Treatment and Outcomes of Radiation-Induced Soft Tissue Sarcomas of the Extremities and Trunk—A Systematic Review of the Literature
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SUBMITTER: Inchaustegui M
PROVIDER: S-EPMC10705150 | biostudies-literature | 2023 Nov
REPOSITORIES: biostudies-literature
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Project description:Soft tissue sarcomas (STS) are rare tumors; they do not even equate to 1% of all malignant tumor cases. One-fifth of all STS occur in the upper extremities, where epithelioid sarcoma, synovial sarcoma, clear cell sarcoma and malignant fibrohistiocytoma are the most frequent subtypes. Surgical resection is the cornerstone of treatment. However, accomplishment of optimal oncological and functional results of STS of the upper extremities may represent a challenge for hand surgeons, due to the complex anatomy. In several cases, preoperative therapies are needed to facilitate tumor resection and improve the oncological outcome. Oligometastatic disease may also be a challenging scenario as curative strategies can be applied. Radiotherapy and chemotherapy are commonly used for this purpose albeit with conflicting evidence. Novel drug combinations have also been approved in the metastatic setting, further improving the quality of life and survival of eligible patients. Thus, prior to any approach, every case should be individually discussed in sarcoma centers with specialized multidisciplinary tumor boards. The aim of the present review was to gather the multidisciplinary experiences of the available therapeutic strategies for STS of the upper extremities.
| S-EPMC6676724 | biostudies-literature
Project description:BackgroundHigh-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.MethodsWe analysed proteomic profiles and clinical outcomes of 123 eSTS patients. A Cox model for overall survival including the Sarculator was fitted to individual data to define four risk groups. A DNA replication protein signature-Sarcoma Proteomic Module 6 (SPM6) was evaluated for association with clinicopathological factors and risk groups. SPM6 was added as a covariate together with Sarculator in a multivariable Cox model to assess improvement in prognostic risk stratification.ResultsDNA replication and cell cycle proteins were upregulated in high-risk versus very low-risk patients. Evaluation of the functional effects of CRISPR-Cas9 gene knockdown of proteins enriched in high-risk patients using the cancer cell line encyclopaedia database identified candidate drug targets. SPM6 was significantly associated with tumour malignancy grade (p = 1.6e-06), histology (p = 1.4e-05) and risk groups (p = 2.6e-06). Cox model analysis showed that SPM6 substantially contributed to a better calibration of the Sarculator nomogram (Index of Prediction Accuracy = 0.109 for Sarculator alone versus 0.165 for Sarculator + SPM6).ConclusionsRisk stratification of patient with STS is defined by distinct biological pathways across a range of cancer hallmarks. Incorporation of SPM6 protein signature improves prognostic risk stratification of the Sarculator nomogram. This study highlights the utility of integrating protein signatures for the development of next-generation nomograms.
| S-EPMC11263812 | biostudies-literature
Project description:There is no consensus regarding follow-up after soft tissue sarcoma (STS) treatment. This study examines the efficacy and the cost-benefit of MRI imaging for discovering recurrence. A retrospective analysis was performed, collecting data on patient demography, tumor characteristics, treatment, and follow-up. Imaging was correlated to the clinical course, and sensitivity, specificity, and predictive values were calculated. The number needed to screen and costs of finding recurrence are reported. Amongst 216 sarcomas, 73 (35%) exhibited local recurrence during a follow-up of 5.3 ± 3.5 years. 173 entities had complete MRI follow-up with 58 (34%) local recurrences. Thirty-three (57%) were discovered by MRI, 8 (14%) by clinical presentation, and 17 (29%) simultaneously. There was a sensitivity of 100.00%, a specificity of 89%, a positive predictive value of 32%, and a negative predictive value of 100% for detecting local recurrence with MRI. Our data confirm the modalities and intervals proposed by the German guidelines for sarcoma care. The recommended MRI intervals should not be extended. MRI is more cost-effective than clinical examination; still, both modalities should be performed together to discover the maximum number of recurrences.
