Search Results (110)

Soft-tissue sarcomas (STSs) are rare malignancies predominantly found in the extremities. Surgery and radiation are standard treatments, but post-operative pulmonary surveillance, involving clinical visits and thoracic imaging, is crucial due to a high recurrence rate, most commonly to the lungs. Current pulmonary surveillance guidelines lack robust evidence. The Surveillance AFter Extremity Tumor SurgerY (SAFETY) randomized controlled trial is designed to determine the impact of pulmonary surveillance frequency (every three versus six months) and chest imaging modality (CXR versus CT) on patient-important outcomes. The pilot phase assessed feasibility of patient enrolment, protocol adherence, and data quality, as well as aggregate outcomes at two years of follow-up. 100 patients were enrolled from 300 screened patients across 17 international sites. Minor protocol deviations were common. Follow-up, data completeness and data quality met the progression criteria of 85%. Of the 100 patients, 15 died, 21 had metastases, seven had local recurrence and 30 experienced at least one serious adverse event. This SAFETY trial study established feasibility of enrolment and data quality, and confirmed the need to emphasize protocol adherence in sarcoma care. The results of this trial are expected to provide crucial evidence to standardize STS pulmonary surveillance practices, ultimately improving patient management and expectations. Full article

Background: Local recurrence for high-risk extremities/trunk soft tissue sarcoma (STS) after treatment can range from 15 to 30%. The modified Eilber protocol (MEP) using low-dose intravenous chemotherapy with a reduced dosage of radiation in the preoperative setting has demonstrated excellent local control and reduced wound complications in these patients. The aim of the current study was to assess long-term local control and overall survival in patients with STS treated with the MEP versus standard preoperative or postoperative radiotherapy. Methods: Patients diagnosed with STS from 2004 to 2016 were identified using the Alberta Cancer Registry. Patients with STS treated with the MEP, preoperative or postoperative radiotherapy, were included. Patient and tumor characteristics, treatments and outcomes were abstracted from the registry and primary chart review. Characteristics were compared using one-way ANOVA for continuous variable and chi-square test and Fisher test for the categorical outcomes. Local recurrence-free survival and overall survival were analyzed using Kaplan–Meier Analysis with Log-rank test. Results: A total of 242 patients with STS were included, among which 100 (41.3%) received the MEP prior to surgery, 91 (37.6%) had preoperative radiation, and 51 (21.1%) had postoperative radiation. After a median follow up of 4.9 years, there were no significant differences in local recurrence or local recurrence-free survival between patients treated with the MEP vs. preoperative or postoperative radiotherapy (10 vs. 6.6% and 7.8%, respectively, p-value NS). There were also no significant differences between groups for recurrence-free survival and overall survival. Conclusions: This study demonstrates that the use of the MEP has non-inferior oncologic outcomes compared to standard preoperative or postoperative radiation in a population-based analysis despite reducing the overall dosage of radiation administered. The modified Eilber preoperative chemoradiation protocol may be considered as an additional option for patients with STS. Full article

Objectives: The management of soft tissue sarcoma in patients aged ≥ 85 years remains a clinical dilemma, as evidence to guide treatment decisions in this population is limited. Here, we aimed to compare the clinical characteristics of patients aged ≥ 85 years who underwent surgery with those who did not, to identify factors influencing surgical decision-making. We also assessed the oncological outcomes and postoperative complications in the patients. Methods: We reviewed 37 consecutive patients with localized disease involving either the extremities or trunk wall, with a median age of 89.0 years. No significant differences in demographics or clinical characteristics were observed between patients who underwent surgery and those who received conservative management. Among the 37 patients, 25 underwent surgery with curative intent. Results: The two-year overall survival rate among surgically treated patients was 77%, with local recurrence-free survival and metastasis-free survival rates of 77% and 57%, respectively. Postoperative complications occurred in approximately one-third of the cases. Compared to those without complications, patients with complications were older (p = 0.025), had poorer performance status (p = 0.017), were likely to have trunk involvement (p = 0.01), and had larger tumor sizes (p = 0.025). No significant prognostic factors for overall survival were identified. Conclusions: While surgery is not without risk, our results can provide useful information for both physicians and patients to discuss and explain possible outcomes with surgical treatment. Full article

