Objective: To investigate the correlation between ATRX expression and alternative lengthening of telomeres (ALT) activation status in uterine smooth muscle tumors, and to evaluate the values of the two indicators in diagnosing uterine leiomyosarcoma (uLMS). Methods: Cases of uLMS and its mimickers diagnosed at the Department of Pathology, Peking University Third Hospital, Beijing, China from January 2024 to October 2025 were retrospectively identified and analyzed. Immunohistochemistry was used to detect ATRX protein expression, while telomere-specific fluorescence in situ hybridization was applied to determine ALT activation status. Next-generation sequencing (NGS) was conducted to detect ATRX gene variations in some cases. Fisher's exact test was used to analyze the correlation between ATRX expression and ALT status, as well as the differences in ATRX expression and ALT activation among different tumor types. Kappa test was applied to evaluate the agreement between ATRX expression and ALT status. Results: There were 60 cases of uLMS, 36 cases of smooth muscle tumors of uncertain malignant potential (STUMP), 77 cases of uterine leiomyoma (uLM), and 6 cases of high-grade endometrial stromal sarcoma. NGS revealed that ATRX gene mutations were mostly accompanied by complete loss of protein expression, while copy number reduction was mostly associated with decreased protein expression. The concordant rate between ATRX expression and ALT activation status was 86.4%, with a significant correlation (P<0.001) and substantial consistency (Kappa=0.68). The rates of complete ATRX loss (35/59, 59.3%) and ALT activation (49/60, 81.7%) in uLMS were significantly higher than those in the other groups: 14.3% (5/35) and 17.1% (6/35) in STUMP, 0 (0/72) and 2.6% (2/77) in uLM, and 0 (0/6) for both markers in high-grade endometrial stromal sarcoma. All intergroup differences were statistically significant (all P<0.001). Conclusions: ATRX loss is highly correlated with ALT activation status. Combined detection of ATRX expression and ALT activation status can effectively assist in the differential diagnosis of uLMS from its mimickers. In addition, the presence of ATRX loss and/or ALT activation in STUMP and uLM suggests a malignant potential and seems to warrant close clinical follow-up and individualized diagnosis and treatment decisions.

Soft-tissue sarcomas (STS) comprise over 150 histological subtypes, with advanced cases showing poor prognosis (5-year survival <10%). Trabectedin, a synthetic alkaloid, is frequently used after anthracycline-based chemotherapy failure. Despite the withdrawal of reimbursement in France in 2016 due to debated efficacy and safety, it remains in clinical use, imposing financial strain on hospitals. This retrospective single-center study evaluated trabectedin's efficacy, safety, and cost in 68 patients treated between 2019 and 2023. L-sarcomas accounted for 78% of cases, including uterine leiomyosarcomas (n = 16), soft-tissue leiomyosarcomas (n = 17), and myxoid liposarcomas (n = 8). Non-L-sarcomas (22%) included mostly synovial sarcomas. The overall disease control rate was 71%, with a median progression-free survival (PFS) of 4.1 months, and an overall survival of 12.3 months. Subtype-specific median PFS was 6.8 months for liposarcomas (11.3 for myxoid vs. 4.5 for other subtypes), 3.1 months for leiomyosarcomas (3.4 months for uterine vs. 3.1 for soft-tissue), and 2.4 months for non-L-sarcomas. Patients received a median of 5 cycles (range: 1-38), with an average total dose of 16 mg [2-81], and an average hospital cost of €9900. Adverse events occurred in 91%, mainly hematological; cardiac toxicity was seen in 9%. This retrospective single-center study in a limited cohort provides real-world insights but should be interpreted with caution. Despite limited reimbursement, trabectedin may retain clinical utility, particularly in L-sarcoma management.

