Juvenile dermatomyositis (JDM), a pediatric subtype of idiopathic inflammatory myopathy, is a relatively rare rheumatic and immune system disease among children. Although paraneoplastic dermatomyositis (DM) has been documented in adult malignancies, its association with acute myeloid leukemia (AML) in pediatric populations remains exceptionally rare. We present a male patient diagnosed with JDM at age 6, characterized by Gottron's papules and muscle weakness. Despite receiving sequential immunosuppressive therapies and biological agents, the disease followed a relapsing-remitting course over 9 years. At age 15, he developed persistent knee arthralgia accompanied by intermittent fever lasting 8 days. Hematologic evaluation revealed thrombocytopenia (platelet count: 76 ×10⁹/L) and leukocytosis (white blood cell count: 36.46 ×10⁹/L), prompting bone marrow aspiration confirmed AML diagnosis (French-American-British M2 subtype). Following induction chemotherapy and two consolidation cycles, allogeneic hematopoietic stem cell transplantation (HSCT) from a matched unrelated donor was successfully performed. Notably, both JDM symptoms and AML achieved sustained remission at 1-year follow-up post-HSCT. This case suggests that HSCT may represent a potential therapeutic approach for refractory autoimmune-hematologic malignancy overlap syndromes, though further studies are required to validate its efficacy.

Uveal melanoma (UM) is a rare intraocular malignancy with limited systemic treatment options and poor outcomes once metastatic. Tebentafusp, an ImmTAC (Immune-mobilizing monoclonal T-cell receptor Against Cancer) targeting gp100 in HLA-A*02:01-positive patients, has improved survival, although durable responses remain uncommon. Radiotherapy (RT) has been shown to induce immunogenic cell death and to modulate antitumor immune responses, supporting a potential synergistic interaction with systemic immunomodulatory therapies.

We described a 39-year-old woman with localized UM initially treated with proton therapy (60 Gy in four fractions), who developed liver and hilar adenopathies metastases seven years later. After confirmation of metastatic melanoma and HLA-A*02:01 positivity, tebentafusp was started and well tolerated. Following four months of therapy, imaging revealed oligoprogression in a hilar adenopathy, while hepatic lesions remained stable. Stereotactic body radiotherapy (SBRT; 30 Gy in 10 fractions) was delivered to the nodal site while tebentafusp was continued. Subsequent MRI at nine months demonstrated partial response across all lesions. The patient has maintained disease control for an additional 35 months, with excellent quality of life (ECOG PS 0).

This case illustrates a durable systemic response after combined tebentafusp and SBRT, suggesting a synergistic interaction between ImmTAC-mediated T-cell activation and radiation-induced immunogenic modulation. Local RT may enhance antigen release and T-cell recruitment, amplifying tebentafusp efficacy and inducing abscopal-like effects. Prospective studies are warranted to evaluate this combination and identify biomarkers predictive of response.

Transplantation-associated thrombotic microangiopathy (TA-TMA) is a severe complication following hematopoietic stem cell transplantation, with its pathogenesis not yet fully understood and a lack of highly effective treatment options currently available. This article reports a case of a female patient with acute myeloid leukemia (AML) who was diagnosed with TA-TMA three months after a second allogeneic hematopoietic stem cell transplantation (allo-HSCT). After promptly discontinuing cyclosporine (CSA), and following ineffective treatments with anti-infective therapy, glucocorticoids, and plasma exchange, a short-course combination regimen-defibrotide (10 days) and eculizumab (3 weeks)-was administered. The TA-TMA-related indicators rapidly improved and returned to normal. During follow-up, the patient's primary disease remained in remission, and she remained free of TA-TMA recurrence for five months. At eight months after the second allo-HSCT, TA-TMA recurred due to concurrent pneumonia and sepsis. The same short-course combination regimen-defibrotide (18 days) and eculizumab (3 weeks)-was administered, and the patient showed improvement in clinical and laboratory parameters again. This study is the first to report the use of a short-course combination therapy with defibrotide and eculizumab for TA-TMA. The combination regimen demonstrated good efficacy and tolerability, offering a potential treatment option for refractory TA-TMA, though its broader applicability requires further research and validation.

