Search Results (57)

Background: Retroperitoneal leiomyosarcomas are aggressive malignancies. Complete surgical resection with negative margins is crucial to decrease the risk of recurrence but can be risky due to vascular involvement. The aim of our study was to evaluate the different approaches to IVC and renal vein management and their impact on postoperative complications. Methods: We performed a retrospective review of patients who underwent surgery for retroperitoneal leiomyosarcoma with IVC and/or renal vein involvement at our institution from 2016 to 2024. Patients were stratified by intraoperative vascular management, including ligation only versus varying forms of vascular reconstruction. Postoperative complications, including bleeding, transfusions, the need for acute and chronic hemodialysis, and thromboembolic events, were recorded. Chi-squared tests were used to compare rates of postoperative complications by vascular management. A p-value of 0.05 was considered statistically significant. Results: We identified 60 patients at our institution who underwent surgery for leiomyosarcoma with IVC and/or renal vein involvement. Ten patients underwent IVC ligation alone due to thrombosis, thirty-six had IVC replacement, and fourteen had patch angioplasty. In the entire cohort, twenty-six patients (43.3%) experienced an adverse event after surgery. When looking at postoperative adverse events by IVC management, we did not find any statistically significant differences among rates of adverse events by group. There were also no statistically significant differences in complications following renal vein ligation versus renal vein reconstruction. Conclusions: Patients with leiomyosarcoma with IVC and/or renal vein involvement have several options for intraoperative vascular management. Our data demonstrates that there are no statistically significant differences in rates of complications among the different groups. Full article

Retroperitoneal sarcomas (RPS) are rare tumours, primarily treated with surgical resection. However, recurrences are frequent. Combining clinical factors with CT-derived radiomic features could enhance treatment stratification and personalization. This study aims to assess whether radiomic features provide additional prognostic value beyond clinicopathological features in patients with high-risk RPS treated with preoperative radiotherapy. This retrospective study included patients aged 18 or older with non-recurrent and non-metastatic RPS treated with preoperative radiotherapy between 2008 and 2016. Hazard ratios (HR) were calculated using Cox proportional hazards regression to assess the impact of clinical and radiomic features on time to event outcomes. Predictive accuracy was assessed with c-statistics. Radiomic analysis was performed on the high-risk group (undifferentiated pleomorphic sarcoma, well-differentiated/de-differentiated liposarcoma or grade 2/3 leiomyosarcoma). Seventy-two patients were included, with a median follow-up of 3.7 years, the 5-year overall survival (OS) was 67%. Multivariable analysis showed older age (HR: 1.3 per 5-year increase, p = 0.04), grade 3 (HR: 180.3, p = 0.02), and larger tumours (HR: 4.0 per 10 cm increase, p = 0.02) predicted worse OS. In the higher-risk group, the c-statistic for the clinical model was 0.59 (time to distant metastasis (TDM)) and 0.56 (OS). Among 27 radiomic features, kurtosis improved OS prediction (c-statistic 0.69, p = 0.013), and Neighbourhood Gray-Tone Difference Matrix (NGTDM) busyness improved it to 0.73 (p = 0.036). Kurtosis also improved TDM prediction (c-statistic 0.72, p = 0.023). Radiomic features may complement clinicopathological factors in predicting overall survival and time to distant metastasis in high-risk retroperitoneal sarcoma. These exploratory findings warrant validation in larger, multi-institutional studies. Full article