Background/Objectives: Recurrent uterine leiomyosarcoma (ULMS) frequently poses a surgical question because systemic options remain limited and recurrence patterns are heterogeneous. We reviewed the published evidence on repeated and extended cytoreductive surgery for recurrent ULMS, focusing on selection criteria, operative boundaries, and the role of multivisceral, thoracic, and peritoneal-directed procedures. Methods: This narrative review synthesizes peer-reviewed literature on surgically managed recurrent or metastatic ULMS, prioritizing contemporary guidelines, retrospective cohorts, pooled analyses, selected systematic reviews when directly relevant to the surgical question, and published illustrative reports. The search covered records available from database inception through 14 May 2026 and used PubMed/MEDLINE, Web of Science Core Collection, Scopus, Google Scholar, selected publisher databases, and citation-linked records. No new patient-level or institution-specific clinical data are presented. Results: The available evidence is entirely retrospective and strongly affected by selection bias, yet it consistently suggests that the best outcomes are observed when complete gross resection is feasible. Across published series, favorable features include isolated or limited recurrence, longer time to relapse, compartmentalized disease, lung-only metastases, and preserved performance status. Contemporary reports also show that repeat surgery may evolve into extensive multivisceral procedures involving bowel resection, upper-abdominal dissection, urinary tract reconstruction, diaphragmatic resection, and thoracic surgery. Peritoneal-directed CRS/HIPEC-type strategies remain supported mainly by small heterogeneous studies and a ULMS-specific systematic review, reinforcing feasibility but not routine use. Published illustrative reports confirm that serial metastasectomies can occasionally support prolonged survival in exceptional patients, but they cannot establish effectiveness. Conclusions: In highly selected patients, repeated and even extensive cytoreductive surgery may remain a rational disease-control strategy for recurrent ULMS. The central unmet need is not proof that surgery can work in exceptional cases, but better criteria to identify who benefits from iterative resection and when escalation to multivisceral or thoracoabdominal surgery is justified.

Among soft tissue tumors, TRIM63 in situ hybridization (ISH) represents an emerging biomarker often positive in alveolar soft part sarcoma(ASPS) and perivascular epithelioid cell tumor (PEComa). However, its expression among gynecological tract mesenchymal tumors has been largely unexplored. Herein, we investigate sensitivity/specificity for TRIM63 ISH in uterine PEComas to aid in distinction from relevant differential diagnostic entities. A total of 33 PEComas, 35 leiomyosarcomas (LMS), 2 smooth muscle tumors of uncertain malignant potential (STUMP),and 10 leiomyomas underwent whole-slide TRIM63 ISH staining on a representative section of tumor. One ASPS was also identified and served as a positive control (not included in sensitivity and specificity calculations). Expression was quantified using a modified H-score system (range 0-12 with negative = 0, weak = 1-4, moderate = 5-8 or strong = 9-12). TRIM63 ISH was positive (H score > 0) in 28/33 (85%) PEComas, with a median H-score of 6; 24/33 (73%) had at least moderate expression (H score ≥ 5). Twenty-five (71%) LMS had H-score of 0, although 1 case had strong expression. Weak expression was also seen in 1 STUMP (50%) and 5 leiomyomas (50%); one leiomyoma had moderate expression (10%). The ASPS had strong expression (H-score 12). Employing a cutoff of > 0 TRIM63 positivity yielded a sensitivity of 85% and specificity of 64% for the diagnosis of uterine PEComa. Using a cutoff of at least moderate (≥ 5) expression decreased sensitivity to 73% but increased specificity to 96%. In the appropriate morphologic context, moderate to strong TRIM63 ISH expression may be a useful adjunct biomarker to conventional immunohistochemistry in the diagnosis of uterine PEComa.

