Clinical presentation, management and outcome of 11,132 patients with liposarcoma patients: a population-based study from the NETSARC+ registry.

Background: Liposarcomas (LPS) are among the most common sarcomas, but gather a diversity of rare to ultrarare molecular subtypes whose presentations and natural histories are partially characterized. The aim of the work was to describe the presentation and outcome of the different LPS histotypes from the NETSARC+ registry.

Methods: NETSARC+ (netsarc.

Defining benchmark values for outcomes of comprehensive resection of primary retroperitoneal liposarcoma: a retrospective multicenter study.

Background: Comprehensive resection represents the standard of care for patients affected by retroperitoneal well- or dedifferentiated liposarcoma (WDLPS/DDLPS). However, reference values to indicate the best achievable results are currently lacking. As such, the study aimed to define clinically relevant benchmark values for intra- and postoperative outcomes of patients undergoing comprehensive resection for primary retroperitoneal WDLPS/DDLPS.

Global Variations in Patterns of Care for Retroperitoneal Sarcoma.

Objective: To examine variations in patterns of care for retroperitoneal sarcoma (RPS) among sarcoma centres globally, including diagnostic work-up, surgical strategies and (neo)adjuvant therapies.

Methods: Retrospective analysis for primary RPS, from 19 RPS referral centres worldwide, prospectively collected within the RESAR repository (NCT03838718) between Feb 2017 - July 2022. Centres were categorised high volume (HVC) or low volume (LVC).

Diagnostic work-up of lipomatous tumors: a decision-making analysis among European sarcoma centers.

Objectives: Lipomatous soft-tissue tumors present a diagnostic burden. The aim of this work was to compare standard operating procedures (SOPs) for the diagnostic management of lipomatous soft-tissue tumors among European academic centers.

Methods: Experts of the Soft Tissue and Bone Sarcoma Group of the European Organization for Research and Treatment of Cancer were asked for their SOPs in the diagnosis of adipocytic soft-tissue tumors in an otherwise healthy patient.

CINSARC and Sarculator in Patients with Primary Retroperitoneal Sarcoma: A Combined Analysis of Single-Institution Data and the EORTC-STBSG-62092 Trial (STRASS).

Purpose: The Complexity INdex in SARComas (CINSARC) predicts the metastatic risk in patients with soft-tissue sarcoma. The aims of this study were to provide the first independent validation of CINSARC in patients with retroperitoneal sarcoma (RPS) and evaluate whether CINSARC could enhance the performance of Sarculator.

Experimental Design: A retrospective cohort included patients with primary localized RPS resected with curative intent (2011-2015) at a single institution.

Radiologic and Pathologic Response Evaluation After Neoadjuvant Chemotherapy for Primary Retroperitoneal Sarcoma: A Transatlantic Australasian Retroperitoneal Sarcoma Working Group (TARPSWG) Collaboration.

Background: Comsplete resection is the primary treatment for retroperitoneal sarcomas (RPS). The role of neoadjuvant chemotherapy (NACT) in improving oncologic outcomes is currently under investigation in the STRASS 2 trial. This study assessed the association between change in tumor size or pathologic response and oncologic outcomes.

Evaluation of the effect of neoadjuvant chemotherapy on the planned resectability of extremity soft-tissue sarcomas.

Aims: In this study, we explore whether neoadjuvant chemotherapy influences the surgical resection strategy devised by surgeons for high-grade soft-tissue sarcoma.

Methods: A total of 12 experienced soft-tissue sarcoma surgeons rated patients who underwent neoadjuvant chemotherapy for a soft-tissue sarcoma of the thigh. Cases were randomly assigned to surgeons, such that each surgeon rated three out of the 12 cases, and each case was rated by three out of 12 surgeons (n = 36 ratings before and after chemotherapy).

Evolving Concepts in the Management of Desmoid Tumors.

DT is a rare monoclonal fibroblastic neoplasm with an unpredictable biologic behavior. Over the past 15 y, the worldwide approach to desmoid tumor (DT) has shifted dramatically from surgery, when feasible, to frontline active surveillance in the majority of cases, to select patients who truly require treatment. Approximately 50% of cases demonstrate indolent disease, while the remaining patients may require active treatment.

Activation of lysosomal iron triggers ferroptosis in cancer.

Iron catalyses the oxidation of lipids in biological membranes and promotes a form of cell death called ferroptosis. Defining where this chemistry occurs in the cell can inform the design of drugs capable of inducing or inhibiting ferroptosis in various disease-relevant settings. Genetic approaches have revealed suppressors of ferroptosis; by contrast, small molecules can provide spatiotemporal control of the chemistry at work.

Impact and safety of pregnancy on desmoid fibromatosis management in the era of active surveillance. An international multicenter retrospective observational study.