Background/Objectives: Surgery remains the mainstay of treatment for retroperitoneal sarcoma (RPS); however, definitive therapeutic strategies for patients with insufficient surgical margins and unresectable disease owing to locally advanced RPS remain unclear. Carbon ion radiotherapy (CIRT) has been employed in patients with unresectable RPS. This study aimed to evaluate the effectiveness of CIRT in this patient population. Methods: A retrospective analysis was conducted in 76 patients with unresectable RPS treated with CIRT. Of these, 95% had a confirmed prognosis until 2022. In 74 patients, the prescribed relative biological effectiveness dose was 70.4 Gy, delivered in 16 fractions over 4 weeks. Respiratory gating was used, and spot scanning irradiation has been performed in all patients since 2016. Results: The 3- and 5-year overall survival rates for the entire cohort were 68.3% and 49.4%, respectively, with a median overall survival time of 58.1 months. The 3- and 5-year local control rates were 79.0% and 72.0%, respectively. Among 47 naïve patients with treatment-naïve tumors, the 3- and 5-year abdominal recurrence-free survival rates were 51.1% and 29.1%, respectively. Late adverse events of grade 3 or higher occurred in 4 (5.2%) patients. Conclusions: CIRT represents a definitive treatment option for patients with unresectable RPS. In the future, multicenter studies should be conducted to evaluate the effectiveness of CIRT for RPS in larger patient cohorts. Full article

For patients with retroperitoneal sarcomas (RPSs), en-bloc resection with macroscopically negative margins remains the only potentially curative treatment. Textbook outcomes (TOs) are composite measures developed to compare ideal surgical outcomes for complex oncologic resections. The aims of this study were as follows: to define TO for RPS resections; to investigate the impact of treating service and other variables on TO; and to investigate the impact of treating service on achieving a TO. Population-based data from the Queensland Oncology Repository (QOR) was used to perform a retrospective review of all adult patients who underwent resection for primary RPS in Queensland between 2012 and 2022. TO was defined as follows: en-bloc resection; macroscopically negative margins; no unplanned ICU admission, no Clavien–Dindo III or greater complications; hospital length of stay of 14 days or less; no readmission within 30 days; and no 90-day mortality. A TO was achieved in 82 (56.94%) of the 144 patients included in the study. A high-grade histological subtype, the resection of three or more contiguous organs, major vascular resection and treatment outside of a high-volume sarcoma centre (HVSC) were significant negative predictors of achieving TOs (p < 0.05). On multivariate analysis, treatment at a high-volume sarcoma centre was independently associated with a 2.6-fold increase in TO (1.18–5.88, p = 0.02). Achieving a TO was associated with higher five-year DFS (61.5% vs. 41.3%, p = 0.03) and OS (76% vs. 59.4%, p = 0.02). In our state, TOs provide a measure of the quality of RPS resection across multiple health services, with patients treated at high-volume sarcoma centres more likely to achieve a TO. TO rates are associated with improved five-year DFS and OS. Full article

Background: There is an ongoing debate about the role of perioperative chemotherapy in retroperitoneal sarcoma (RPS). The aim of this study was to evaluate the effectiveness of perioperative chemotherapy in subtypes of RPS that are considered chemosensitive, including retroperitoneal angiosarcoma, undifferentiated pleomorphic sarcoma, myxoid liposarcoma, spindle cell sarcoma, and synovial sarcoma. Methods: This is a population-based retrospective cohort study. Patients with localized retroperitoneal sarcoma who underwent surgery were included from the US National Cancer Database (NCDB). After propensity score matching for the factors age, sex, grade, margin status, and tumor size, multivariable logistic and Cox regression analyses were used to identify factors associated with systemic therapy and their potential impact on the survival of patients with localized RPS. Results: We included 851 patients who underwent surgery between 2004 and 2020 (85% white, 41% female, and mean age 62 years). Of those, 227 patients (26.7%) received perioperative chemotherapy. In multivariable logistic regression, age ≤ 60 and tumor grading GIII/IV vs. GI/II were associated with a higher probability of receiving perioperative chemotherapy. After propensity score matching, we detected no difference in overall survival between patients who received chemotherapy and those who did not (HR 0.89, CI 0.55–1.43; and log-rank p = 0.92). Patient age ≤80 and tumor grading GI/II vs. GIII/IV were associated with improved overall survival. Conclusions: In this large analysis, the use of perioperative chemotherapy was not associated with improved survival in rare, chemosensitive subtypes of retroperitoneal sarcoma. However, selection bias must be considered when interpreting these findings. Full article