Li-Fraumeni syndrome (LFS) is a cancer predisposition syndrome characterized by germline TP53 mutations and increased risk of various malignancies. While rare uterine leiomyosarcomas (LMS) have been reported, other uterine smooth muscle tumours (uSMT) have not been studied in this context. We describe clinicopathological features of uSMT from 10 LFS patients (four uSMT of uncertain malignant potential (STUMP), two leiomyomas with bizarre nuclei [LMBN] and four with only conventional leiomyomas [LM]) and next-generation sequencing results of eight tumours. Among six p53-aberrant tumours (four STUMPs, one LMBN, one LM) by immunohistochemistry (IHC), three STUMPs had biallelic TP53 inactivation and three uSMT (two STUMPs, one LMBN) had loss of fumarate hydratase (FH) by IHC with inactivating FH variants. One patient with a p53-aberrant, FH-deficient LMBN had a concurrent p53-wild-type, FH-proficient LM with a novel ACTG2::BRAF fusion. Two STUMPs with TP53 biallelic inactivation and intact FH had additional alterations, one with LMS features, including chromosome instability, ATRX, and RB1 alterations; the other with del(22q), CYLD, and ELOC mutations. The remaining two sequenced LM showed MED12 alterations, one with del(22q). No recurrences were seen in nine patients with follow-up and no LMS were diagnosed. uSMTs exhibit a broad morphologic spectrum in LFS, and multiple molecular alterations may drive tumorigenesis, including MED12, FH, TP53, RB1, ATRX, and a novel ACTG2::BRAF fusion. Although some may be incidental, uSMT in this setting appears enriched for atypical morphology, FH deficiency, and aberrant p53 expression, suggesting an interplay between p53 and FH pathways in tumorigenesis.

Tumor-infiltrating CD8+Ki-67+ and CD8+LAG-3+ T cells are associated with improved patient survival in retroperitoneal dedifferentiated liposarcomas and leiomyosarcomas.

Mismatch repair-deficient (dMMR) sarcomas are rare and incompletely characterized. Here, we studied 39 sarcomas with high microsatellite instability (MSI-H) and/or biallelic MMR gene inactivation (21 MSH2, 9 MLH1, 4 PMS2, 4 MSH6, 1 EPCAM). All tumors evaluated with immunohistochemistry (IHC; n = 36) showed loss of MMR protein expression. Eight sarcomas were index neoplasms among the 15 patients with documented Lynch syndrome. Eighteen of 1531 sarcomas (1.2%) in our sequencing database were dMMR, with enrichment in pleomorphic rhabdomyosarcoma (PRMS; 1/5), uterine leiomyosarcoma (LMS; 7/124; 5.6%), and unclassified/undifferentiated pleomorphic sarcoma (UPS; 6/259; 2.3%). MMR IHC screening of independent cases confirmed MMR deficiency in PRMS (2/20), uterine LMS (2/20), and unclassified/UPS (1/20). Two histologic patterns were identified among unclassified/UPS. Ten tumors, designated "distinctive lobulated inflammatory sarcoma" (DLIS), showed lobular architecture, florid inflammation, and histiocytoid, variably pleomorphic neoplastic cells. All 6 patients with DLIS and follow-up (median: 6.0 y; range: 3 mo to 8.6y) were alive with no evidence of disease (ANED), and 2 DLIS responded completely to immune checkpoint inhibition. A morphologically different group of 6 unclassified high-grade sarcomas showed sheets of epithelioid-to-rhabdoid cells with eosinophilic cytoplasm; among 5 patients with follow-up (median: 1.1y; range: 4 mo to 6.9y), only 1 was ANED. Surprisingly, all 3 PRMS patients with follow-up (median: 5.7 y; range: 4.2 to 8.3y) were ANED, including 2 with complete responses of metastases to systemic therapy. We conclude that PRMS, uterine LMS, and unclassified/UPS showed sufficiently prevalent MMR deficiency to justify prospective MMR IHC screening for Lynch syndrome and to identify patients who might benefit from immune checkpoint inhibition. Histologic subtyping of unclassified sarcomas predicted prognosis and therapeutic response. We propose universal MMR IHC screening of (1) PRMS, (2) uterine LMS, (3) unclassified/UPS, and (4) any sarcoma in a patient with a personal or family history of Lynch syndrome.

ASO Visual Abstract: Efficacy and Safety of Cytoreductive Surgery with Hyperthermic Intraperitoneal Chemotherapy for Recurrent Uterine Leiomyosarcoma: Results of a Phase 2 Study.