Malignant gastrointestinal neuroectodermal tumor (MGNET) and clear cell sarcoma (CCS) of soft tissue represent related, extremely rare, malignant mesenchymal neoplasms. Both entities are genetically characterized by the same molecular alterations, EWSR1::CREB1 fusions. Malignant gastrointestinal neuroectodermal tumor has significant morphological overlap with CCS, although it tends to lack overt features of melanocytic differentiation. Recently, rare MGNET cases were reported in extragastrointestinal sites. The diagnosis represents a major challenge and significantly impacts therapeutic planning. In this study, we reported the clinicopathologic features of a molecularly confirmed MGNET of the neck and provided a review of the pertinent literature. Full article

Background and Clinical Significance: Undifferentiated pleomorphic sarcoma (UPS) is a highly malignant soft tissue tumor formerly known as malignant fibrous histiocytoma. In the fifth edition of the WHO classification (2020), UPS is classified as an undifferentiated/unclassifiable sarcoma diagnosed via exclusion. While UPS commonly occurs in the extremities, its incidence in the head and neck region is rare (3%), with only a few reported cases in the oropharynx. Surgical resection is the primary treatment; however, tumors at the tongue base pose significant challenges due to the complex anatomy and the presence of critical neurovascular structures. This case highlights a rare instance of tongue-base UPS successfully treated with transoral videolaryngoscopic surgery (TOVS), demonstrating its feasibility as a minimally invasive approach. Case Presentation: A 68-year-old male presented with pharyngeal discomfort, dysphagia, and nocturnal dyspnea. Clinical examination revealed a pedunculated tumor originating from the left tongue base, occupying the pharyngeal cavity. Imaging studies showed a 5 cm mass without lymph node metastasis. A biopsy confirmed UPS (cT3N0M0). Given the tumor’s characteristics, TOVS was performed using an FK-WO TORS laryngo-pharyngoscope retractor. The tumor was resected with a ≥10 mm margin, achieving complete histological resection. The patient’s dyspnea resolved immediately, and oral intake resumed the next day. No adjuvant radiotherapy was administered, and no recurrence was observed for 50 months. Conclusions: This is the first reported case of UPS of the tongue base successfully resected using TOVS. This minimally invasive approach provides a safe and effective alternative for managing oropharyngeal UPS. Full article

Sarcomas are rare heterogenous mesenchymal tumors with over seventy-five different subtypes, with varying biology and outcomes, with no clear inciting factor in the vast majority. To determine the prevalence of pathogenic germline variants (PGV) in patients with sarcomas, we undertook a prospective multi-site study of germline sequencing using an 84-gene next-generation sequencing panel among patients receiving care at the four Mayo Clinic Cancer Centers. Of 115 patients with bone and soft tissue sarcoma, the median age was 60 years, 49.6% were female, 82.6% were White. The anatomical location of the primary tumor included extremities (34.8%), retroperitoneum (19.1%), trunk (13.0%), and head and neck (7.8%). Family history of cancer was present in 62.6% of the study population. Ten patients (8.7%) had a pathogenic/likely pathogenic variant (PGV). Of these, three had stage IV sarcoma, and seven had earlier-stage sarcoma (stages I–III). Among the 55 (48.7%) patients who had variant of uncertain significance (VUS), 41.1% (22/55) had stage IV sarcoma and 58.9% (33/55) had earlier-stage disease. Of the ten patients with PGV, high-to-moderate penetrance gene abnormalities were identified in eight patients (80%) involving TP53 (3), BRCA1 (1), SDHA (1), ATM (2), and NBN (1) genes. The vast majority of the PGVs (70%) would not have been detected using the current guidelines. Because of the paucity of sarcomas and lack of effective treatment options for advanced disease, germline testing in sarcomas represents a potentially impactful strategy to assess therapeutic options and for assessment of familial risk. Full article

Clear Cell Sarcoma (CCS) of soft tissue is a rare and highly malignant neoplasm primarily affecting young adults, often presenting in the deep soft tissues of the extremities. Despite morphological and immunophenotypic similarities to melanoma, CCS arises from connective tissues and is characterized by a distinct genetic hallmark: the EWSR1-ATF1 fusion resulting from t(12;22)(q13;q12) translocation. This genetic signature is absent in melanoma, making molecular diagnosis essential for accurate differentiation. Additionally, recent evidence highlights the utility of PRAME as an immunohistochemical marker to distinguish CCS from melanoma and other neoplasms. Clinically, CCS commonly involves tendons and aponeuroses, with metastatic potential leading to poor prognoses despite optimal local disease management. Histologically, CCS features lobular growth, spindle-to-epithelioid cells with clear cytoplasm, and low mitotic activity, often necessitating a multimodal diagnostic approach incorporating histopathology, immunohistochemistry, and molecular testing. Therapeutically, wide surgical excision remains the cornerstone for localized disease, with sentinel lymph node biopsy aiding in staging. Adjuvant radiotherapy is considered in select cases, while chemotherapy has limited efficacy in metastatic settings. Emerging treatments, including targeted therapies focusing on EWSR1-ATF1-driven pathways and immune checkpoint inhibitors, offer hope for improved outcomes. This review synthesizes current knowledge on CCS, emphasizing diagnostic challenges, the role of PRAME, and advancements in therapeutic strategies to enhance patient care. Full article