| S-EPMC8100420 | biostudies-literature
Project description:The extremity site is a peculiar location for soft-tissue sarcomas (STS) of children and adolescents. Here we report the experience of the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan, Italy, concerning 204 patients with STS of the limbs treated between 1977 and 2006.The study series included 52 patients with rhabdomyosarcoma (RMS)(65% of which were of the alveolar subtype), nine with extraosseous Ewing sarcoma and 143 with non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), 38% of which were synovial sarcoma. Patients were treated with a multimodality approach including surgery, chemotherapy, and radiotherapy.For the RMS patients, the 5-year event-free survival (EFS) rate was 37.1%, with distant metastases being the main cause of treatment failure. The outcome was particularly poor for patients with large invasive tumors, hand/foot involvement and/or nodal or distant metastases, and for patients who were not given radiotherapy. For the extraosseous Ewing sarcoma cases, 5-year EFS was 74%. For the NRSTS cases, the 5-year EFS was 72.6%: tumor size and local invasiveness, tumor grade, malignant peripheral nerve sheath tumor (MPNST) histology, and distant metastases were the main prognostic factors.While the limbs are the most common sites of NRSTS and are often characterized by a more favorable prognosis than for axial tumors, the clinical features of extremity RMS often differ from those of RMS of other sites, with a higher incidence of unfavorable prognostic factors (e.g., alveolar subtype) and consequently unsatisfactory treatment results. The treatment of these patients is complex and necessarily multidisciplinary, and it demands not only adequate experience of treating children and adolescents in clinical trials, but also particular skills in the field of orthopedic surgery.
| S-EPMC2656726 | biostudies-other
Project description:BackgroundThe quality of surgical margins is the most important factor affecting local control in soft tissue sarcoma (STS). Despite this, there is no universally accepted consensus on the definition of an adequate surgical margin or on which patients should be offered radiation therapy. This study focuses on local control and its prognostic factors in patients with trunk wall and extremity STS.MethodsAdult patients with a final diagnosis of trunk wall or extremity STS referred to a single tertiary referral centre between August 1987 and December 2016 were identified from a prospective institutional database. Patients were treated according to a protocol instituted in 1987. The classification of surgical margins and indications for radiation therapy were based on anatomy and strict definition of surgical margins as metric distance to the resection border. Local treatment was defined as adequate if patients received either surgery with wide margins alone or marginal surgery combined with radiation therapy. Margins were considered wide if the tumour was excised with pathological margins greater than 2.5 cm or with an uninvolved natural anatomical barrier. After treatment, patients were followed up with local imaging and chest X-ray: 5 years for high-grade STS, 10 years for low-grade STS.ResultsA total of 812 patients were included with a median follow-up of 5.8 (range 0.5-19.5) years. Forty-four patients had a grade 1 tumour: there were no instances of recurrence in this group thus they were excluded from further analysis. Five-year local control in the 768 patients with grade 2-3 STS was 90.1 per cent in patients receiving adequate local treatment according to the protocol. Altogether, 333 patients (43.4 per cent) were treated with wide surgery alone and their 5-year local control rate was 91.1 per cent. Among patients treated with wide surgery alone, deep location was the only factor adversely associated with local relapse risk in multivariable analysis; 5-year local control was 95.3 per cent in superficial and 88.3 per cent in deep-sited sarcomas (hazards ratio 3.154 (95% c.i. 1.265 to 7.860), P = 0.014).ConclusionA high local control rate is achievable with surgery alone for a substantial proportion of patients with STS of the extremities or superficial trunk wall.
| S-EPMC10144696 | biostudies-literature
Project description:Due to the heterogeneity of soft tissue sarcomas (STS), the choice of the proper perioperative treatment regimen is challenging. Neoadjuvant therapy has attracted increasing attention due to several advantages, particularly in patients with locally advanced disease. The number of available neoadjuvant modalities is growing continuously. We may consider radiotherapy, chemotherapy, targeted therapy, radiosensitizers, hyperthermia, and their combinations. This review discusses possible neoadjuvant treatment options in STS with an emphasis on available evidence, indications for each treatment type, and related risks. Finally, we summarize current recommendations of the STS neoadjuvant therapy response assessment.
| S-EPMC7464514 | biostudies-literature
Project description:BackgroundSoft tissue sarcomas (STS) are a heterogeneous group of rare tumours that represent less than 1% of all malignant, solid tumours in adults. There is limited epidemiological information regarding STS in Latin America. Therefore, the objective of this study is to present an epidemiological profile of these tumours observed at a single reference centre.MethodsA retrospective study was carried out based on hospital records obtained from a registry of 879 patients with STS of the extremities who were treated at the National Cancer Institute of Mexico from January 1, 1994 to December 31, 2017. Epidemiological variables and relevant clinical data were collected. Five-year survival rates were analysed using Kaplan-Meier estimates, and a multivariate Cox proportional-hazards model measured associations.ResultsA total of 879 records were collected. The median age was 45 years (15-95 years), and the ratio of men to women was 1:1, with 433 men (49.3%), and 446 women (50.7%). The median tumour size was 11.4 cm (2-49 cm). The most prevalent histological variants were liposarcomas and synovial sarcomas. The lower limb was the most frequently affected extremity, with the thigh being the most common site followed by the leg. A majority of the patients were diagnosed at clinical stages IIIA-IV.ConclusionsThe data collected from the present cohort provides an overview of the epidemiological profile of STS at a single reference centre in Latin America, and allow comparison with global data.