Alternative lengthening of telomeres (ALT) is a telomere elongation mechanism activated during oncogenesis and primarily acting in tumors of mesenchymal origin. Although the proteins involved in the machinery enabling ALT to elongate telomeres are becoming better understood, the underlying biology of this mechanism remains unclear. In the present study, we took advantage of a fully characterized cohort of 98 leiomyosarcomas (LMS) from the French Sarcoma Group to further our understanding of the ALT mechanism. We first compared the transcriptomic profiles of ALT+ and TERT+ LMS and demonstrated a strong enrichment of the CINSARC signature in ALT+ tumors. The establishment of an ALT+-related signature in these LMS confirmed the close association between CINSARC and ALT in two additional cohorts of non-translocation-related sarcomas. In vitro mesenchymal models of spontaneous ALT induction showed increased CINSARC expression following acquisition of the ALT mechanism. Conversely, ALT inactivation, through BLM inhibition, led to decreased CINSARC expression. These results establish CINSARC as a new hallmark of the ALT mechanism in non-translocation-related sarcomas and demonstrate the association of a cellular biological process with the CINSARC prognostic signature, namely the ALT mechanism.

Uterine leiomyosarcoma (ULMS) is a rare yet aggressive uterine malignancy with high recurrence and poor survival, prompting an urgent search for better treatments. We investigated whether atorvastatin, an HMG-CoA reductase inhibitor, could suppress ULMS growth by targeting mevalonate pathway-dependent prenylation. Human ULMS cell lines received atorvastatin ± isoprenoids to assess proliferation, cell-cycle distribution, and smooth muscle contractility, whereas proteomic profiling (LC-MS/MS) and in vivo xenografts were used to evaluate molecular pathways and antitumor efficacy. Atorvastatin inhibited ULMS proliferation in a dose-dependent manner, induced G₀/G₁ cell-cycle arrest, and diminished the contractile phenotype. Geranylgeranyl pyrophosphate rescued these effects, implicating geranylgeranylation as the key dependency, and Rap1A/Rap1B Western blotting confirmed functional GGPP depletion. In xenografts, atorvastatin suppressed ULMS tumor growth by ~50% with minimal toxicity, as evidenced by normal serum ALT and creatinine levels and preserved organ histology. These findings identify protein geranylgeranylation as a novel therapeutic vulnerability in ULMS and support statin repurposing as a promising treatment strategy.

We aimed to compare oncologic and peri-operative outcomes after minimally invasive surgery versus open surgery among women with clinical stage I uterine leiomyosarcoma. We performed a retrospective cohort study using the National Cancer Database (2010-2021). Eligible cases were clinical stage I leiomyosarcoma (cT1a/cT1b) treated with hysterectomy and bilateral salpingo-oophorectomy. Patients were grouped by surgical approach (minimally invasive surgery [laparoscopy/robotic] vs open); conversions were analyzed descriptively and excluded from outcome models. Primary outcome was overall survival; secondary outcomes were length of stay and 30-day readmission. Survival was assessed with Kaplan-Meier and log-rank tests; multi-variable Cox models adjusted for age, adjuvant therapy, margin status, and pathologic stage. Among 683 patients, 208 (30.5%) underwent minimally invasive surgery, 447 (65.4%) open surgery, and 28 (4.1%) had conversions. Minimally invasive surgery patients more often were White (85.1% vs 70.0%) and privately insured (66.8% vs 54.4%), had higher neighborhood income, more clinical stage IA disease (30.3% vs 15.2%), and smaller tumors (median 7.2 cm vs 9.9 cm, all p <.001). Adjuvant chemotherapy and radiotherapy rates were similar between groups. Minimally invasive surgery was associated with a shorter hospital stay (median 1 vs 3 days, p <.001) and comparable 30-day readmission (both 3.8%). In clinical stage IA, unadjusted overall survival favored minimally invasive surgery (68.0% alive at 94 months vs 51.5% open, log-rank p =.028), whereas stage IB showed no difference (median overall survival 79 vs 79 months, p =.72). In multi-variable analysis, surgical approach was not independently associated with overall survival (adjusted hazard ratio 1.16, 95% confidence interval 0.91 to 1.49). Worse overall survival was associated with increasing age, higher stage, and positive margins. The surgical approach was not independently associated with survival. Future prospective cohorts should capture specimen extraction and tumor disruption to better define which patients can safely undergo minimally invasive approaches.