Background: RPS are rare tumors requiring complex surgery. The impact of postoperative complications on long-term outcomes is still debated. This study aimed to identify predictors of severe complications and assess their influence on overall survival (OS) and disease-free survival (DFS). Methods: We retrospectively analyzed 61 patients who underwent curative surgery for primary RPS between 2013 and 2023 at a tertiary cancer center. Patients with pelvic or recurrent sarcomas were excluded. Severe complications were defined as Clavien–Dindo (CD) grade ≥ 3A. Cox regression and Fine–Gray competing risk models were used for OS and DFS, respectively. Sarculator and P-POSSUM scores were included in multivariable models. Results: Severe complications occurred in 14.8% of patients; 30-day mortality was 1.6%. Predictive factors for complications included longer operative time, gastric resection, intraoperative complications, and transfusions. In multivariable analysis, Sarculator was significantly associated with both DFS (HR 0.97; p = 0.004) and OS (HR 0.97; p = 0.008). The P-POSSUM mortality score predicted OS (HR 1.12; p = 0.002). Severe complications were not independently associated with DFS or OS. Conclusions: In this cohort, severe complications did not impact long-term oncological outcomes. Prognostic tools such as Sarculator and P-POSSUM may enhance risk stratification in RPS surgery. Prospective validation is warranted. Full article

Liver metastases from soft tissue sarcoma (STS) (excluding gastrointestinal stromal tumors) are rare and more commonly arise from retroperitoneal and intra-abdominal primary sites. Chemotherapy remains the mainstay of treatment for disseminated disease, but its effectiveness is limited and patients typically have a dismal prognosis with short survival. However, when metastases are confined to the liver (without pulmonary involvement), some patients may benefit from local techniques, either surgical or nonsurgical, that can provide long periods of disease-free survival. Due to the rarity of STS, especially with liver metastases, and the heterogeneity of histologies and biological behavior, there is a lack of standardized treatment guidelines and universally accepted criteria for this specific setting. To fill this gap, a multidisciplinary working group of experts in sarcoma and liver surgery reviewed the literature and available evidence and developed a set of clinical recommendations to be voted and discussed in the I Ibero-American Consensus on the Management of Metastatic Sarcoma, held at the III Spanish-Portuguese Update Meeting on the Treatment of Sarcomas in May 2024. Herein, the voting results of this meeting and the resulting consensus recommendations are presented, and their applicability, strengths, and limitations are discussed. Full article

Retroperitoneal liposarcoma (RPLPS) is one of the most common histologic subtypes of soft tissue sarcoma (STS). Complete surgical resection remains the mainstay treatment, while the high rate of locoregional recurrence constitutes the predominant cause of mortality. Well-differentiated (WDLPS) and dedifferentiated (DDLPS) liposarcoma are the most frequent subtypes of RPLPS and present amplified MDM2 gene as a hallmark. However, there are few reports evaluating the role of alternatively spliced MDM2 transcripts in RPLPS. In this study, we assessed MDM2-ALT2 expression levels in a cohort of RPLPS patients and evaluated the biological functions of the MDM2-ALT2 isoform in vitro in DDLPS cell lines. Using BaseScope™ and qPCR, we demonstrated that MDM2-Full Length (MDM2-FL) and MDM2-ALT2 expression levels were upregulated in RPLPS patient-derived tissue samples compared to normal adjacent to tumor tissue (NAT). DDLPS cells overexpressing MDM2-FL or MDM2-ALT2 had higher proliferation rates and increased migration and invasion capacities, as well as increased protein levels of p-AKT, mTOR, p70S6K, MMP2, and cJun. Simultaneous overexpression of MDM2-ALT2 and AKT silencing showed that AKT inhibition impaired p-p70S6K and MMP2 protein increased levels and led to significantly decreased proliferation and migration rates compared to cells overexpressing MDM2-ALT2 only. Taken together, our data suggest that MDM2-ALT2 may promote RPLPS progression. 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

