Normal tissue outlining guidelines development for soft tissue sarcoma of the extremities.

Introduction: Radiotherapy (RT) plans for soft tissue sarcoma of the extremities (STSE) are optimised to achieve maximum target coverage whilst avoiding high doses to weight-bearing bones and intermediate doses to the normal tissue (NT) limb corridor. Within this study, novel lower extremity NT outlining guidelines and atlas were developed based on the hypothesis that using these for RT planning may reduce RT toxicity. Usability and applicability of the guidance were also investigated.

Ossifying fibromyxoid tumours: A case series.

Background: Ossifying fibromyxoid tumour is a rare mesenchymal soft tissue sarcoma with uncertain differentiation and variable metastatic potential.

Patients And Methods: This study offers a retrospective analysis of 23 patients diagnosed with OFMT between 1993 and 2024.

Results: The tumours most commonly arose in the extremities and trunk, with all patients undergoing surgical resection of the primary tumour.

The efficacy of combination immunotherapy with ipilimumab plus nivolumab in metastatic myxofibrosarcoma.

We present the case of a patient with Myxofibrosarcoma (MFS), a mesenchymal type of soft tissue sarcoma (STS) and the response to combination immunotherapy with anti PD-1 and anti-CTLA-4 therapy, following disease progression after Standard chemotherapy (SACT) and Radiotherapy (RT). We have shown a timeline of treatment and responses, as well as the overall safety profile and the management of immunotherapy related adverse events. This study demonstrates the potential of checkpoint inhibitors as therapeutic agents in the treatment of MFS.

Biphenotypic Sinonasal Sarcoma: Case Reports of Diagnostic Findings and Clinical Management of 3 Cases.

Background: Biphenotypic sinonasal sarcoma (BSNS) is a rare spindle cell sarcoma distinctly arising in the sinonasal area, with dual myogenic and neural differentiation, and characterised by the presence of PAX3 gene fusion, typically with MAML3. Although the majority may be indolent, up to 25% of cases reported in the literature are locally aggressive, with invasion of adjacent critical structures in the head and neck region.

Case Report: We report 3 cases of BSNS reviewed at our institution between 2016-2020 in addition to the current literature.

Case report: L5 tomita En bloc spondylectomy for oligometastatic liposarcoma with post adjuvant stereotactic ablative radiotherapy.

Introduction: Tomita En-bloc spondylectomy of L5 is one of the most challenging techniques in radical oncological spine surgery. A 42-year-old female was referred with lower back pain and L5 radiculopathy with a background of right shoulder liposarcoma excision. CT-PET confirmed a solitary L5 oligometastasis.

Radiomic Features From Diffusion-Weighted MRI of Retroperitoneal Soft-Tissue Sarcomas Are Repeatable and Exhibit Change After Radiotherapy.

Background: Size-based assessments are inaccurate indicators of tumor response in soft-tissue sarcoma (STS), motivating the requirement for new response imaging biomarkers for this rare and heterogeneous disease. In this study, we assess the test-retest repeatability of radiomic features from MR diffusion-weighted imaging (DWI) and derived maps of apparent diffusion coefficient (ADC) in retroperitoneal STS and compare baseline repeatability with changes in radiomic features following radiotherapy (RT).

Materials And Methods: Thirty patients with retroperitoneal STS received an MR examination prior to treatment, of whom 23/30 were investigated in our repeatability analysis having received repeat baseline examinations and 14/30 patients were investigated in our post-treatment analysis having received an MR examination after completing pre-operative RT.

The development and validation of a decision aid to facilitate patient choice of surgery versus radiotherapy for high-risk basal cell carcinoma.

Basal cell carcinoma (BCC) is an increasingly common cancer. For high-risk BCCs, there are several treatment options, with similar efficacies. The current best practice in deciding upon a particular treatment is for a patient-centred approach.