5559 Background: Initial results from our trial showed the anti-tumor activity and good tolerance of CDK4/6 inhibitor dalpiciclib combined with NSAIs in patients (pts) with ER+ R/M ovarian cancer and uterine neoplasms (SGO, 2024, #S283; IGCS, 2025, #397). Here, we report updated results. Methods: This single-center, single-arm, open label, phase 2 trial enrolled pts with ER+, R/M ovarian cancer and uterine neoplasms which met the following criteria: pretreated low-grade serous ovarian carcinoma (LGSOC), pretreated uterine endometrioid carcinoma (EC), uterine leiomyosarcoma (LMS), or untreated low-grade endometrial stromal sarcoma (LGESS). Pts received dalpiciclib (150mg po d1-21 q28d) and NSAIs (mainly letrozole 2.5mg po qd) until disease progression, unacceptable toxicity or consent withdrawn. The primary endpoint was 12-week progression-free survival (12wPFS) rate. A Simon’s two-stage design was used (one-sided α=0.05, power=80%), and the null hypothesis (P0=0.2) would be rejected if totally &gt;10 of 33 pts achieved primary endpoints. Results: As the data cutoff (Jan 5, 2026), 35 pts with a median of one prior therapy for R/M disease were enrolled, including 11 with LGSOC, 15 with EC, 8 with LGESS and 1 with LMS. With a median follow-up of 11.4 months (range, 0.1-52.2), 22 of 29 efficacy-evaluable pts had reached the primary endpoint, with a 12wPFS rate of 75.9% (95% CI 56.5-89.7), suggesting the value of further investigation. Detailed efficacy results are shown in Table 1. Grade 3-4 treatment-related adverse events (TRAEs) occurred in 82.9% (29/35) of pts, mostly neutrophil count decreased (71.4%), white blood cell count decreased (45.7%) and platelet count decreased (14.3%). Sixteen pts had dose reduction of dalpiciclib, mostly due to hematologic toxicities. No serious adverse events or treatment-related deaths occurred. Twelve pts were still on-treatment, with 3 pts treated for &gt;4 years. Conclusions: The trial achieved its positive primary endpoint, showing meaningful efficacy of dalpiciclib + NSAIs in ER+ R/M ovarian cancer and uterine neoplasms. No new safety signals were observed. Clinical trial information: ChiCTR2000040597. Efficacy results of evaluable pts. OverallN=29 LGSOCn=9 ECn=11 LGESSn=8 LMSn=1 12wPFS rate[95%CI] 75.9% (22/29)[56.5-89.7] 77.8% (7/9)[40.0-97.2] 63.6% (7/11)[30.8-89.1] 100% (8/8)[63.1-100] 0% (0/1)[0-97.5] Clinical Response Partial response (PR) 5 (17.2%) 1 (11.1%) 2 (18.2%) 2 (25.0%) 0 (0%) Stable disease (SD) 17 (58.6%) 6 (66.7%) 5 (45.5%) 6 (75.0%) 0 (0%) SD≥24 weeks 14 (48.3%) 5 (55.6%) 4 (36.4%) 5 (62.5%) 0 (0%) Progressive disease (PD) 7 (24.1%) 2 (22.2%) 4 (36.4%) 0 (0%) 1 (100%) ORR (CR+PR, 95% CI) 17.2% (5.85-35.8) 11.1% (0.28-48.3) 18.2% (2.28-51.8) 25.0% (3.19-65.1) 0% (0-97.5) CBR (CR+PR+SD≥24 weeks, 95% CI) 65.5% (45.7-82.1) 66.7% (29.9-92.5) 54.5% (23.4-83.3) 87.5% (47.4-99.7) 0% (0-97.5)

Magnetic resonance imaging (MRI) of the myometrium - benign and malignant disease.

