Treatment Of Retroperitoneal Sarcomas Research Articles

Financial burden of surgical treatment for retroperitoneal sarcoma.

The purpose of this study was to compare the financial burden of surgery for retroperitoneal sarcoma (RPS) and gastric cancer (GC). All patients who underwent surgery for GC or RPS between 2020 and 2021 at Nagoya University Hospital were included. The clinical characteristics, surgical fees per surgeon, and surgical fees per hour were compared between the two groups. The GC and RPS groups included 35 and 63 patients, respectively. In the latter group, 37 patients (59%) underwent tumor resection combined with organ resection; the most common organ was the intestine (n = 23, 37%), followed by the kidney (n = 16, 25%). The mean operative time (248 vs. 417min, p < 0.001) and intraoperative blood loss (423 vs. 1123ml, p < 0.001) were significantly greater in the RPS group than in the GC group. The mean surgical fee per surgeon was USD 1667 in the GC group and USD 1022 in the RPS group (p < 0.001) and USD 1388 and USD 777 per hour, respectively (p < 0.001). The financial burden of surgical treatment for RPS is unexpectedly higher than that for GC.

Clinical characteristics and management of primary retroperitoneal sarcoma: A literature review.

Retroperitoneal sarcoma (RPS) is a rare tumor classified into many histological types. It is also often detected only after it has grown to a considerable size and requires extensive resection of the surrounding organs, making it difficult to offer optimal patient-tailored management. Evidence supporting specific treatment modalities for RPS is insufficient, owing to its rarity. The Japanese clinical practice guidelines for RPS were published in December 2021, with the aim of accumulating existing evidence and indicating the optimal practice for RPS. These guidelines provide important clinical questions (CQs) concerning the diagnosis and treatment of RPS. This review, with a particular focus on primary RPS, attempts to introduce clinical problems in the diagnosis and treatment of RPS and to assess those problems along with the CQs in the guidelines. According to these guidelines, although chemotherapy and radiotherapy are expected to have therapeutic effects, the level of evidence to support these treatments is not very high at present. Accordingly, complete resection of the tumor is the first and only option for managing primary RPS. However, as with other tumors, the demand for multidisciplinary treatment for RPS is increasing. These guidelines will undoubtedly represent a milestone in clinical practice in relation to RPS in the future, and further evidence is expected to be accumulated based on the CQs that have been proposed.

Surgical treatment of recurrent retroperitoneal sarcoma in its different patterns: A 15-years’ two-centers experience

IntroductionThere is limited data available regarding the role of surgery in the treatment of retroperitoneal sarcoma (RPS) recurrences. We herein report the short- and mid-term outcomes of patients who underwent surgical treatment of RPS recurrences at two Italian centers over a 15-years’ experience. Materials and methodsFrom January 2005 to January 2020, 33 patients underwent surgical treatment of isolated locally recurrent RPS (LR group), locally recurrent RPS associated with the presence of distant recurrence (LR + DM group), and distant-only recurrent RPS (DM group). Only procedures performed to obtain a macroscopically radical treatment with curative intent were included. Data regarding pre-, intra-, post-operative course, and follow-up, collected in an Institutional database, were retrospectively analyzed, and compared. ResultsLR-group was composed of 15 patients, LR + DM group of 9 patients, and DM group of 9 patients. During the follow-up, 78.5% of the LR group, 77.8% of the DM group and 100% of the LR + DM group (p = 0.244) experienced a second recurrence. 7/11 (63.6%) patients in the LR group, 2/7 (28.5%) patients in the DM-group, and 0/9 (0.0%) patients in the LR + DM group underwent to almost one further local treatments of their recurrences (p = 0.010). No differences in the mean disease-free survival (p = 0.127), overall survival (OS) (p = 0.165) was reported among the three groups. Repeated surgery was an independent factor affecting survival in multivariate analysis (p = 0.01). ConclusionsA surgical treatment of RPS recurrences should always be taken into consideration, also in metastatic patients and/or in those who have already undergone surgery for previous RPS recurrence, because this approach may offer survival benefits.

