Sarcoma: Last Year's Practice Changing Papers.

Background: In 2023 and 2024, a wide variety of new studies have been published in the field of soft tissue sarcomas, representing the enormous heterogeneity of sarcoma histotypes, anatomical location, treatment variability, and biological behavior.

Patients And Methods: This article summarizes the, in our view, seven most important publications in the field that will have an impact on the surgical practice and future treatment strategies of our patients.

Results: In the last year, we gained more insight in the genetic background of patients with sarcoma from a large Australian study, which will have an impact on future counseling and screening of our patients.

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, France) on active surveillance (AS) in patients with extra-abdominal desmoid-type fibromatosis (DTF) support AS as a frontline 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.

Safety and efficacy of pembrolizumab, radiation therapy, and surgery versus radiation therapy and surgery for stage III soft tissue sarcoma of the extremity (SU2C-SARC032): an open-label, randomised clinical trial.

Background: Approximately half of patients with localised, high-risk soft tissue sarcoma of the extremity develop metastases. We aimed to assess whether the addition of pembrolizumab to preoperative radiotherapy and surgery would improve disease-free survival.

Methods: We completed an open-label, randomised clinical trial in patients with grade 2 or 3, stage III undifferentiated pleomorphic sarcoma or dedifferentiated or pleomorphic liposarcoma of the extremity and limb girdle.

Radiomic features of primary retroperitoneal sarcomas: a prognostic study.

Background: Risk-stratification of patients with retroperitoneal sarcomas (RPS) relies on validated nomograms, such as Sarculator. This retrospective study investigated whether radiomic features extracted from computed tomography (CT) imaging could i) enhance the performance of Sarculator and ii) identify G3 dedifferentiated liposarcoma (DDLPS) or leiomyosarcoma (LMS), which are currently consider in a randomized clinical trial testing neoadjuvant chemotherapy.

Methods: Patients with primary localized RPS treated with curative-intent surgery (2011-2015) and available pre-operative CT imaging were included.

Proximal and Classic Epithelioid Sarcomas are Distinct Molecular Entities Defined by MYC/GATA3 and SOX17/Endothelial Markers, Respectively.

Epithelioid sarcoma (ES) is a rare tumor hallmarked by the loss of INI1/SMARCB1 expression. Apart from this alteration, little is known about the biology of ES. Despite recent advances in treatment, the prognosis of ES remains unsatisfactory.

International Multicenter Retrospective Study From the Ultra-rare Sarcoma Working Group on Low-grade Fibromyxoid Sarcoma, Sclerosing Epithelioid Fibrosarcoma, and Hybrid Forms: Outcome of Primary Localized Disease.

The aim of the study was to report the outcome of primary localized low-grade fibromyxoid sarcoma (LGFMS), sclerosing epithelioid fibrosarcoma (SEF), and hybrid LGFMS/SEF (H-LGFMS/SEF). Patients with primary localized LGFMS, SEF, or H-LGFMS/SEF, surgically treated with curative intent from January 2000 to September 2022, were enrolled from 14 countries and 27 institutions. Pathologic inclusion criteria were predefined by expert pathologists.

One-Stage Surgical Resection and Functional Reconstruction for Upper Limb Soft Tissue Sarcoma.

Background: Currently, the gold standard of treatment for extremity soft tissue sarcoma (STS) is limb-sparing surgery. When the upper extremity is involved, the functional outcome is frequently poor. A 1-step resection and functional reconstruction would be advisable to obtain a fast recovery.

Timeline of surgery in localized angiosarcoma of the breast: Improving outcome following multidisciplinary treatment optimization.

Introduction: Primary (PAS) and radiation-associated angiosarcomas (RAAS) of the breast are rare tumors of vascular origin with poor survival. In this retrospective cohort study, we aimed to assess the impact of multidisciplinary treatment optimization on the prognosis of patients who underwent surgery at a national referral center.

Materials And Methods: Cases of operable angiosarcoma of the breast evaluated by a multidisciplinary team including surgeons, medical oncologists and radiation oncologists expert in the field and treated from January 2012 to January 2023 were retrieved from a prospectively maintained database.

Preoperative nutritional support to tackle morbidity in multivisceral resection for retroperitoneal sarcoma. Early outcomes from a novel nutritional prehabilitation program in a prospective cohort.

Objective: Evaluate the feasibility and outcomes of the implementation of a routinely prehabilitation nutritional program (PNP) in retroperitoneal sarcoma (RPS) patients.

Summary Background Data: Rate of preoperative malnutrition is scarcely evaluated in RPS patients and the efficacy of a PNP in detecting and reverting malnutrition has not been studied.

Methods: Prospective study in a high-volume reference center for RPS; adult patients with primary or persistent RPS deemed surgically resectable were enrolled in a PNP.

Histology-Tailored Approach to Soft Tissue Sarcoma.

Soft tissue sarcomas are a diverse and heterogeneous group of cancers of mesenchymal origin. Each histological type of soft tissue sarcoma has unique clinical particularities, which makes them challenging to diagnose and treat. Multidisciplinary management of these rare diseases is thus key for improved survival.

Extent of macroscopic vascular invasion predicts distant metastasis in primary leiomyosarcoma of the inferior vena cava.

Background: In retroperitoneal leiomyosarcoma (RP LMS), the predominant issue is distant metastasis (DM). We sought to determine variables associated with this outcome and disease-specific death (DSD).

Methods: Data were retrospectively collected on patients with primary RP LMS treated at a high-volume center from 2002 to 2023.

Opportunities and Challenges in Soft Tissue Sarcoma Risk Stratification in the Era of Personalised Medicine.

Soft tissue sarcomas (STS) are a rare and heterogeneous group of cancers. Treatment options have changed little in the past thirty years, and the role of neoadjuvant chemotherapy is controversial. Accurate risk stratification is crucial in STS in order to facilitate clinical discussions around peri-operative treatment.

Surgical Outcome in Retroperitoneal Sarcoma Surgery: Accuracy of P-POSSUM, ACS-NSQIP, and Inflammatory Biomarkers Prognostic Index (IBPI) Risk-Calculators for Prediction of Severe and Overall Morbidity.

Introduction: Retroperitoneal sarcoma often requires comprehensive resection, leading to severe postoperative morbidity. The lack of disease-procedure specific tools for morbidity risk and the questionable accuracy of existing tools (ACS-NSQIP and P-POSSUM) in RPS surgery drove this study to assess these calculators' accuracy.

Methods: Retrospective analysis of primary RPS cases undergoing surgery at two sarcoma-referral centers was conducted.

Proteomic profiling improves prognostic risk stratification of the Sarculator nomogram in soft tissue sarcomas of the extremities and trunk wall.

Background: High-risk soft tissue sarcomas of the extremities and trunk wall (eSTS), as defined by the Sarculator nomogram, are more likely to benefit from (neo)adjuvant anthracycline-based therapy compared to low/intermediate-risk patients. The biology underpinning these differential treatment outcomes remain unknown.

Methods: We analysed proteomic profiles and clinical outcomes of 123 eSTS patients.

Survival after resection of malignant peripheral nerve sheath tumors: Introducing and validating a novel type-specific prognostic model.

Background: This study aimed to assess the performance of currently available risk calculators in a cohort of patients with malignant peripheral nerve sheath tumors (MPNST) and to create an MPNST-specific prognostic model including type-specific predictors for overall survival (OS).

Methods: This is a retrospective multicenter cohort study of patients with MPNST from 11 secondary or tertiary centers in The Netherlands, Italy and the United States of America. All patients diagnosed with primary MPNST who underwent macroscopically complete surgical resection from 2000 to 2019 were included in this study.