Recent research has explored the potential of using neoadjuvant dual immune checkpoint inhibitors (ICIs) combined with de-escalated chemotherapy in several locally advanced tumors to determine if such a combined regimen can enhance tumor response while minimizing toxicity. However, few related studies are focused on locally advanced cervical cancer (LACC). In this study, we present a case from the NICE-CC trial evaluating the feasibility of neoadjuvant dual immune checkpoint inhibitor (ICI) combined with a de-escalated chemotherapy regimen for LACC. A patient with stage IIB LACC had a high tumor burden and a presumed "immune cold" status, indicated by PD-L1 negativity with a Combined Positive Score (CPS) of 0. The patient achieved a pathological complete response (pCR) after receiving one cycle of neoadjuvant iparomlimab and tuvonralimab (simultaneously targeting PD-1 and CTLA-4) combined with standard chemotherapy, followed by two additional cycles of iparomlimab and tuvonralimab. Mechanically, the tumor microenvironment (TME) in this case was characterized by an abundance of tumor-infiltrating lymphocytes (TILs) and tertiary lymphoid structures (TLSs), which might be associated with improved responses to ICI therapy. In conclusion, this case highlights the potential of one cycle of neoadjuvant dual immunotherapy combined with standard chemotherapy, followed by two additional cycles of dual immunotherapy, for the treatment of LACC. This innovative treatment regimen warrants further investigation in the ongoing NICE-CC trial.

This meta-analysis systematically evaluated the efficacy and safety of B-cell maturation antigen (BCMA)-directed chimeric antigen receptor (CAR) T-cell therapy in patients with relapsed or refractory multiple myeloma (RRMM) with extramedullary disease (EMD).

PubMed, Embase, Web of Science, and the Cochrane Library were searched for studies published up to December 2024 reporting CAR T-cell therapy in RRMM patients with EMD. Studies were screened according to predefined inclusion and exclusion criteria. Data were extracted and the methodological quality was assessed using the Newcastle-Ottawa Scale (NOS) and the MINORS tool; one low-quality study was excluded. A total of 42 studies were included, comprising 242 RRMM patients with EMD and 1,485 without EMD. Fixed- or random-effects models were applied to pool effect sizes. Primary outcomes included objective response rate (ORR), complete response (CR), progression-free survival (PFS), and overall survival (OS). Secondary outcomes included cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS).

The pooled ORR and CR rates were 79% (95%CI: 71%-86%) and 42% (95%CI: 32%-51%) in the EMD group, and 90% (95%CI: 86%-93%) and 49% (95%CI: 40%-58%) in the non-EMD group, respectively. Reported median PFS ranged from 3 to 18.8 months in the EMD grouPand from 1 to 38 months in the non-EMD group, while median OS ranged from 6 to 13.9 months and from 12.2 to 38 months, respectively. The pooled incidences of grade ≥3 CRS and ICANS were 18% (95%CI: 8%-27%) and 5% (95%CI: 3%-7%) in the EMD group, compared with 13% (95%CI: 7%-19%) and 6% (95%CI: 2%-9%) in the non-EMD group; none of the differences were statistically significant (P> 0.05). Due to inconsistent reporting and lack of individual patient-level data, hazard ratios and pooled time-to-event analyses were not feasible.

Although RRMM patients with EMD exhibited lower ORR and CR rates than those without EMD, BCMA-directed CAR T therapy demonstrated notable clinical activity with a manageable safety profile. However, no direct comparisons with conventional therapies were performed in this analysis.

https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42025613422, identifier CRD42025613422.

IgG4-SC typically requires long-term corticosteroid therapy. We report a distinctive case of a 65-year-old woman with biopsy-supported IgG4-SC that closely mimicked malignancy.