Background: The clinical outcome of inoperable sarcoma patients treated with LATTICE (LRT) is limited and therefore the objective of our study was to report treatment response, overall survival (OS), local-recurrence free survival (LRFS) and toxicity. Methods: This retrospective observational study includes 15 histologically proven inoperable non-extremity sarcoma patients with no treatment options or no response to systemic therapy, treated at our institution between 2020 and 2024. The patients were treated with a combination of LRT and normo- or hypo-fractionated external beam radiotherapy. Treatment response was evaluated by RECIST1.1 criteria, toxicity by CTCAE 5.0 and OS and LRFS by Kaplan–Meier curves. Results: The median follow-up (F-UP) since the beginning of the treatment was 10 months (range 4–32). Nine patients were male and six female. Their mean age was 60 years. The median gross tumor volume (GTV) was 1058 cm³ (range 142–6103 cm³). The median number of spheres was 9 (4–30). All patients with symptoms reported symptoms’ relief. Based on RECIST1.1 criteria, 10 patients (67%) had stable local disease at 1–2 months F-UP on computed tomography (CT). Surgical resection was feasible in five patients. Three of them are alive without disease and two died due to metastatic progression. From 10 (67%) non operated patients, 5 patients died (50%) due to disease. The remaining five patients (50%) are alive, three with stable disease at 21, 22, and 32 months of F-UP and two with disease progression who are currently receiving palliative chemotherapy treatment. Reported G2 toxicity was as follows: gastrointestinal (2), asthenia (1). Two patients had G3 toxicity: esophagitis (1) and inguinal dermatitis (1). No acute or chronic G4–G5 toxicity was observed. Conclusions: LRT is a feasible and well-tolerated radiation technique for inoperable bulky soft-tissue sarcomas. Further studies are needed to establish protocols to determine which patients could benefit from palliative or preoperative treatment. Full article

Background and Objectives: The primary objective of surgeons treating bone and soft tissue sarcomas (STS) is to achieve optimal local tumor control, ensuring a tumor-free margin and preventing local recurrence. However, the impact of surgical resection margin status on extremity STSs remains an area that requires further exploration. Therefore, this study aims to investigate the effects of surgical resection margin status on both local recurrence and overall survival rates. Materials and Methods: One hundred and eighty-five patients who underwent surgical resection with a diagnosis of soft tissue sarcoma were studied. The study recorded patient demographics, tumor characteristics, surgical margin distance (in millimeters), and disease-related outcomes. Results: The minimum follow-up period was 24 months (24–168). The mean time to local recurrence after resection was 103.2 months (95% CI 91.73 to 114.64). The mean local recurrence-free survival was found to be 7.23 months in patients with positive surgical margins, 87.42 months in patients with ≤1 mm, and 139.80 months in patients with >1 mm (p < 0.001). Patients with surgical margins ≤1 mm were more likely to have local recurrence than patients with >1 mm (0.41 [0.21–0.81], p = 0.010). The mean overall survival was 106.72 months (95% CI 95.98 to 117.46). Positive surgical margins were associated with decreased overall survival (3.58 [1.46–8.80], p = 0.005). There was a statistically significant difference between the histologic grade in terms of local recurrence (4.50 [95% CI 2.57 to 7.88]; p < 0.001) and overall survival (3.12 [95% CI 1.52 to 6.41]; p = 0.002). Conclusions: Achieving a negative surgical margin distance of more than 1 mm appears to be correlated with a reduced risk of local recurrence. Positive surgical margins are a risk factor that detrimentally impacts overall patient survival. However, determining the appropriate margin distance for all patients poses a significant challenge. Full article