Treatments and Outcomes in Oligometastatic Soft Tissue Soft Sarcoma - A Single Centre Retrospective Analysis.

Background/aim: Distinguishing true oligometastatic disease from early polymetastatic disease is vital in patients with soft tissue sarcoma as contemporary treatment strategies differ significantly. Clinical factors such as tumour biology, organ involved, number of lesions, and patient fitness influence clinical decisions.

Patients And Methods: A retrospective search of a prospective database identified patients with new distant relapse, treated between 2009 and 2012.

A phase II study on the neo-adjuvant combination of pazopanib and radiotherapy in patients with high-risk, localized soft tissue sarcoma.

Purpose: A prior phase I study showed that the neo-adjuvant combination of pazopanib and radiotherapy was well tolerated, and induced promising pathological responses in soft-tissue sarcoma patients. Results of the subsequent prospective, multicenter phase II, PASART-2 trial are presented here, further investigating the efficacy and safety of this combination.

Patients And Methods: Patients with high-risk, localized soft-tissue sarcoma received neo-adjuvant radiotherapy, 50 Gy in 25 fractions (PASART-2A) or with a subsequent dose de-escalation to 36 Gy in 18 fractions (PASART-2B).

Descriptive analysis of MRI functional changes occurring during reduced dose radiotherapy for myxoid liposarcomas.

Objectives: Myxoid liposarcomas (MLS) show enhanced response to radiotherapy due to their distinctive vascular pattern and therefore could be effectively treated with lower radiation doses. This is a descriptive study to explore the use of functional MRI to identify response in a uniform cohort of MLS patients treated with reduced dose radiotherapy.

Methods: 10 patients with MLS were imaged pre-, during, and post-radiotherapy receiving reduced dose radiotherapy and the response to treatment was histopathologically assessed post-radiotherapy.

Clinical management and outcomes of primary ovarian leiomyosarcoma - Experience from a sarcoma specialist unit.

Ovarian sarcomas account for 1% of all ovarian malignancies and amongst these, primary ovarian leiomyosarcoma is the rarest subtype. Primary ovarian leiomyosarcoma has a very poor prognosis, with less than 20% of patients being alive at 5 years. Only a few cases have been published in the literature and there is very limited knowledge on the clinical behaviour and optimal management of these tumours.

Efficacy of Gemcitabine-based Chemotherapy in Clear Cell Sarcoma of Soft Tissue.

Background/aim: Clear cell sarcoma (CCS) is an aggressive sarcoma subtype, resistant to conventional anthracycline-based chemotherapy and radiation. The diagnosis is often challenging due to similarities with malignant melanoma.

Patients And Methods: We aimed to analyse the activity of gemcitabine-based chemotherapy in a cohort of patients with CCS treated at the Royal Marsden Hospital.

The route to diagnosis of sarcoma patients: Results from an interview study in the Netherlands and the United Kingdom.

Introduction: Sarcomas are rare tumours. Early diagnosis is challenging, but important for local control and potentially survival and quality of life(QoL). We investigated (1)the route to diagnosis (RtD) experienced by sarcoma patients, including factors contributing to the length of the RtD from patients' perspective; (2)the impact of the RtD on QoL and care satisfaction; and (3)differences in aims 1-2 between English and Dutch patients.

Dose Reduction of Preoperative Radiotherapy in Myxoid Liposarcoma: A Nonrandomized Controlled Trial.

Importance: Currently, preoperative radiotherapy for all soft-tissue sarcomas is identical at a 50-Gy dose level, which can be associated with morbidity, particularly wound complications. The observed clinical radiosensitivity of the myxoid liposarcoma subtype might offer the possibility to reduce morbidity.

Objective: To assess whether a dose reduction of preoperative radiotherapy for myxoid liposarcoma would result in comparable oncological outcome with less morbidity.

Health-Related Quality of Life and Experiences of Sarcoma Patients during the COVID-19 Pandemic.