Aim: To evaluate desmoid fibromatosis (DF) progression and obstetric outcomes during and after pregnancy, providing evidence to inform clinical decision-making and counseling.

Methods: This international, multicenter, retrospective observational study included data from 157 women with DF, contributing 177 pregnancies. Women were classified into three groups: DF diagnosed during pregnancy (Group A), pregnancy occurring after the diagnosis of DF (Group B: DF in situ during pregnancy), or pregnancy in previously resected DF (Group C).

Genetic predisposition in sarcomas: clinical implications and management.

Unlabelled: Recent studies indicate up to 20% of sarcomas may be associated with predisposition genes, and this number will probably increase as genetic testing becomes more available. Evidence on the management of patients with sarcoma and genetic predisposition remains, however, scarce. This review compiles available research on genetic predisposition syndromes associated with sarcoma and sarcoma treatment within such syndromes, addressing key gaps in knowledge.

Palliative care outpatient interventions to limit aggressive care at end-of-life for patients with advanced soft tissue sarcomas.

Purpose: Factors reducing aggressive care at the end of life (EOL) for soft tissue sarcoma (STS) are unknown. We aim to evaluate the association between outpatient palliative care (PC) interventions and aggressive care at EOL for STS patients.

Methods: All deceased STS patients in our center were included retrospectively over two years.

Conference on challenges in sarcoma (CCS) 2024: Expert opinions on non-evidence-based management aspects.

Background: Soft tissue sarcomas (STS) and other mesenchymal tumours belong to rare, heterogeneous neoplasms with over 150 subtypes that pose significant challenges in diagnosis and clinical decision making. While guidelines address evidence-based diagnostic and therapeutic procedures, clinical situations and scenarios without evidence remain controversial in daily practice. The 2024 Conference on Challenges in Sarcoma (CCS2024) aimed to narrow these gaps with the support of an international and multidisciplinary panel of sarcoma experts.

Celiac ganglion resection as an intraoperative factor associated with delayed gastric emptying in retroperitoneal sarcoma surgery: A multicentric prospective cohort study.

Introduction: Delayed gastric emptying (DGE) is a common but underexplored complication following retroperitoneal sarcoma surgery. The celiac ganglion, often excised during these procedures, plays a key role in gastric motility and its removal may contribute to DGE development.

Materials And Methods: Data from 42 patients were prospectively collected at three referral centers.

Active Surveillance in Patients with Extra-abdominal Desmoid-Type Fibromatosis: A Pooled Analysis of Three Prospective Observational Studies.

Purpose: Three prospective observational studies (Italy, the Netherlands, and France) on active surveillance (AS) in patients with extra-abdominal desmoid-type fibromatosis support AS as a first-line approach. Identifying prognostic factors for the failure of AS will help determine the strategy. The aim of this study was to investigate the prognostic impact of clinical and molecular variables in a larger series.

Comparing epidemiological and clinical data from RPS patients documented in a German cancer registry to a cohort from TARPSWG reference centres.

Purpose: Retroperitoneal sarcomas (RPS) are rare, heterogeneous tumours. Treatment recommendations are mainly derived from cohorts treated at reference centres. The applicability of data from cancer registries (CR) is controversial.

Cellular origin and clonal evolution of human dedifferentiated liposarcoma.

Dedifferentiated liposarcoma (DDLPS) is the most frequent high-grade soft tissue sarcoma subtype. It is characterized by a component of undifferentiated tumor cells coexisting with a component of well-differentiated adipocytic tumor cells. Both dedifferentiated (DD) and well-differentiated (WD) components exhibit MDM2 amplification, however their cellular origin remains elusive.

The Landmark Series: Multimodal Management of Oligometastatic Sarcoma.

The concept of "oligometastatic" disease suggests the presence of intermediate states between localized disease and widespread metastases, which may be potentially treatable with curative therapeutic strategies. Metastases local therapy (MLT) can be accomplished through various techniques such as stereotactic ablative radiotherapy (SABR), radiofrequency ablation (RFA), microwave ablation (MWA), cryoablation, or surgical metastasectomy. The incorporation of MLT in the multidisciplinary treatment of patients with metastatic sarcoma is complex.

Activation of lysosomal iron triggers ferroptosis in cancer.

Iron catalyses the oxidation of lipids in biological membranes and promotes a form of cell death referred to as ferroptosis. Identifying where this chemistry takes place in the cell can inform the design of drugs capable of inducing or inhibiting ferroptosis in various disease-relevant settings. Whereas genetic approaches have revealed underlying mechanisms of lipid peroxide detoxification, small molecules can provide unparalleled spatiotemporal control of the chemistry at work.