bims-myxlip Biomed News
on Myxoid liposarcoma
Issue of 2025–03–16
two papers selected by
Laura Mannarino, Humanitas Research



  1. ESMO Open. 2025 Mar 07. pii: S2059-7029(25)00167-X. [Epub ahead of print]10(3): 104299
       BACKGROUND: We report the results of the pre-planned secondary analysis of radiologic responses (RRs) of ISG-STS 1001, a randomized trial comparing anthracycline + ifosfamide (AI) versus histology-tailored (HT) neoadjuvant chemotherapy for primary localized high-risk soft-tissue sarcomas of the extremities and trunk wall.
    PATIENTS AND METHODS: Patients with undifferentiated pleomorphic sarcoma (UPS), leiomyosarcoma (LMS), malignant peripheral nerve sheath tumor, synovial sarcoma or myxoid liposarcoma (MLPS) were randomized, whereas patients with myxofibrosarcoma, pleomorphic liposarcoma, pleomorphic rhabdomyosarcoma or unclassified sarcoma were included in the observational arm (O) and treated with AI. Patients with UPS, LMS or MLPS needing concurrent preoperative radiotherapy were included in O. We evaluated associations between: disease-free survival (DFS)/overall survival (OS) and centrally reviewed RR, assessed with RECIST 1.1 and as percent dimensional variation (D; both dichotomized and continuous); DFS/OS and histology; RR and histology.
    RESULTS: Four hundred and thirty-five patients were included (287 randomized, 148 observed). The analysis of RRs comprised 236 patients (154 randomized, 82 observed) with measurable disease and available for central review. RECIST best responses were: 28 (11.9%) partial response (PR), 195 (82.6%) stable disease (SD), 13 (5.5%) progressive disease (PD). RECIST significantly correlated with DFS [PD versus PR: hazard ratio (HR) 8.18, 95% confidence interval (CI) 2.96-22.58; SD versus PR: HR 2.96, 95% CI 1.30-6.75] and OS (PD versus PR: HR 12.61, 95% CI 3.40-46.84; SD versus PR: HR 4.24, 95% CI 1.34-13.47). The median value of D was -1.6%. Patients with D >-1.6% had worse clinical outcomes than those with D <-1.6% (DFS: HR 1.73, 95% CI 1.19-2.50; OS: HR 1.86, 95% CI 1.21-2.86). D in continuous scale inversely correlated with DFS (HR 1.53, 95% CI 1.25-1.87) and OS (HR 1.78, 95% CI 1.41-2.25).
    CONCLUSIONS: These results confirm the prognostic value of RRs as per RECIST and D and demonstrate that any variation in size predicts the proportional efficacy of treatment.
    Keywords:  RECIST; neoadjuvant chemotherapy; prognosis; randomized clinical trial; sarcoma; tumor response
    DOI:  https://doi.org/10.1016/j.esmoop.2025.104299
  2. Med Oncol. 2025 Mar 08. 42(4): 94
      Selinexor is a new compound studied for the treatment of liposarcoma, particularly dedifferentiated liposarcoma (DDLPS), where treatment options remain limited. As a first-in-class oral exportin-1 (XPO1) inhibitor, selinexor has shown anti-tumour activity in preclinical models, particularly in MDM2- and CDK4-amplified DDLPS, where it induces apoptosis, inhibits tumour growth and promotes nuclear retention of p53. Preclinical studies have also suggested potential synergy with doxorubicin and eribulin, although these findings have yet to be validated in randomised clinical trials. The phase II/III SEAL trial (NCT02606461) evaluated selinexor versus placebo in patients with advanced, previously treated DDLPS. While the study demonstrated a statistically significant, albeit modest, improvement in median progression-free survival (PFS) from 2.1 to 2.8 months, no overall survival benefit was observed. In addition, selinexor was associated with significant toxicity, including fatigue, nausea and weight loss. Similarly, a phase Ib/II study (NCT03042819) evaluating selinexor in combination with doxorubicin reported a 21% response rate and a median PFS of 5.5 months, although this regimen was also associated with high rates of neutropenia and fatigue. Despite these results, selinexor is not currently approved for the treatment of liposarcoma and its clinical utility remains under investigation. Ongoing studies, such as the SeliSarc trial (NCT04595994) evaluating selinexor in combination with gemcitabine and the NRSTS2021 trial (NCT06239272) evaluating selinexor in paediatric soft tissue sarcoma, aim to further define its role. The results of these studies will be critical in determining whether selinexor can be incorporated into standard sarcoma treatment.
    Keywords:  Dedifferentiated liposarcoma; Liposarcoma; Sarcoma; Selinexor; XPO1
    DOI:  https://doi.org/10.1007/s12032-025-02651-2