Uterine sarcomas are rare, heterogeneous malignancies with distinct pathological behaviors. This study aimed to identify clinicopathological characteristics, prognostic risk factors, and potential therapeutic targets to enhance clinical management. A retrospective analysis was conducted on 148 patients with uterine sarcoma treated at Peking University People's Hospital between 1996 and 2025. Clinical outcomes, pathological subtypes, and immunohistochemical profiles were assessed. Additionally, bioinformatics analyses from RNA bulk sequencing of GEO datasets (GSE87581, GSE85383, GSE222045 and GSE64763) were performed to elucidate molecular characteristics across subtypes. The most prevalent subtypes were uterine leiomyosarcoma (uLMS; 38.5%) and low-grade endometrial stromal sarcoma (LG-ESS; 29.7%). The 5-year recurrence rate was 50.5%, with frequent metastases to the pelvis and lungs. LG-ESS demonstrated the most favorable 5-year survival rate (90.3%), significantly higher than that of uLMS (61.8%) and undifferentiated uterine sarcoma (50.0%). Multivariate analysis identified histological subtype, stage, and coagulative necrosis as independent prognostic factors for overall and progression-free survival. Transcriptomic profiling revealed immunosuppression (CSF1R/CSF3R expression) in high-grade ESS, while uLMS exhibited activation of cell cycle and homologous recombination pathways. Histological subtype, stage, and coagulative necrosis were critical prognostic factors in uterine sarcoma. The findings suggest that vigilant pulmonary surveillance and further investigation into tailored therapeutic strategies may be warranted-including endocrine therapy for hormone-receptor-positive tumors, immunotherapy for high-grade ESS, and PARP inhibitors for uLMS. However, these hypotheses require thorough preclinical and clinical validation. Additionally, caution should be exercised to avoid overtreatment of chemotherapy in early-stage uLMS.

Primary cutaneous leiomyosarcoma (LMS) is a rare smooth muscle neoplasm encompassing a spectrum of dermal and subcutaneous tumors with distinct clinical behaviors. Dermal LMS typically presents as a small, firm nodule arising from arrector pili muscle and generally follows an indolent course, whereas subcutaneous LMS originates from vascular smooth muscle and demonstrates greater infiltrative potential, larger size at presentation, and a substantially higher risk of recurrence, metastasis, and disease-specific death. Histologically, LMS is characterized by intersecting fascicles of spindle cells with smooth muscle differentiation, with the diagnosis being confirmed by smooth muscle actin and desmin immunoreactivity. Recent molecular profiling has identified tumor suppressor pathway dysregulation-particularly TP53 and RB1 loss-and widespread copy-number instability as central drivers of cutaneous LMS. Clinically, outcomes are governed principally by depth, grade, and margin status: dermal, low-grade tumors rarely metastasize, whereas lesions with higher-grade or subcutaneous extension account for most adverse events. Complete surgical excision with negative margins remains the cornerstone of therapy, with Mohs micrographic surgery offering precise margin control for select superficial tumors. Ongoing debate regarding the atypical intradermal smooth muscle neoplasm (AISMN) designation for dermally-confined tumors reflects evolving understanding, although we argue that risk stratification is best guided by histologic features rather than nomenclature alone.

