Retroperitoneal soft tissue sarcomas: A pilot study of intraoperative radiation therapy

This pilot study was conducted to evaluate the feasibility and tolerance of a multimodal therapy of retroperitoneal soft tissue sarcoma (STS), including intraoperative radiation therapy (IORT). Nineteen patients (14 primarily treated patients and 5 treated for a recurrent tumor) were included. Surgery included a complete resection (14), a partial resection (2), and no resection (2). The median IORT dose was 17 Gy. Thirteen patients also received an external radiation therapy (ERT). Nine patients received chemotherapy. There was no postoperative mortality. Immediate postoperative complications occurred in four patients (21%). Delayed complications occurred in six patients, including one lethal iliac artery disruption. With a median follow-up of 17 months, the 2-year disease-free survival rate was 60%, and the 2-year actuarial local control rate was 76%. A multimodality approach of treatment, including IORT and ERT and eventually chemotherapy, appears feasible in patients with retroperitoneal STS. However, the treatment-related morbidity appeared relatively high in this study. © 1996 Wiley-Liss, Inc.     

Outcome after surgical resection of multiple recurrent retroperitoneal soft tissue sarcoma

IntroductionLocal recurrences (LR) and distant metastases (DM) are common in retroperitoneal soft tissue sarcoma (RPS). Longer time to recurrence and resection of the recurrent lesion have been identified as beneficial prognostic factors for overall survival (OS) upon first tumor relapse. However, prognostic factors concerning OS upon subsequent recurrences are scarcely defined. In this study, we aimed to identify prognostic factors for post-relapse outcome in multiple recurrent RPS.MethodsPatients undergoing resection of primary and recurrent RPS at the University Hospital Heidelberg were retrospectively analyzed. Multivariable Cox regression analyses were performed to identify predictors of overall, LR- and DM-free survival. Subgroup analyses were performed for liposarcoma and leiomyosarcoma patients.Results201 patients with primary disease, 101 patients with first, 66 patients with second and 43 patients with third LR as well as 75 patients with DM were analyzed. More than 12 months to recurrence and resection of recurrence were associated with improved OS after resection of first and second LR (5-year OS for first/second LR; resection: 64%/62%, no resection: 20%/46%). Gross macroscopic incomplete resection of first (p < 0.001), second (p = 0.001), and third recurrences (p < 0.001) was an independent prognostic factor for poor OS.ConclusionDevelopment of LR and DM is frequent in RPS. Once a tumor relapsed, patients benefit from tumor resection not only in case of first, but also in case of subsequent recurrences.     

Retroperitoneal soft tissue sarcoma: an analysis of radiation and surgical treatment

PURPOSE: To evaluate the clinical outcomes of patients with localized retroperitoneal soft tissue sarcoma (STS) treated with complete surgical resection and radiation. METHODS AND MATERIALS: The medical records of 83 patients were reviewed retrospectively. Sixty patients presented with primary disease and the remaining 23 had recurrence after previous surgical resection. RESULTS: With a median follow-up of 47 months, the actuarial overall disease-specific survival (DSS), distant metastasis-free survival, and local control (LC) rates were 44%, 67%, and 40%, respectively. Of the 38 patients dying of disease, local disease progression was the sole site of recurrence for 16 patients and was a component of progression for another 11 patients. Multivariate analysis indicated that histologic grade was associated with the 5-year rates of DSS (low-grade, 92%; intermediate-grade, 51%; and high-grade, 41%, p = 0.006). Multivariate analysis also indicated an inferior 5-year LC rate for patients presenting with recurrent disease, positive or uncertain resection margins, and age greater than 65 years. The data did not suggest an improved local control with higher doses of external-beam radiation (EBRT) or with the specific use of intraoperative radiotherapy (IORT). Radiation-related complications (10% at 5 years) developed in 5 patients; all had received their EBRT postoperatively. CONCLUSIONS: Although preoperative radiation therapy and aggressive surgical resection is well tolerated in patients, local disease progression continues to be a significant component of disease death. In this small cohort of patients, the use of higher doses of EBRT or IORT did not result in clinically apparent improvements in outcomes.     

