenges remain, including resistance mechanisms, limited durable responses in unselected populations, and the lack of large randomized trials. Future research should focus on novel combination strategies and further exploration of biomarkers to refine patient selection.
Adjuvant therapy in small intestinal leiomyosarcoma: A comparative analysis of outcomes.
Authors: Nicholas Wingate Morgenstern
Journal: Journal of Clinical Oncology (2025)

e23556 Background: Leiomyosarcoma of the small intestine is a rare gastrointestinal cancer that has been sparsely studied compared to other intraabdominal cancers. It is a malignant tumor of smooth muscle and typically grows into the subserosal layer of the small intestine. This progression of growth away from the lumen often delays presentation until the disease has progressed significantly. Although surgical resection remains the mainstay of treatment, adjuvant treatment such as chemotherapy, radiation therapy, and combined chemo-radiation therapy continue to have use clinically. However, a comprehensive analysis comparing the efficacy of these different therapies for is lacking. This study aims to address this gap by evaluating the differences among these treatment modalities. Methods: The National Cancer Database (NCDB) was used to identify patients diagnosed with Leiomyosarcoma from 2004 to 2019 using the histology code 8890 as assigned by the Commission on Cancer Accreditation program. Kaplan-Meier, ANOVA Chi-Square, and Cox Proportional Hazards tests were performed. Data was analyzed using SPSS version 27 and statistical significance was set at α = 0.05. Results: In a sample size of 81 patients diagnosed with small intestine leiomyosarcoma, 66 (81.5%) patients received adjuvant chemotherapy (AC), 8 (9.9%) patients received adjuvant radiation therapy (AR), and 7 (8.6%) patients received adjuvant combined chemotherapy and radiation. AC patients had a shorter mean survival time (34.7 months) than AR patients (79.1 months) (p&lt;.03). AR patients were more likely to have no comorbidities (p&lt;0.009) and interestingly less likely to receive care at Academic/Research program (p&lt;0.035). There were no significant differences found between the adjuvant treatment groups in terms of age at diagnosis, tumor size, and positive regional lymph nodes. Although not statistically significant, there was a small difference in patients with no residual tumor on surgical margins being more likely to have received AC versus adjuvant AR (p&lt;.111). Conclusions: This study demonstrates that among adjuvant therapies for the treatment of leiomyosarcoma of the small intestine, radiation therapy is associated with the longest mean survival time. This finding may be influenced by many factors, including the preference for chemotherapy over radiation in patients with comorbidities and the lack of standardized guidelines for the use of adjuvant therapy in this cancer. Therefore, further research is needed to better understand the true causes behind the disparity.
The optimal choice and combination strategy of vascular-targeting therapy for patients with advanced soft tissue sarcoma: A systematic review and network meta-analysis.
Authors: Yan Wang, Hangcheng Xu, Qiang Sa, Yiran Zhou, Binghe Xu, Jiayu Wang
Journal: Journal of Clinical Oncology (2025)

e23532 Background: Soft tissue sarcomas (STS) are rare, heterogeneous tumors with poor prognosis, often characterized by high recurrence rates and limited response to conventional chemotherapy, especially in advanced stages. Vascular-targeting agents (VTAs) have shown promising efficacy in treating advanced STS, yet optimal agent selection and combination strategies remain undetermined. Methods: This systematic review and network meta-analysis evaluated the efficacy and safety of various VTAs used as monotherapy or in combination with chemotherapy or immunotherapy for advanced STS. We searched PubMed, Embase and Cochrane Library for randomized controlled trials (RCTs) involving VTAs in advanced STS. Progression-free survival (PFS), overall survival (OS), objective response rate (ORR), and treatment-related adverse events (TRAEs) were assessed. Pooled hazard ratios (HRs) and odds ratios (ORs) with credible intervals (CrIs) were generated. A Bayesian random-effects model was used to rank different treatments based on surface under the cumulative ranking curve (SUCRA) values. Results: A total of 24 articles covering 22 studies were included in this meta-analysis. All VTA-containing interventions demonstrated improved efficacy over placebo in either PFS or OS. Among these, chemotherapy combined with large molecular monoclonal antibodies showed a trend toward prolonged OS compared with tyrosine kinase inhibitors (TKI) (HR 0.49; 95% CI 0.25-0.99). SUCRA values indicated that anti-angiogenic monoclonal antibodies plus chemotherapy ranked in the top three for PFS (66.33%), OS (82.70%), and ORR (65.11%). Additionally, TKI combined with chemotherapy and vascular-disrupting agents plus chemotherapy were identified as the most likely to enhance PFS, OS, and ORR. TKI-containing regimens were associated with higher incidences of hypertension and diarrhea, while chemotherapy-containing strategies showed higher incidences of anemia, fatigue and constipation. Conclusions: Anti-angiogenic monoclonal antibody plus chemotherapy showed relatively better efficacy than other drugs and combination strategies in short-term efficacy and long-term survival in patients with advanced STS. All the TRAEs were moderate and well-tolerated.
Immune landscape of sarcoma patients treated with an autologous dendritic cell vaccine.
Authors: Aditi Dhir, Shannon Saigh, Eric Wieder, Eli Gilboa, Andrew Rosenberg, Daniel Bilbao Cortes, Gina Z. D'Amato, Jonathan C. Trent, Breelyn A. Wilky
Journal: Journal of Clinical Oncology (2025)