Uterine leiomyosarcoma (uLMS) is an aggressive malignancy with high rates of peritoneal recurrence and poor survival. Although surgery for recurrent uLMS has shown encouraging results, addition of hyperthermic intraperitoneal chemotherapy (HIPEC) is not well described. We evaluate safety and efficacy of cytoreductive surgery (CRS)-HIPEC with gemcitabine followed by systemic dacarbazine for recurrent uLMS. This was an open-label, single-arm phase 2 study of patients with recurrent uLMS who underwent CRS-HIPEC at a single tertiary center (March 2021-May 2025). HIPEC was performed for 60min with gemcitabine (1000mg/m2), followed by six doses of adjuvant dacarbazine (1000mg/m2). Primary study objective was 1-year progression-free survival. Secondary outcomes were adverse events, intraperitoneal recurrence, and quality of life. A total of 17 patients underwent CRS-HIPEC with gemcitabine for recurrent uLMS (median 55 years). Most patients had received prior systemic therapy (76%) and undergone at least two operations for uLMS (65%). Median PFS was 11 months, significantly longer than historical control of 3 months (p < 0.001). Median OS was not reached at follow up time over 3 years. The study was closed early due to slow accrual: at time of closure, two patients remained without evidence of disease recurrence. Median PCI was 7; all patients achieved CCR 0. There were no Clavien-Dindo grade 3 complications, and median postoperative stay was 7 days. Two patients experienced grade 4 toxicities (hypokalemia and neutropenia). CRS with gemcitabine HIPEC and adjuvant systemic chemotherapy is feasible without increased morbidity and has potential to improve outcomes and achieve long-term survival in patients with recurrent uLMS.

Uterine epithelioid leiomyosarcoma with PGR::NR4A3 fusion gene: report of a case

Rationale:Differentiation between uterine leiomyoma and leiomyosarcoma of uterus(LMS) is often challenging, especially in the context of pregnancy, which may delay subsequent diagnosis and treatment.Patient concerns:A 35-year-old pregnant woman was found to have uterine fibroids based on preand prenatal ultrasound findings, and she remained asymptomatic. Due to the large tumor size, which affected fetal delivery and incision closure, she underwent cesarean section combined with myomectomy. Postoperative pathology revealed LMS. Further CT scans indicated pulmonary metastases, leading to a diagnosis of stage IVB LMS. Subsequently, the patient underwent laparoscopic total hysterectomy with bilateral salpingo-oophorectomy, pelvic lymphadenectomy, peritoneal biopsies, and omental sampling. The patient remains under follow-up.Diagnoses:The diagnosis was made based on postoperative pathology and relevant imaging findings.Interventions:The patient was treated with surgery and chemotherapy.Outcomes:The patient underwent surgery and chemotherapy, and was still under follow-up.Lessons:Attention should be paid to the diagnosis of LMS in the context of pregnancy. When facing a fibroid of uncertain nature during cesarean section, it is necessary to assess whether myomectomy is feasible and whether intraoperative autologous blood transfusion can be performed. Therefore, timely diagnosis through comprehensive clinical and imaging findings is essential to avoid misdiagnosis and ensure prompt intervention.

Leiomyosarcoma (LMS) is an aggressive soft-tissue sarcoma characterized by epigenomic dysregulation and variable responsiveness to checkpoint inhibitors (CPIs). In this study, we evaluated a novel liquid biopsy platform based on circulating cell-free DNA active chromatin (cfDNAac) profiling to identify baseline biomarkers associated with clinical benefit rate (CBR) in 30 LMS patients treated with durvalumab plus olaparib or cediranib. A total of 1,570 molecular features across genomic regions were identified, and recursive feature elimination was applied to select discriminative cfDNAac signatures. Patients achieving CBR demonstrated enrichment of pathways related to cell death, interferon-γ signaling, and immune activation. Signatures reflecting B-cell activation, T-cell activation, and extracellular matrix organization were significantly associated with improved progression-free survival (PFS; p < 0.05). In contrast, a baseline tumor fraction >5% was negatively associated with CBR (p = 0.041) and correlated with inferior PFS (p = 0.008). Distinct copy number variation profiles further characterized patients with CBR and were significantly associated with PFS. In this analysis, cfDNAac profiling represents a promising non-invasive strategy to predict clinical benefit from CPI-based therapy in LMS. Prospective validation in independent cohorts is warranted to clarify its clinical utility.

Data science meets FTIR Imaging: a promising probe to improve the diagnosis of human uterine muscle lesions.