Intraoperative electron radiation therapy after salvage surgery in gynecological cancers and retroperitoneal sarcomas: outcomes and adverse effects.

Salvage surgery is considered an option for isolated recurrences of retroperitoneal and pelvic tumors, in patients who have undergone previous radiotherapy. In order to increase local control intra operative electron radiation therapy (IOERT) can be used in these patients to administer additional radiation dose. We evaluated the outcomes and adverse effects in patients with retroperitoneal sarcoma and gynecologic tumors after salvage surgery and IOERT. Twenty patients were retrospectively analyzed. Twenty-three IOERT treatments were performed after surgery. Six (30%) were sarcoma and 14 (70%) were gynecological carcinoma. Administered dose depended on previous dose received with external beam radiotherapy (EBRT) and proximity to critical structures. The toxicities were scored using the Common Terminology Criteria for Adverse Events version 4.0. The median age of the patients was 51 years (range 34-70). After a median follow-up of 32 months (range 1-68), in the sarcoma group the local control rate was 66.6%; while in the gynecological group the local control rate was 64.3%. In relation to late toxicity, one patient had a Grade 2 vesicovaginal fistula, and one patient presented Grade 4 enterocolitis and enteric intestinal fistula. IOERT could have a role in the treatment of retroperitoneal sarcomas in primary tumors after EBRT, as it may suggest a benefit in local control or recurrences after surgical resection in those at high risk of microscopic residual disease. The addition of IOERT to salvage resection for isolated recurrence of gynecologic cancers suggest favorable local control in cases with concern for residual microscopic disease.

Surgical treatment of locally advanced retroperitoneal sarcomas.

e23552 Background: Surgery with negative resection margins is now considered the main treatment for retroperitoneal sarcomas improving the survival and quality of life of patients. The most common obstacle to surgery with R0 resection in locally advanced tumors is the involvement of the great vessels. The purpose of this study was to analyze the results of surgical treatment in patients with locally advanced retroperitoneal sarcomas involving the inferior vena cava or iliac veins. Methods: The study included 34 cancer patients who underwent tumor resection with major vessel resection in 2015-2021. The mean tumor size was 15.5 cm (from 6 to 41 cm). The most common tumors were moderately differentiated liposarcoma (38%) and pleomorphic liposarcoma (26%). Results: Resection of the inferior vena cava was performed in 19 cases, resection of the iliac segment - 15. Circular vein resection without reconstruction was performed in 5 patients, wedge resection - 7, segmental resection with end-to-end anastomosis - 1, replacement of the venous segment with a prosthesis - 25. Resection of adjacent organs was required in 26.5% of surgical interventions. According to the final pathoanatomical examination, the rate of complete tumor resections (R0-1) was 88.2%. During the 30-day postoperative period, mortality and complication rates were acceptable, 29% and 0%, respectively. During the follow-up period, the median disease-free survival was 13 months, and the median overall survival was not reached. Conclusions: Surgical interventions expanded due to resection of the major veins in locally advanced retroperitoneal sarcomas allow achieving a high rate of radical tumor resection and improving relapse-free and overall survival rates.

Critical impact of radiotherapy protocol compliance and quality in the treatment of retroperitoneal sarcomas: Results from the EORTC 62092-22092 STRASS trial.