Background: Soft-tissue sarcomas (STSs) are rare and challenging to diagnose due to their heterogeneous presentation. In 2009, Denmark introduced the Cancer Patient Pathways for sarcomas (CPPs) to improve sarcoma treatment by streamlining diagnostic and therapeutic processes. The primary objective of this study is to evaluate the impact of the CPPs on the overall survival of patients with deep-seated, high-grade STSs, comparing outcomes from before and after CPP implementation. Methods: A retrospective cohort study was conducted using data from 712 patients diagnosed with high-grade STSs in the extremities or trunk wall between 2000 and 2018. Patients were grouped into pre-CPP (2000–2008) and post-CPP (2010–2018) cohorts. Overall survival was analyzed using Kaplan–Meier estimates. Results: The five-year overall survival improved from 43% in the pre-CPP cohort to 52% post-CPP (p = 0.05). Time-to-treatment was significantly reduced in the post-CPP cohort, with a median decrease of 3 days (18 vs. 15 days, p < 0.001). We found only a very weak tendency toward larger tumor sizes in the pre-CPP cohort and no difference regarding the percentage of patients that had distant metastases at diagnosis between cohorts. In the post-CPP cohort, the percentage of whoops operations decreased and the use of oncological services increased. Conclusions: After the introduction of the CPPs for the sarcoma patients, overall survival improved and time to treatment was reduced. This study highlights the importance of efficient referral pathways in improving cancer outcomes but cannot exclude that other factors could also have contributed. Full article

Background/Objectives: The historically most commonly used preoperative radiotherapy regimen for soft tissue sarcomas (STSs) consists of 50 Gray (Gy) delivered in 25 fractions over 5 weeks, achieving excellent local control, but with significant challenges due to prolonged treatment duration and early side effects. Reducing therapy duration while maintaining optimal local and distant control would be highly beneficial for patients. We aimed to investigate the outcome of an ultrahypofractionated radiotherapy (uhRT) regimen which may represent a shorter and more patient-friendly alternative. Methods: This multi-center, open-label, phase 2 clinical trial with a clustered cohort design was conducted within the Swiss Sarcoma Network (SSN). Adult patients (aged ≥ 18 years) with STS of the extremities or superficial trunk and an Eastern Cooperative Oncology Group (ECOG) performance status of 0–3 were included. Participants were assigned to either normofractionated radiotherapy (nRT) at 50 Gy in 25 fractions or uhRT at 25 Gy in 5 fractions. Data were collected prospectively in real-world-time clinical settings. The primary outcome was local recurrence-free survival (LRFS), with overall survival (OS) and wound complications as secondary outcomes. Results: Between March 2020 and October 2023, 138 patients were included in the study; 74 received nRT and 64 received uhRT. The median follow-up times were 2.2 years for uhRT and 3.6 years for nRT. The LRFS rates at 1 year were 97.0% for nRT and 94.8% for uhRT (p = 0.57). The two-year LRFS rates were 91.9% and 94.8%, respectively (p = 0.57). The one- and two-year OS rates were 97.1%/86.3% and 98.2%/88.8%, respectively (p = 0.72). The wound complication rate was comparable between the nRT (12.0%) and uhRT (12.5%) groups (p = 0.99). Conclusions: UhRT for STSs offers an effective and safe alternative to traditional nRT, with comparable early LRFS, OS and wound complication rates. Given the two-year median follow-up, which is critical for evaluating local recurrence, uhRT shows promise as a shorter and more convenient treatment regimen. UhRT may be a safe and effective alternative treatment option to traditional nRT. Full article

Background/Objectives: Soft tissue sarcomas (STSs) are extremely uncommon tumors with a high rate of local recurrence that often require very demolitive surgery. The aim of our study is to propose a specific rehabilitation protocol for patients with STSs, based on the kind of demolition and reconstructive surgery performed, and evaluate its effects. Methods: The protocol was developed on the basis of the clinical experiences of physiatrists and surgeons, as well as data from the literature, recommending timelines for postural steps, verticalization, walking, and therapeutic exercises, in accordance with wound healing times and in order to prevent complications from disuse and immobility. The modified Barthel Index Scale (mBI), the Numeric Rating Scale (NRS), the Adapted Patient Evaluation Conference System (APECS), and the 10 Meter Walk Test (10 MWT) were used to clinically evaluate patients before and after the rehabilitation treatment. Results: Thirty-one patients with primary STSs were enrolled. Following the rehabilitation program, we found a statistically significant improvement in mBI (p < 0.001), APECS (p ≤ 0.001), and NRS (p = 0.001). In a subgroup of patients (n = 18) assessed with the 10 MWT, a statistically significant increase in walking speed was observed (p = 0.012). Conclusions: Patients who completed rehabilitation following the proposed protocol, customized according to the surgical intervention type, demonstrated marked improvements in motor performance, ability in daily activities, walking, and pain. The proposed protocol can assist the multidisciplinary team of surgeons, oncologists, and rehabilitators in maintaining alignment on patient management, thereby ensuring clear indications regarding the activities that patients can and cannot undertake during the recovery period. Full article