Sarcomas are rare cancers with a spectrum of clinical needs and outcomes. We investigated care experiences and health-related quality of life (HRQoL) in sarcoma patients during the COVID-19 pandemic. Patients with appointments during the first two months of the UK lockdown were invited to complete a survey.

Telemedicine During the COVID-19 Pandemic: Impact on Care for Rare Cancers.

Purpose: Many patients with cancer, often those with rare cancers such as sarcomas, travel long distances to access expert care. The COVID-19 pandemic necessitated widespread changes in delivery of cancer care, including rapid adoption of telemedicine-based care. We aimed to evaluate the impact of telemedicine on patients, clinicians, and care delivery at the Royal Marsden Hospital (RMH) Sarcoma Unit during the pandemic.

Metastatic Soft Tissue Sarcomas in Adolescents and Young Adults: A Specialist Center Experience.

Soft tissue sarcomas (STS) account for 8% of all cancers in adolescents and young adults (AYAs). Metastatic STS contribute significantly to disease-related mortality in this age group; however, data are limited due to under-representation in clinical trials. AYAs aged 18-39 years, diagnosed with metastatic STS between 1990 and 2012, were identified from The Royal Marsden Hospital database.

Low-grade Fibromyxoid Sarcoma: Treatment Outcomes and Efficacy of Chemotherapy.

Background: Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma subtype with a generally indolent pattern of clinical behaviour, but treatments for advanced disease are limited.

Patients And Methods: A retrospective search of a prospectively maintained institutional database identified 102 patients treated from December 1994 to August 2018. We evaluated the outcome of patients and the efficacy and safety of non-surgical therapies in LGFMS.

Adult soft tissue myoepithelial carcinoma: treatment outcomes and efficacy of chemotherapy.

Soft tissue myoepithelial carcinomas are a rare, malignant subgroup of myoepithelial tumours mostly arising in the extremities with equal predilection for women and men. The mainstay of management of localised disease is complete surgical resection. Despite optimal treatment, 40-45% of tumours recur.

Outcome of Primary Desmoid Tumors at All Anatomic Locations Initially Managed with Active Surveillance.

Background: The behavior of desmoid tumors is unpredictable and varies from spontaneous remission to symptomatic and radiologic progression. This study aimed to evaluate the radiologic and symptomatic course of the disease in patients initially managed with active surveillance.

Methods: Patients with a primary desmoid tumor at any anatomic location diagnosed between 1998 and 2016 were identified in a prospectively maintained database from a single sarcoma reference center in the United Kingdom.

The Role of Local Therapy in Multi-focal Epithelioid Haemangioendothelioma.

Background/aim: Epithelioid haemangioendothelioma (EHE) is a rare tumor with a wide spectrum of clinical behavior. There is no consensus on the role of local therapy in symptomatic, multi-focal disease.

Patients And Methods: A retrospective review of patients presenting to the Royal Marsden Hospital between January 2000 and December 2017 was conducted.

Utility of Multi-Parametric Quantitative Magnetic Resonance Imaging for Characterization and Radiotherapy Response Assessment in Soft-Tissue Sarcomas and Correlation With Histopathology.

To evaluate repeatability of quantitative multi-parametric MRI in retroperitoneal sarcomas, assess parameter changes with radiotherapy, and correlate pre-operative values with histopathological findings in the surgical specimens. Thirty patients with retroperitoneal sarcoma were imaged at baseline, of whom 27 also underwent a second baseline examination for repeatability assessment. 14/30 patients were treated with pre-operative radiotherapy and were imaged again after completing radiotherapy (50.

Gemcitabine Re-challenge in Metastatic Soft Tissue Sarcomas: A Therapeutic Option for Selected Patients.

Background/aim: Treatment options for patients with metastatic soft tissue sarcomas are limited. Re-challenge with a previously successful gemcitabine-based regimen is common. There are no published data to support this practice.