The European Organization for Research and Treatment of Cancer 22092-62092 STRASS trial failed to demonstrate the superiority of neoadjuvant radiotherapy (RT) over surgery alone in patients with retroperitoneal sarcoma. Therefore, an RT quality-assurance program was added to the study protocol to detect and correct RT deviations. The authors report results from the trial RT quality-assurance program and its potential effect on patient outcomes. To evaluate the effect of RT compliance on survival outcomes, a composite end point was created. It combined the information related to planning target volume coverage, target delineation, total dose received, and overall treatment time into 2 groups: non-RT-compliant (NRC) for patients who had unacceptable deviation(s) in any of the previous categories and RT-compliant (RC) otherwise. Abdominal recurrence-free survival (ARFS) and overall survival were compared between the 2 groups using a Cox proportional hazard model adjusted for known prognostic factors. Thirty-six of 125 patients (28.8%) were classified as NRC, and the remaining 89 patients (71.2%) were classified as RC. The 3-year ARFS rate was 66.8% (95% confidence interval [CI], 55.8%-75.7%) and 49.8% (95% CI, 32.7%-64.8%) for the RC and NRC groups, respectively (adjusted hazard ratio, 2.32; 95% CI, 1.25-4.32; P = .008). Local recurrence after macroscopic complete resection occurred in 13 of 89 patients (14.6%) versus 2 of 36 patients (5.6%) in the RC and NRC groups, respectively. The current analysis suggests a significant benefit in terms of ARFS in favor of the RC group. This association did not translate into less local relapses after complete resection in the RC group. Multidisciplinary collaboration and review of cases are critical to avoid geographic misses, especially for rare tumors like retroperitoneal sarcoma.

Retroperitoneal Sarcoma Care in 2021.

Simple SummarySoft-tissue sarcomas are exceedingly rare, accounting for <1% of all adult malignancies. Sarcomatous tumors are located within the retroperitoneum in <10% of cases. International collaborations have been instrumental in advancing our understanding of these rare tumors in the last decade. Notably, standard treatment has become upfront primary surgical resection for most subtypes. Radiation and systematic therapy have a limited role. In this 2021 review, we detail the anatomical boundaries of the retroperitoneum, risk factors including genetic predispositions, clinical characteristics, histologic-specific management, contemporary standard of care, recent advancements and limitations of knowledge in the treatment of retroperitoneal sarcoma care. Soft-tissue sarcomas are biologically heterogenous tumors arising from connective tissues with over 100 subtypes. Although sarcomas account for <1% of all adult malignancies, retroperitoneal sarcomas are a distinct subgroup accounting for <10% of all sarcomatous tumors. There have been considerable advancements in the understanding and treatment of retroperitoneal sarcoma in the last decade, with standard treatment consisting of upfront primary surgical resection. The evidence surrounding the addition of radiation therapy remains controversial. There remains no standard with regards to systemic therapy, including immunotherapy. Adjunctive therapy remains largely dictated by expert consensus and preferences at individual centers or participation in clinical trials. In this 2021 review, we detail the anatomical boundaries of the retroperitoneum, clinical characteristics, contemporary standard of care and well as recent advancements in retroperitoneal sarcoma care. Ongoing international collaborations are encouraged to advance our understanding of this complex disease.

Mild-term outcomes in the treatment of retroperitoneal sarcomas: a 12-year single-institution experience

Mild-term outcomes in the treatment of retroperitoneal sarcomas: a 12-year single-institution experience

Assessment of Surgical Management Modalities and Their Postoperative Outcome of Retroperitoneal Sarcomas: A Study in a Tertiary Care Hospital, Dhaka, Bangladesh

Background: Retroperitoneal sarcomas(RPS) are rare tumors with an expected incidence of 5-1 new cases/100,000 inhabitants per year.1 Despite the advent of modern imaging, the associated increase incidental diagnoses, retroperitoneal soft tissue sarcoma (RPS) remains sararemalignancy occurring 0.5 to 1.0 per 100,000 populations.1 The rarity of these tumors and the complexity of their treatment require multi-disciplinary management in specialized centers to improve oncologic and clinical outcomes.2 Moreover, sarcomas in the retroperitoneum have a worse prognosis than sarcomas in the extremities.3 Surgery remains the curative treatment for RPS. Unlike extremity sarcomas, however, RPS can expand massively in the confines of the retroperitoneum prior to detection and diagnosis, making these resections challenges. Objective: The main objective of this study was to assess the surgical management modalities and their post-operative outcomes of retroperitoneal sarcomas. Methods and Materials: This was a cross-sectional study from initially all the patients were enrolled by purposive sampling. Thereafter, they were scrutinized by eligibility criteria. All the patients underwent definitive surgery. A preformed structured peer-reviewed data collection sheet was prepared which was used to collect data. Data were compiled, edited, and analyzed by SPSS version 24.0. The result was tabulated in table and figure form. Data analysis was done by Pearson’s chi-square test and student’s t-test. P-value was significant at &lt;0.05. Results: Out of 30 patient’s maximum of 13(43.33%) patients belonged to the 50-59 years ' age group which was subsequently followed by 6(20%) in the&gt;60 years’ age group. Rest 5(16.67%), 4(13.33%) and 2(6.66%) patients belonged to 40-49 years, 30-39 years, and ≤ 29 year’s age group respectively. Out of 30 patients, 25(83.33%) were male and 5(16.67%) were female. The male-female ratio was 5:1. Among the total 30 patients, 22(73.33) and 8(26.67%) were primary .....