Soft tissue sarcomas are defined as relatively rare, wide-ranging mesenchymal tumors that present several forms of aggressive behavior. In order to improve the therapeutic result and thus the patient's prognosis, reliable diagnostic and evaluation tools are needed, capable of establishing the evolutive pattern of each patient. Materials and Methods. An analytical observational study was conducted in the period 2016–2023 in the Orthopedics Department of the Bucharest University Emergency Hospital, on patients diagnosed with soft tissue sarcoma. Data such as sex, age, site, size, depth, histotype, grade and margin status, vessel or bone involvement, adenopathies, adjuvant therapy, clinical findings, etc., were analyzed. Diagnosis included immunohistochemistry after macroscopic pathological findings were carefully reviewed by a dedicated pathologist. A molecular study was performed to increase the accuracy of diagnosis, prognosis and clinical management of selected sarcomas. Results. In adult soft tissue sarcomas, histotype has been reported to be a significant predictor of overall survival. In terms of survival rate, vascular invasion appears to be a significant pathological factor for progression in extremity STS. Even though the local control of the disease has improved, the development of systemic metastases seems to be largely influenced by the biological characteristics of the tumor. Conclusions. Significant prognostic factors for the likelihood of a lower survival and death rate are tumor size, tumor depth, histology type, and vascular invasion. The results of the study support and complement the literature data, thus improving the understanding of the prognosis of soft tissue sarcomas. Full article

Two national sarcoma centers have had different approaches for selecting patients with grade 2–3 deep-seated soft tissue sarcomas (STS) for postoperative radiotherapy (RT). We evaluated potential differences in local recurrence in patients treated at the two centers. At Sarcoma Center 1 (SC1), RT was the standard treatment for all tumors except certain small tumors excised with a margin wider than 1 cm. Sarcoma Center 2 (SC2) avoided RT for tumors regardless of tumor size if removed with a margin wider than 1 cm and/or a solid barrier. We included 386 patients (SC1/SC2 = 196/190) over 18 years of age diagnosed with a non-metastatic grade 2–3, deep-seated STS of the extremities or trunk wall, who underwent primary surgical treatment (only tumors excised with a negative margin) from 1 January 2000, to 31 December 2016. Kaplan–Meier survival analysis, competing risk analysis, and cause-specific Cox regression were applied. A total of 284 patients received primary RT, 163 (83%) at SC1 and 121 (64%) at SC2 (p < 0.001). The cumulative incidence of local recurrence at five years was 15% (95% CI: 10–19%) at SC1 and 14% (95% CI: 9–19%) at SC2. Multivariate analysis showed no significant differences in local recurrence between the centers. We concluded that when entering all available patients into the analysis using an intention-to-treat principle, a more selective approach to postoperative RT in patients with grade 2–3 deep-seated STS did not lead to a higher local recurrence rate. However, with this study design, we cannot rule out if the local recurrence rate could have been lower if RT was administered to all tumors removed with a margin wider than 1 cm and/or a solid barrier. Full article

Mesenchymal tumors originate from mesenchymal cells and can be either benign or malignant, such as bone, soft tissue, and visceral sarcomas. Surgery is a cornerstone treatment in the management of mesenchymal tumors, often requiring complex procedures performed in high-volume referral centers. However, the COVID-19 pandemic has highlighted this need for alternative non-surgical approaches due to limited access to surgical resources. This review explores the role of non-surgical treatments in different clinical scenarios: for improving surgical outcomes, as a bridge to surgery, as better alternatives to surgery, and for non-curative treatment when surgery is not feasible. We discuss the effectiveness of active surveillance, cryoablation, high-intensity focused ultrasound, and other ablative techniques in managing these tumors. Additionally, we examine the use of tyrosine kinase inhibitors in gastrointestinal stromal tumors and hypofractionated radiotherapy in soft tissue sarcomas. The Sarculator tool is highlighted for its role in stratifying high-risk sarcoma patients and personalizing treatment plans. While surgery remains the mainstay of treatment, integrating advanced non-surgical strategies can enhance therapeutic possibilities and patient care, especially in specific clinical settings with limitations. A multidisciplinary approach in referral centers is vital to determine the optimal treatment course for each patient. Full article