The patient presented with 10 kg weight loss and CA19-9 of 315 U/mL. She achieved sustained clinical and biochemical remission after repeated endoscopic mechanical decompression alone, with no recurrent obstructive cholestasis during long-term clinical and biochemical follow-up. However, serum IgG4 remained elevated at long-term reassessment, and complete immunologic remission could not be assumed.

This case raises the possibility of an "obstruction-dominant" presentation of IgG4-SC and suggests that intensive endoscopic biliary decompression may serve as a temporary or, in selected cases, sustained steroid-sparing strategy in carefully selected high-risk patients, without challenging the current role of corticosteroids as standard first-line therapy.

Transcatheter chemoembolization (TACE) combined with tyrosine kinase inhibitors (TKIs) and immune checkpoint inhibitors (ICIs) shows promising efficacy in treating unresectable hepatocellular carcinoma (uHCC), but the specific patient population that would benefit most from this regimen remains unclear. This study aims to evaluate the prognoses of uHCC patients receiving triple therapy and develop a practical prognostic scoring model to identify those with the best beneficial.

This multicenter retrospective study enrolled 270 uHCC patients who received first-line triple therapy across 20 centers. These participants were divided into the training (n=190) and external validation (n=80) cohorts. Treatment response was assessed by the modified Response Evaluation Criteria in Solid Tumors (mRECIST), and safety was evaluated via treatment-related adverse events (TRAEs) using National Cancer Institute Common Terminology Criteria for Adverse Events version 5.0 (NCI-CTCAE v5.0). Cox proportional hazards regression was used to identify independent prognostic factors for overall survival, which were utilized to develop the SAGES score; Kaplan-Meier curves and area under the receiver operating characteristic curve (AUC) were employed to validate the model's performance.

In the training cohort, the objective response rate was 47.9% and disease control rate was 63.2%. The median progression-free survival was 15.9 months, with 3-year overall survival and progression-free survival rates of 52.2% and 30.7%, respectively. Independent prognostic factors for poor overall survival included albumin-bilirubin grade 2-3, alpha-fetoprotein ≥400 ng/mL, maximum tumor size ≥8 cm, presence of extrahepatic metastasis, and absence of conversion surgery. Integrating these five factors, the SAGES score effectively stratified patients into low- (0-3 points), intermediate- (4-7 points), and high-risk (8-10 points) groups with significantly divergent survival outcomes in both cohorts (all p<0.001). The model exhibited robust discriminative ability, with AUCs of 0.78 in the training cohort and 0.75 in the validation cohort, outperforming individual prognostic factors.

Triple therapy showed promising clinical outcomes and the SAGES score provides reliable prognostic stratification and facilitates personalized treatment decisions for uHCC patients receiving this triple regimen.

Early postoperative complications significantly influence short-term recovery after radical prostatectomy. Although laparoscopic radical prostatectomy (LRP) provides perioperative advantages compared with open radical prostatectomy (ORP), its impact on early morbidity remains uncertain. This study aimed to compare 30-day postoperative complications between ORP and LRP and to identify independent predictors of early morbidity. This retrospective two-center cohort included 149 consecutive patients undergoing ORP (n = 98) or LRP (n = 51) between 2022 and 2024. Complications were graded using the Clavien-Dindo classification. Multivariable logistic regression models evaluated independent predictors, including age, body mass index (BMI), pelvic lymph node dissection (PLND), and surgical approach. LRP was associated with shorter operative time (193.1 ± 18.9 vs 231.0 ± 27.0 minutes; P < 0.001), lower estimated blood loss (396.1 ± 114.4 vs 534.7 ± 108.3 mL; P < 0.001), and shorter hospitalization (4.92 ± 1.07 vs 6.50 ± 1.12 days; P < 0.001). Early complications occurred in 18.1% of patients (21.4% ORP vs 11.8% LRP; absolute difference 9.6%; 95% CI, -2.4% to 21.6%; P = 0.146). The distribution of complication severity was similar between groups. In multivariable analysis, BMI independently predicted early complications (OR = 1.76 per kg/m²; P < 0.001), and PLND was associated with increased risk in the extended model (OR = 5.51; P = 0.009). The surgical approach was not independently associated with complications. Despite clear perioperative advantages of LRP, early postoperative morbidity appeared more strongly associated with BMI and PLND than with surgical access. Larger prospective studies are warranted.