Critical impact of radiotherapy protocol compliance and quality in the treatment of retroperitoneal sarcomas: Results from the 62092-22092 STRASS trial.

11566 Background: The EORTC 22092-62092 STRASS trial failed to detect a superiority of neoadjuvant radiotherapy in patients with retroperitoneal sarcoma as compared to surgery alone. As radiotherapy (RT) was the experimental treatment, a comprehensive quality assurance program (RTQA) has been included in the study protocol in order to detect and correct potential RT deviations. We report here the overall trial RTQA results and its potential impact on patient’s outcomes in this international phase III trial. Methods: Plans from all patients randomized to the experimental preoperative RT arm were submitted to a multidisciplinary RTQA team, consisting of medical physicists and radiation-oncologists. Target volume parameters and tumor dose coverage were prospectively reviewed by the RTQA team but not all plans were made compliant. In order to evaluate the impact on oncological outcomes, a composite endpoint, overall RT compliance status, was created; patients were classified into two major groups: RT compliant (RC) group and non-compliant (NRC) group, defining whether RT was as concisely per protocol or not. This composite endpoint combined the information related to PTV coverage, target delineation, total dose received and overall treatment time. Both abdominal recurrence-free survival (ARFS) and OS were compared between RC and NRC patients using Cox’s proportional hazard model adjusted for age, sex, WHO performance status, tumor grade, histological subtype and tumor size at baseline (millimeters). Results: After final review, 75.2% (94 out of 125) of patients had compliant RT plans (65.6% were already compliant at first submission to RTQA team and 9.6% were made compliant after review). Most patients in the NRC (77.4%) had deviations linked to incorrect target volume delineations. 3-year ARFS was 67.2% (95% Confidence interval (CI): 58.0 – 77.8%) and 48.4% (34.3 – 68.2%) for RC and NRC group, respectively (adjusted HR: 2.64, 95% CI: 1.38 – 5.07, p = 0.003). Corresponding OS at 3 years was 89.9% (95% CI: 83.6 – 96.3%) and 75.4% (95% CI: 61.9 – 91.8%) in the RC and NRC group with a trend in favor of RC (adjusted HR: 2.76, 95% CI: 0.91 – 8.43, p = 0.074). Conclusions: To our knowledge, this is the first RTQA evaluation of a phase III sarcoma trial. The data suggests a significant benefit in terms of ARFS and a trend for OS in favor of the RT compliant group. RTQA in prospective clinical trials, investigating new RT techniques, dose levels and indications, continues to be an important and integral part of trial designing. Funding Source: EORTC, EORTC Cancer Research Fund, EUROSARC FP7 278472 and Kom op tegen Kanker (Stand up to Cancer), the Flemish Cancer Society.