Peritoneal sarcomatosis is a rare malignant disease with a poor prognosis, secondary to peritoneal dissemination of abdominopelvic soft tissue sarcomas. Its rarity, together with the characteristic histological heterogeneity and the historically poor response to systemic treatments, has prevented the establishment of widely accepted treatment criteria with curative intent. In this sense, radical cytoreductive surgery (CRS) with peritonectomy procedures and hyperthermic intraperitoneal chemotherapy (HIPEC), widely used in peritoneal carcinomatosis with excellent results, have not had the same evolutionary development in patients with peritoneal sarcomatosis. A multidisciplinary working group of experts in sarcomas and peritoneal oncological surgery established a series of recommendations based on current scientific evidence for the management of peritoneal sarcomatosis, taking into account the different histological subgroups of abdominopelvic sarcomas that can cause it depending on their origin: retroperitoneal sarcomas, uterine sarcomas, and visceral/peritoneal sarcomas of GIST (gastrointestinal stromal tumor) and non-GIST origin. This article shows the results of sarcoma experts’ voting on the recommendations presented during the I Ibero-American Consensus on the Management of Peritoneal Sarcomatosis, which took place during the recent celebration of the III Hispanic-Portuguese Meeting for Updates on the Treatment of Sarcomas. Full article

Synovial sarcomas are soft tissue tumours of uncertain origin, most commonly found in the upper or lower extremities. They are characterised by distinctive chromosomal rearrangements involving the gene SS18. Synovial sarcomas can occasionally arise also in visceral sites, but retroperitoneal SSs are very unusual. Among them, a few primary renal synovial sarcomas have been described in the scientific literature. Primary renal synovial sarcomas tend to be monophasic and often show cystic changes. Histologically, they can closely resemble other primary kidney tumours, mainly paediatric tumours such as nephroblastoma and clear cell sarcoma of the kidney. In the current work, a primary synovial sarcoma of the kidney with unusual morphological features (extensively myxoid stroma and immunohistochemical positivity for BCOR) is described. Molecular analysis, through targeted RNA sequencing, was of invaluable help in reaching the correct diagnosis. Despite locally advanced disease at presentation, the patient showed an unexpectedly brilliant response to chemotherapy. Full article

Histiocytic sarcoma (HS) is an extremely rare but aggressive hematopoietic malignancy, and the prognosis has been reported to be rather unfavorable with a median overall survival of merely 6 months. We presented a 58-year-old female patient complaining of abdominal pain and fever, who was admitted to our institution in September 2021. Fluorine-18-fluorodeoxyglucose (FDG) positron emission tomography–computed tomography (PET/CT) scan showed enlargement of generalized multiple lymph nodes. Subsequently, laparoscopic retroperitoneal lesion biopsy and bone marrow aspiration were performed. The pathological findings indicated the diagnosis of HS concurrent with follicular lymphoma. The immunohistochemistry (IHC) staining of the tumor lesion revealed a high expression of CD38 and PD-L1 proteins. Furthermore, KRAS gene mutation was identified by means of next-generation sequencing. The patient exhibited poor treatment response to both first- and second-line cytotoxic chemotherapies. Therefore, she underwent six cycles of Daratumumab (anti-CD38 monoclonal antibody), Pazopanib (multi-target receptor tyrosine kinases inhibitor) combined with third-line chemotherapy, followed by involved-site radiotherapy and maintenance therapy with the PD-1 inhibitor Tislelizumab. Long-term partial remission was finally achieved after multi-modality treatment. Duration of remission and overall survival reached 22 and 32 months, respectively. Our case indicated that immuno-targeted treatment coupled with chemotherapy and radiotherapy might constitute a potential therapeutic option for HS. Full article