e23530 Background: The role of the sarcoma immune tumor microenvironment (TME) is a critical area of ongoing research, particularly to understand responses to immunotherapy. We previously conducted a phase I trial (2014-2019) of autologous dendritic cell (DC) vaccination in 19 patients with advanced or metastatic sarcomas (NCT01803152). Median PFS was 9.5 months (95% CI 5.6-28.7), with 4 of 19 patients (20%) exhibiting durable PFS with a median follow-up of two years, including two patients with pleomorphic myxofibrosarcoma, one with leiomyosarcoma and one pediatric patient with Ewing sarcoma, far exceeding outcomes expected with recurrent metastatic sarcoma. We hypothesized that increased pro-inflammatory immune cells at baseline in the TME would associate with prolonged PFS after treatment with DC vaccine. Methods: Components of the immune TME were assessed in archival tumor tissue from study patients using imaging mass cytometry with the Standard Biotools Hyperion platform. A panel was designed to quantify and phenotype the following immune cells: CD8+ cytotoxic T cells, CD4+ helper T cells, FOXP3+ T reg cells, B cells, natural killer (NK) and NK-T cells, and multiple myeloid populations including M1 pro-inflammatory tumor-associated macrophages (TAMs), M2 suppressive TAMs, and DC and MDSC subsets. We also analyzed expression of checkpoint proteins PD-1, PD-L1, CTLA-4, TIM3. Imaging data was analyzed using the Visiopharm Phenoplex Guided Workflow module (v2023.09.4.15595 x64). An Unpaired t-test with Welch correction for each population was performed to compare proportion of cells across the responders and non-responder samples. The pixel intensity for Granzyme B and PDL-1, was compared using Mann-Whitney test and the Kolmogorov-Smirnov test. Results: The TME of the responders (n = 4) was different compared to the non-responders (n = 7), as evidenced by the t-SNE plot. Although not statistically significant, tissue from the responders exhibited a 2.5 fold higher density of M2 Macrophages (mean 10.46% SD 15.97 vs 4.15% SD 3.12), a 7.5 fold higher % of CD4 T cells (mean 3.88%, SD 6.41 vs 0.52%, SD 0.63), 11.7 fold higher % of CD8 T cells (mean 4.11%, SD 6.97 vs 0.35%, SD 0.36 ) and 2-fold higher M1 macrophages (mean 19.90%, SD 38.27 vs 11.45%, SD 19.80) when compared with non-responders, respectively. While there was no statistical difference in the percentage of Granzyme B+ and PD-L1+ cells between responders and non-responders, we did note a statistically significant difference in the expression as evidenced by difference in the pixel intensities, p &lt; 0.0001. Conclusions: Responding patients to autologous DC vaccines demonstrate more robust baseline immune infiltration, in line with previous studies showing higher immune related gene expression correlates with pre-existing immune responses and better outcomes with immunotherapy.
Deep learning-based contouring of Couinaud segments on CT: Utility for volumetric analysis of future liver remnant.
Authors: Tejas Mathai, Praveen T.S. Balamuralikrishna, Vivek Batheja, Michael Kassin, Ifechi Ukeh, Cathleen Hannah, Jonathan Matthew Hernandez, Ronald M. Summers
Journal: Journal of Clinical Oncology (2025)

4113 Background: Hepatocellular carcinoma (HCC) is the most common primary liver tumor, and the liver is a frequent site of metastasis of other cancers. High tumor burden, proximity to hepatic vessels, and other comorbidities render only 30% of patients as candidates for curative treatment: transplantation, resection, or ablation. Surgical resection requires a 20 - 40% future liver remanent (FLR) to avoid post-operative complications. Delineation of the Couinaud segments is essential for volumetric analysis of FLR and targeted localization of tumors during pre-surgical treatment planning. Currently, manual annotation of the Couinaud segments in one CT volume can take two or more hours, which makes it cumbersome, and can benefit from automation. The purpose of this work is to develop an automated Couinaud segmentation tool for fast and accurate FLR estimation. Methods: Three CT datasets were used: 1) 161 patients from the public Medical Segmentation Decathlon (MSD) Hepatic Vessels dataset, 2) 43 patients with cirrhosis and metabolic diseases having ascites and splenomegaly imaged at the National Institutes of Health (NIH), and 3) 197 patients in the public TCIA Colorectal Liver Metastasis (CRLM) dataset. FLR annotation in the CRLM dataset was done by an expert radiologist. The Couinaud segments in the MSD and NIH datasets were manually annotated by two physicians using ITK-SNAP. The MSD and NIH datasets were used for training, while the CRLM dataset was reserved for testing. A 3D nnU-Net model was trained with default hyperparameters to outline the Couinaud segments. On the test dataset, the predicted Couinaud segments were overlaid on the FLR annotation, and metrics, such as Dice Similarity Coefficient (DSC), Hausdorff Distance (HD) error (in mm), and volume error (in cc) were calculated. The performance was compared to a previously described 3D U-Net model developed to quantify liver segmental volume ratio (LSVR) in patients with cirrhosis. Results: The 3D nnU-Net obtained a DSC of 0.99 ± 0.01 (IQR: 0.991, 0.998), HD error of 0.87 ± 1.83 mm (IQR: 0, 1.02), and volume error of 13.7 ± 28.1 cc (IQR: 3.4, 15.3). In contrast, the LSVR U-Net model attained a DSC of 0.97 ± 0.01 (IQR: 0.972, 0.984), HD error of 9.56 ± 3.61 mm (IQR: 7.14, 11.93), and volume error of 47.9 ± 51.8 cc (IQR: 24.5, 55.4). The 3D nnU-Net model achieved significantly different results for DSC (p &lt; 0.001, large effect size 0.86), HD error (p &lt; 0.001, large effect size 0.87), and volume error (p &lt; 0.001, large effect size 0.91). Conclusions: The model showed acceptable generalizability to the external TCIA CRLM dataset. Future work may be directed towards accurate volumetric analysis on patients undergoing portal vein embolization to increase the FLR, and automatic tumor localization on specific Couinaud segments.