A retrospective study to evaluate the safety and efficacy of anlotinib plus camrelizumab in management of advanced retroperitoneal sarcoma.

e23545 Background: Several studies have demonstrated the antitumor activity of single-agent anlotinib in the treatment of soft tissue sarcoma (STS). However, the most effective results were from cases of alveolar soft part sarcoma (ASPS), which was a rare subtype in retroperitoneal sarcoma (RPS). The therapy of RPS is still dismal. Thus, we evaluated the effectiveness and safety of anlotinib plus camrelizumab for the treatment of RPS. Methods: We retrospectively reviewed the data of 36 patients with advanced/metastatic RPS in Sarcoma Centre of Peking University Cancer Hospital from July 2019 to February 2021. Anlotinib was given 12mg Qd 2 weeks on and 1 week off, and camrelizumab was transfused 200mg at Q3w. Patients that underwent at least 4 cycles of therapy were enrolled in this study. The primary endpoint is objective response rate (ORR) and evaluated according to the irRECIST 1.1 criteria. Results: The pathologic subtypes of enrolled cases include liposarcoma (LP) (n = 16), leiomyosarcoma (LMP) (n = 5), uncertain differentiation sarcoma (n = 7), fibroblastic and myofibroblastic tumours (n = 4), rhabdomyosarcoma (n = 2) and malignant peripheral nerve sheath tumour (MPNST) (n = 2). Two patients (5.6%) achieved CR and 9 patients (25.0%) achieved PR, with an ORR of 30.6%. Eighteen patients (50.0%) achieved SD and 7 patients (19.4%) were evaluated as PD, with the disease control rate (DCR) of 80.6%. The progression-free rate (PFR) at six months was 60.0%. Five patients underwent further radical surgical resection. The two CR cases were diagnosed as dedifferentiated liposarcoma (DDLP) and MDM2 amplified MPNST respectively, and both patients have been under CR status for over 12 months until now. The only grade 3 or higher treatment related adverse event was hypertension (22.2%). Camrelizumab related reactive cutaneous capillary endothelial proliferation (RCCEP) was not observed in this group of patients. Conclusions: The combination of anlotinib and camrelizumab demonstrated encouraging efficacy and safety in the treatment of RPS. A further clinical study with biomarker exploration should be performed in the future.

Morbidity and Outcomes After Distal Pancreatectomy for Primary Retroperitoneal Sarcoma: An Analysis by the Trans-Atlantic Australasian Retroperitoneal Sarcoma Working Group.

Multi-visceral resection often is used in the treatment of retroperitoneal sarcoma (RPS). The morbidity after distal pancreatectomy for primary pancreatic cancer is well-documented, but the outcomes after distal pancreatectomy for primary RPS are not. This study aimed to evaluate morbidity and oncologic outcomes after distal pancreatectomy for primary RPS. In this study, 26 sarcoma centers that are members of the Trans-Atlantic Australasian Retroperitoneal Sarcoma Working Group (TARPSWG) retrospectively identified consecutive patients who underwent distal pancreatectomy for primary RPS from 2008 to 2017. The outcomes measured were 90-day severe complications (Clavien-Dindo ≥ 3), postoperative pancreatic fistula (POPF) rate, and oncologic outcomes. Between 2008 and 2017, 280 patients underwent distal pancreatectomy for primary RPS. The median tumor size was 25 cm, and the median number of organs resected, including the pancreas, was three. In 96% of the operations, R0/R1 resection was achieved. The 90-day severe complication rate was 40 %. The grades B and C POPF complication rates were respectively 19% and 5% and not associated with worse overall survival. Administration of preoperative radiation and factors to mitigate POPF did not have an impact on the risk for the development of a POPF. The RPS invaded the pancreas in 38% of the patients, and local recurrence was doubled for the patients who had a microscopic, positive pancreas margin (hazard ratio, 2.0; p = 0.042). Distal pancreatectomy for primary RPS has acceptable morbidity and oncologic outcomes and is a reasonable approach to facilitate complete tumor resection.