| S-EPMC8079324 | biostudies-literature
Project description:Background The aim of the study was to evaluate the management, toxicity and treatment responses of patients treated with neoadjuvant radiotherapy (NART) for soft tissue sarcomas (STS) and to analyse the potential of radiomic features extracted from computed tomography (CT) scans. Materials and methods This is a retrospective and exploratory study with patients treated between 2006 and 2019. Acute and chronic toxicities are evaluated. Local progression free survival (LPFS), distant progression free survival (DPFS) and overall survival (OS) are analysed. Radiomic features are obtained. Results A total of 25 patients were included. Median follow-up is 24 months. Complications in surgical wound healing were observed in 20% of patients, chronic fibrosis was documented as grade 1 (12%) and grade 2 (12%) without grade 3 events and chronic lymphedema as grade 1 (8%) and grade 2 (20%) without grade 3 events. Survival variables were LPFS 76%, DPFS 62% and OS 67.2% at 2-year follow-up. CT radiomics features were associated significantly with local control (GLCM-correlation), systemic control (HUmin, HUpeak, volume, GLCM-correlation and GLZLM-GLNU) and OS (GLZLM-SZE). Conclusions STS treated with NART in our centre associate with an OS and toxicity comparable to other series. CT radiomic features have a prognosis potential in STS risk stratification. The results of our study may serve as a motivation for future prospective studies with a greater number of patients.
| S-EPMC8575355 | biostudies-literature
Project description:BackgroundTo identify the clinical and imaging characteristics of soft tissue sarcomas (STS) of the trunk and extremities that undergo unplanned excision.MethodsThis retrospective study evaluated the data of patients with STS in the trunk or extremities between January 2008 and December 2021. Patients were divided into unplanned and planned excision groups based on their initial treatment. The distribution of histologic subtypes was analyzed, and the magnetic resonance imaging features were evaluated. Multivariable logistic regression was performed to identify variables independently associated with unplanned excision.ResultsA total of 305 patients (mean age±standard deviation, 51±16.4 years; 179 males) were analyzed. The most prevalent subtype in the unplanned excision group was myxofibrosarcoma (22.3%). The unplanned excision group had a significantly smaller tumor size (p < 0.001) and more frequent superficial (p < 0.001) locations. Lobulated shape and peritumoral abnormalities were present in 70.0% and 50.0% of the unplanned excision group, respectively. Tumor size (adjusted odds ratio [OR], 0.87 per 1 cm increase; 95% confidence interval [CI], 0.77-0.98; p = 0.025) and superficial location (adjusted OR, 3.48; 95% CI, 1.57-7.72; p = 0.002) were independently associated with unplanned excision.ConclusionMyxofibrosarcoma is associated with a high frequency of unplanned excision. A significant number of patients in the unplanned excision group demonstrated a lobulated shape and peritumoral abnormalities. Only small tumor size and superficial location were independently associated with unplanned STS excision.
| S-EPMC11620571 | biostudies-literature
Project description:Background and purposeIn this work, we validate a texture-based model computed from positron emission tomography (PET) and magnetic resonance imaging (MRI) for the prediction of lung metastases in soft-tissue sarcomas (STS). We explore functional imaging at different treatment time points and evaluate the feasibility of radiotherapy dose painting as a potential treatment strategy for patients with higher metastatic risk.Materials and methodsWe acquired fluorodeoxyglucose (FDG)-PET, fluoromisonidazole (FMISO)-PET, diffusion weighting (DW)-MRI and dynamic contrast enhanced (DCE)-MRI data for 18 patients with extremity STS before, during, and after pre-operative radiotherapy. We tested the lung metastases prediction model using pre-treatment images. We evaluated the feasibility of dose painting using volumetric arc therapy (VMAT) via treatment re-planning with a prescription of 50 Gy to the planning target volume (PTV50Gy) and boost doses of 60 Gy to the FDG hypermetabolic gross tumour volume (GTV60Gy) and 65 Gy to the low-perfusion DCE-MRI hypoxic GTV contained within the GTV60Gy (GTV65Gy).ResultsThe texture-based model for lung metastases prediction reached an area under the curve (AUC), sensitivity, specificity and accuracy of 0.71, 0.75, 0.85 and 0.82, respectively. Dose painting resulted in adequate coverage and homogeneity in the re-planned treatments: D95% to the PTV50Gy, GTV60Gy and GTV65Gy were 50.0 Gy, 60.3 Gy and 65.4 Gy, respectively.ConclusionsTextural biomarkers extracted from FDG-PET and MRI could be useful to identify STS patients that might benefit from dose escalation. The feasibility of treatment planning with double boost levels to intratumoural GTV functional sub-volumes was established.
| S-EPMC7807871 | biostudies-literature