This detailed review focuses on retroperitoneal undifferentiated pleomorphic sarcoma (UPS), a particularly aggressive soft-tissue sarcoma that poses unique diagnostic and therapeutic challenges due to its rarity and complex presentation. By documenting a new case of retroperitoneal UPS and conducting a comprehensive review of all known cases, this article aims to expand the existing body of knowledge on the epidemiology, molecular pathogenesis, and treatment strategies associated with this rare disease. The complexity of diagnosing UPS is emphasized given that it rarely occurs in the retroperitoneal space and its histological and molecular complexity often complicates its recognition. This review highlights the need for specialized diagnostic approaches, including advanced imaging techniques and histopathological studies, to accurately diagnose and stage the disease. In terms of treatment, this paper advocates a multidisciplinary approach that combines surgery, radiotherapy and chemotherapy and tailors it to individual patients to optimize treatment outcomes. This review highlights case studies that illustrate the effectiveness of surgical intervention in the treatment of these tumors and emphasize the importance of achieving clear surgical margins to prevent recurrence. Furthermore, this review discusses the potential of new molecular targets and the need for innovative therapies that could bring new hope to patients affected by this challenging sarcoma. Full article

Sarcomas, particularly undifferentiated small round cell sarcomas of bone and soft tissue, pose significant diagnostic challenges due to their nonspecific morphology and the necessity for comprehensive molecular analyses. This paper discusses a rare case of round cell sarcoma exhibiting the EWSR1-CREM fusion, offering insights into the complexities of its diagnosis and management. The patient, a 15-year-old female with a history of Type 1 diabetes, presented with persistent right thigh tenderness and swelling. MRI revealed a large necrotic mass in the retroperitoneal region. Histological analysis showed a well-demarcated tumor with diverse cellular morphologies and distinct necrotic areas. Immunohistochemical (IHC) tests identified dot-like staining for Desmin and Vimentin but negative results for several markers, including Cytokeratin and CD45. Strong ALK positivity was noted. Next-generation sequencing with the Illumina TruSight™ Oncology 500 assay revealed the fusion gene EWSR1-CREM, along with benign and uncertain mutations in other genes. The tumor’s morphology and immunoprofile, along with molecular findings, led to a diagnosis of round cell sarcoma with EWSR1-CREM fusion. This case adds to the spectrum of tumors associated with this fusion, often presenting diverse morphologies. The rarity of EWSR1-CREM fusion sarcomas poses a challenge in treatment, highlighted by the development of pulmonary metastases and disease progression after surgical excision in this patient despite the lack of an effective targeted therapy. In conclusion, this case emphasizes the need for a multidisciplinary diagnostic approach in complex sarcomas and highlights the importance of continued research on rare sarcomas, their genetic underpinnings, and potential therapeutic targets. Full article

Introduction: Liposarcomas are the most common of all sarcomas. A well-differentiated liposarcoma can transform into a dedifferentiated liposarcoma with myogenic, osteo- or chondrosarcomatous heterologous differentiation. Genomic amplification of MDM2 gene is then characteristic. Treatment usually involves surgical resection to radically remove the tumor. Other treatments such as chemotherapy and radiotherapy may also be used. Case report: A 60-year-old patient was admitted to the hospital for surgical treatment of a left renal mass. The true location of the tumor was discovered only intraoperatively. The lesion was completely removed laparoscopically with preservation of the capsule. Genomic amplification of MDM2 gene was confirmed. One and a half years after surgery, despite the removal of the tumor without the surrounding margin of healthy tissue, the patient remains without recurrence. Conclusion: Dedifferentiated liposarcoma with osteosarcomatous differentiation is a sporadic case and may occur in various locations of the retroperitoneal space, also mimicking a renal tumor. The laparoscopic technique is a safe surgical treatment for tumors of unclear origin. Removal of dedifferentiated liposarcoma with osteosarcomatous differentiation tumor with preservation of the lesion capsule without maintaining a margin of healthy tissue also allows for long-term cure. Precise immunohistochemical and molecular studies may have an impact on the effectiveness of further treatment and the prognosis of the patient. A patient after surgical treatment of liposarcoma requires constant outpatient follow-up for the reason of the high risk of local and distant recurrence. Full article