P35: BODY COMPOSITION: ASSOCIATION WITH OPERATIVE AND ONCOLOGIC OUTCOMES IN THE MODERN MANAGEMENT OF RETROPERITONEAL SARCOMA – RESULTS OF A FEASIBILITY STUDY

Abstract Introduction Sarcopenia and obesity in cancer may confer negative outcomes, but their prevalence and impact on modern regimens for retroperitoneal sarcoma (RPS) have not been systematically studied. Sarcopaenia and obesity measurements by computed tomography (CT) measurements at L3 may be confounded by the disease process itself. The aim of this study is to determine feasibility in providing the prevalence of sarcopenia and visceral obesity in the management of RPS to assess their potential impact on operative and oncologic outcomes. Method Consecutive sample patients undergoing treatment for RPS from our database were retrospectively studied. Total, subcutaneous and visceral fat areas (VFA), myosteatosis, skeletal muscle index (SMI) lean body mass and fat mass were determined at diagnosis by CT. Sarcopenia will be defined by CT at L3 as SMI &amp;lt;52.4 cm2/m2 for males and SMI &amp;lt;38.5 cm2/m2 for females, and visceral obesity as VFA &amp;gt;163.8cm2 for men and &amp;gt;80.1cm2 for women. Result 40 consecutive patients, 21 [52.5%] female, 56.7±15.1 years, were studied. The most common histologic types were leiomyosarcoma (8 [20.0%]), dedifferentiated liposarcoma (7 [17.5%]), well differentiated liposarcoma (7 [17.5%]), myxoid liposarcoma (4 [10.0%]). Mean±SD body composition measures were: lean body mass, 50.4±12.0 kg; total fat mass, 27.2±8.6 kg; visceral fat area, 148.5±120.2 cm2; subcutaneous fat area, 222.2±20.4 cm2; myosteatosis, 8.9±7.3 cm2. Conclusion Assessment of body composition among patients with RPS is feasible. Analysis to identify the prevalence and significance of sarcopenia and visceral obesity and its relationship to operative and oncologic outcomes is ongoing. Take-home message Assessment of body composition in RPS is feasible and its effect on outcomes is under investigation

A critical narrative review of radiotherapy for retroperitoneal soft tissue sarcoma.

Retroperitoneal sarcomas (RPSs) are rare diseases, and the 5-year overall survival rate remains low. Management of RPSs is challenging, and the quality of treatment strategy is a crucial prognostic factor. Treatment decisions and the treatment department to which the patients is referred must be centralized. Overall survival rate is positively correlated with the number of patients treated in the centers. En bloc surgery is the standard of care for effective treatment and potential for cure, whereas perioperative chemotherapy or radiotherapy has not yet been validated. Pre- or postoperative radiotherapy was considered controversial, but the STRASS trial publication illustrated that preoperative radiation could be useful in some conditions. Retroperitoneal liposarcoma might become a future target of prospective trials. Furthermore, some retrospective studies have shown that preoperative radiotherapy increases the rate of R0-R1 resections. This article reviews the role of external beam radiotherapy in the management of RPS and highlights the optimal volumes, doses, and radiotherapy techniques for the treatment of RPS. As accurately predicting prognosis in patients with RPS is challenging, we also emphasize the utility of nomograms in the field of radiotherapy. However, these nomograms do not include radiotherapy as a predictive factor. Although some authors could conclude that use of radiotherapy should be avoided based on the results of the STRASS trial, some data argue that radiotherapy still has utility in select cases.

Retroperitoneal soft-tissue sarcomas: Radiotherapy experience from a tertiary cancer center and review of current evidence.