Elevated inflammatory markers are consistently linked to poor outcomes in cancer, whereas favorable nutritional status correlates with improved survival. This retrospective study examined the interaction between nutritional-inflammatory indices and their impact on outcomes in patients with cervical cancer.

Data from 465 cervical cancer patients treated at the Second Affiliated Hospital of Soochow University between January 2015 and June 2025 were retrospectively analyzed. The neutrophil-to-lymphocyte ratio (NLR), lymphocyte-to-monocyte ratio (LMR), Prognostic Nutritional Index (PNI), and Naples Prognostic Score (NPS) were calculated. Associations of these indices with overall survival (OS) and progression-free survival (PFS) were assessed using the Kaplan-Meier method and Cox regression models. Prognostic value was further evaluated through construction of a nomogram, with predictive accuracy validated using receiver operating characteristic (ROC) curves, decision curve analysis (DCA), and calibration analysis.

Significant differences between deceased patients and survivors were observed in body mass index (BMI), tumor burden, tumor markers, nutritional/inflammatory indices, pathological characteristics, and treatment status (all p < 0.05). Higher LMR, lower NLR, elevated PNI, and lower NPS were associated with improved OS and PFS (p < 0.001). Multivariate analysis identified BMI, PNI, Federation Internationale de Gynecologie et d'Obstetrique (FIGO) stage, differentiation, CA125, HPV infection, and targeted therapy as independent determinants of OS, while BMI, tumor volume, PNI, HPV infection, histology, differentiation, FIGO stage, and NPS independently predicted PFS. BMI exhibited a linear relationship with both OS and PFS, while PNI demonstrated a linear negative correlation with OS and a significant non-linear relationship with PFS. Nomograms incorporating eight variables for OS and six for PFS achieved AUC values ≥ 0.86 at 3-, 5-, and 10-year timepoints, demonstrating reliable discrimination, accurate calibration, and greater net clinical benefit compared with individual markers. Subgroup analyses consistently indicated protective effects of PNI (HR∼0.9) and low NPS (HR 0.3-0.9), though prognostic strength varied slightly according to clinical context and treatment approach.

PNI was validated as a robust independent prognostic factor for both OS and PFS, while NPS demonstrated independent predictive value specifically for PFS. As easily obtainable nutritional-inflammatory indices, they complement conventional clinicopathological parameters. The integrated prognostic model established in this study shows exploratory potential in estimating OS and PFS. Pending future external validation, it may serve as an adjunctive reference for risk stratification and follow-up optimization.

Osteosarcoma patients who have undergone prosthetic lower limb reconstruction following tumour resection walk at a slower preferred speed than healthy controls. In addition, they demonstrate lower peak isokinetic knee extension torques, which may explain the observed differences in sagittal plane knee joint kinematics during walking. However, mechanisms for this and the associated knee joint kinetics are not well understood, particularly at matched walking speeds.

This observational case-control study compared sagittal plane lower limb walking gait characteristics between patients who have undergone prosthetic reconstruction and matched healthy controls across their preferred and matched walking speeds. Data were collected from 18 control participants and 17 patients while walking on a force-instrumented treadmill at the different speeds. Spatiotemporal variables, peak knee flexion angle, peak knee extensor moments (torques), and peak knee joint power were compared between groups.

Patients walked with a slower preferred speed than the control group. When comparing lower limb gait mechanics at matched speeds, patients demonstrated lower magnitude peak knee extensor moments than the control group, and these differences were greater at faster speeds. At the fastest walking speed, patients also displayed lower peak knee joint power compared to the control group. The differences in knee joint kinetics observed between groups may be due to an inability amongst patients to generate the magnitudes of knee extensor force that the control group can generate.