Surgery remains to be the main therapeutic approach for retroperitoneal sarcomas (RPS) although evidence supports that complementary radiotherapy increases local-control and survival. We present a multidisciplinary management and experience of a tertiary cancer center in the treatment of RPS and analyze current evidence of radiotherapy efficacy. We retrospectively reviewed 19 patients with primary or relapsed RPS treated between November 2009 and October 2018. Multidisciplinary approach comprised complete resection in 15 patients (79%) achieving resection R0 in 11 patients (58%), R1 in 4 patients (21%) and R2 in 2 patients (10%). Seven patients (37%) underwent a preoperative radiation (PRORT), 10 patients (53%), post-operative radiation (PORT) and 2 patients (10%), received radiotherapy exclusively. Ten patients (53%) received adjuvant chemotherapy. With a median follow-up of 24 months (2-114 months), actuarial rates of loco-regional relapse free survival (LRFS) at 1, 2 and 3 years were 77%, 77% and 67%, respectively. Actuarial rates of distant-metastases-free survival (DMFS), disease-free survival (DFS) and overall survival (OS) at 1, 2 and 3 years were 100%, 100% and 80% for DMFS; 94%, 77% and 67% for DFS and 100%, 91% and 91% for OS, respectively. Only surgical margins (negative vs. positive) showed significance for 3y-LRFS: 100% vs. 34.3%, p=0.018. Treatment tolerance was acceptable with no acute or late toxicity higher than grade 2. Complementary radiotherapy appears to be useful and well tolerated for the multidisciplinary management of RPS. Presence of positive surgical margins seems to be the most relevant prognostic factor through the follow-up.

Correlation between percutaneous biopsy and final histopathology for retroperitoneal sarcoma: a single-centre study.

Retroperitoneal sarcomas are rare soft tissue tumours accounting for 10-15% of soft tissue sarcomas. Patient prognosis and treatment recommendations (including extent of surgery and neoadjuvant strategies) are determined by the pre-operative histopathological subtype and grade obtained from biopsy and thus it is important to understand the accuracy of biopsy in retroperitoneal masses. This study presents a case series of primary retroperitoneal sarcomas managed at Peter MacCallum Cancer Centre (PMCC) between 2008 and 2019. Statistical analyses were performed to determine correlation between histopathology from percutaneous biopsy and surgical excision. A total of 117 patients who underwent percutaneous core biopsy and surgical excision of retroperitoneal sarcoma were included. Diagnostic accuracy varied with histopathological diagnosis, but overall precise concordance between biopsy and final histopathology was seen in 61% (κ = 0.57). Biopsy was most sensitive for identifying well-differentiated liposarcoma (WDLPS) (sensitivity 85%, 95% CI 0.06-0.96) and leiomyosarcoma (sensitivity 81%, 95% CI 0.54-0.96) and was least sensitive for identifying de-differentiated liposarcoma (DDLPS) (sensitivity 40%, 95% CI 0.25-0.56). Overall agreement between biopsy and final histopathology increased with use of PET/CT scan pre-biopsy and with use of fluorescence in situ hybridisation testing on biopsy, however, neither test improved recognition of de-differentiated components within WD/DDLPS on core biopsy. Pre-operative biopsy is important for clinical decision making in the treatment of retroperitoneal sarcoma. A significant portion of patients with a WDLPS will have a de-differentiated component identified at the time of resection that was not identified on initial biopsy.

Leiomyosarcoma of inferior vena cava

A 57-year-old female presented with intermittent abdominal pain and bilateral pedal oedema. She was diagnosed as leiomyosarcoma from inferior vena cava (IVC). Imaging suggested encasement and occlusion of infra-renal IVC. She had received palliative chemotherapy from elsewhere, with no regression. We excised the tumour. At 14 months after the surgery, she is disease-free. Surgery is the mainstay in the treatment of retroperitoneal sarcomas. Even locally advanced retroperitoneal sarcomas may be successfully managed with complete surgical resection. Reconstruction of resected IVC segment may be avoided in selected cases.

Neoadjuvant radiotherapy followed by surgery compared with surgery alone in the treatment of retroperitoneal sarcoma: a population-based comparison.

Retroperitoneal sarcoma (rps) encompasses a heterogeneous group of malignancies with a high recurrence rate after resection. Neoadjuvant radiotherapy (nrt) is often used in the hope of sterilizing margins and decreasing local recurrence after excision. We set out to compare local recurrence-free survival (lrfs) and overall survival (os) in patients treated with or without nrt before resection. Patients diagnosed with rps from February 1990 to October 2014 were identified in the Alberta Cancer Registry. Patients with complete gross resection of rps and no distant disease were included. Patient, tumour, treatment, and outcomes data were abstracted in a primary chart review. Baseline characteristics were compared using the Wilcoxon nonparametric test for continuous data and the Fisher exact test for dichotomous and categorical data. Survival was analyzed using Kaplan-Meier curves with log-rank test. Cox regression was performed to control for age, sex, tumour size, tumour grade, date of diagnosis, multivisceral resection, and intraoperative rupture. Resection alone was performed in 62 patients, and resection after nrt, in 40. Use of nrt was associated with multivisceral resection and negative microscopic margins. On univariate analysis, nrt was associated with superior median lrfs (89.3 months vs. 28.4 months, p = 0.04) and os (119.4 months vs. 75.9 months, p = 0.04). On multivariate analysis, nrt, younger age, and lower tumour grade predicted improved lrfs and os; sex, tumour size, date of diagnosis, multivisceral resection, and tumour rupture did not. In this population-based study, nrt was associated with superior lrfs and os on both univariate and multivariate analysis. When feasible, nrt should be considered until a randomized controlled trial is completed.

Overcoming a travel burden to high-volume centers for treatment of retroperitoneal sarcomas is associated with improved survival

BackgroundGuidelines recommend treatment of retroperitoneal sarcomas (RPS) at high-volume centers. However, high-volume centers may not be accessible locally. This national study compared outcomes of RPS resection between local low-volume centers and more distant high-volume centers.MethodsPatients treated for RPS were identified from the National Cancer Database (1998–2012). Travel distance and annual hospital volume were divided into quartiles. Two groups were identified: (1) short travel to low-volume hospitals (ST/LV), (2) long travel to high-volume hospitals (LT/HV). Outcomes were adjusted for clinical, tumor, and treatment characteristics.ResultsTwo thousand five hundred ninety-nine patients met the inclusion criteria. The LT/HV cohort was younger and more often white (p < 0.01). The LT/HV group had more comorbidities, higher tumor grade, and more often radical resections and radiotherapy (all p < 0.05). The ST/LV group underwent significantly more R2 resections (4.4% vs. 2.6%, p = 0.003). Thirty-day mortality was significantly lower in the LT/HV group (1.2% vs. 2.8%, p = 0.0026). Five-year survival was better among the LT/HV group (63% vs. 53%, p < 0.0001). After adjustment, the LT/HV group had a 27% improvement in overall survival (HR 0.73, p = 0.0009).ConclusionsThis national study suggests that traveling to high-volume centers for the treatment of RPS confers a significant short-term and long-term survival advantage, supporting centralized care for RPS.

Role of radiation therapy for retroperitoneal sarcomas: An eight-institution study from the US Sarcoma Collaborative.

The use of radiation therapy in the treatment of retroperitoneal sarcomas has increased in recent years. Its impact on survival and recurrence is unclear. A retrospective propensity score matched (PSM) analysis of patients with primary retroperitoneal soft tissue sarcomas, who underwent resection from 2000 to 2016 at eight institutions of the US Sarcoma Collaborative, was performed. Patients with metastatic disease, desmoid tumors, and palliative resections were excluded. Total 425 patients were included, 56 in the neoadjuvant radiation group (neo-RT), 75 in the adjuvant radiation group (adj-RT), and 294 in the no radiotherapy group (no-RT). Median age was 59.5 years, 186 (43.8%) were male with a median follow up of 31.4 months. R0 and R1 resection was achieved in 253 (61.1%) and 143 (34.5%), respectively. Overall 1:1 match of 46 adj-RT and 59 neo-RT patients was performed using histology, sex, age, race, functional status, tumor size, grade, resection status, and chemotherapy. Unadjusted recurrence-free survival (RFS) was 35.9 months (no-RT) vs 33.5 months (neo-RT) and 27.2 months (adj-RT), P = .43 and P = .84, respectively. In the PSM, RFS was 17.6 months (no-RT) vs 33.9 months (neo-RT), P = .28 and 19 months (no-RT) vs 27.2 months (adj-RT), P = .1. Use of radiotherapy, both in adjuvent or neoadjuvent setting, was not associated with improved survival or reduced recurrence rate.