Recurrent retroperitoneal sarcomas: Clinical outcomes of surgical treatment and prognostic factors

Background and purposeLocoregional recurrence after resection of primary retroperitoneal sarcoma (RPS) is a challenging therapeutic issue. The objective of this study was to identify clinicopathological factors predictive of overall survival (OS) and disease specific survival (DSS) after reoperation for recurrent RPS.Patients and methodsWe retrospectively collected data from the medical records of 800 patients who underwent resection for sarcoma at our Institution, from 1983 to 2015. Among these patients, 120 were treated for retroperitoneal sarcoma and 55 had a locoregional recurrence (LR). Four of them did not undergo surgery and thus were excluded from this study leaving 51 cases available for data analysis. Univariate and multivariate survival analyses were performed to identify prognostic factors.ResultsMedian overall survival was 33 months. The 1-year, 3-year and 5-year OS rates were 75.5%, 47.1% and 31.6% respectively. Multivariate Cox regression analysis suggested that extension of surgery (P = 0.026), surgical margin status (P = 0.015) and histological grade of recurrent tumor (P = 0.047) were independent prognostic factors for OS. Median DSS was 48 months. The 1-year, 3-year and 5-year DSS rates were 79.2%, 53.1% and 40.9%, respectively. At multivariate analysis, predictors of DSS were extension of surgery (P = 0.004), margin status (P = 0.011), histological grade of recurrent tumor (P = 0.008), and disease free interval (DFI) (P = 0.020). As regards histological subtype of recurrent RPS, at univariate analysis, well-differentiated liposarcoma (WDLS) was associated with better OS and DSS (P = 0.052 and P = 0.016 respectively) compared to dedifferentiated liposarcoma (DDLS).ConclusionsAccording to our findings, surgery is more beneficial in patients with low-grade sarcoma, WDLS and long DFI. The achievement of clear resection margins, rather than performing a multivisceral resection, appears to be a key factor to improve OS and DSS.     

Intraoperative radiation therapy

The modern use of intraoperative radiation therapy (IORT) was initiated by the studies of Abe and colleagues at the University of Kyoto. This work stimulated significant laboratory and clinical investigation into the use of IORT throughout Japan, Europe, and the United States. Because of this experience, single high doses of irradiation can be safely delivered to a tumor volume in appropriate clinical situations. Most importantly, this high dose of additional radiation treatment yields improved local control of selected tumors. Treatment programs of external beam radiation therapy, surgical resection, and IORT for patients with locally advanced primary and recurrent rectal carcinoma and retroperitoneal sarcoma have yielded excellent local control and higher survival rates. The future of IORT will be in the successful integration of this therapy into multimodality treatment programs of chemotherapy, external beam irradiation, and surgery for locally advanced malignancies. Received: August 20, 2001     

Neoadjuvant radiation influences the pseudocapsule in soft tissue sarcoma: A histopathologic and radiographic evaluation

Background and objectivesA pseudocapsule surrounds soft tissue sarcoma (STS). Its composition, response to neoadjuvant radiation, and clinical significance are poorly understood.MethodsSeventeen cases of high-grade undifferentiated pleomorphic sarcoma (UPS) were reviewed, ten of which were treated with neoadjuvant radiation. Magnetic resonance imaging (MRI) studies, pathology slides, and patient records were reviewed.ResultsIrradiated pseudocapsules were well-demarcated with fewer viable tumor cells and were thicker on both pathology and MRI measurements when compared to non-irradiated pseudocapsules (p < 0.001, p = 0.04, respectively). Pseudocapsule mean pathology width (MPW) was positively correlated with tumor necrosis percentage (p = 0.044), and negatively correlated with mitotic rate (p = 0.043), though pseudocapsule width measured on MRI did not correlate with these prognostic factors. Despite an evident treatment response to neoadjuvant radiation, viable tumor cells were present within the pseudocapsule and the surrounding healthy tissue.ConclusionsThe pseudocapsule in STS responds to radiation and there appears to be a correlation between pseudocapsule width and tumor necrosis and mitotic activity. As viable tumor cells are present beyond the pseudocapsule, surgeons should remain cautious in determining margins of resection in STS when using the pseudocapsule as a palpable landmark. This novel study is the most detailed to date to describe the histopathologic and radiographic characteristics of the STS pseudocapsule. Further studies are needed to determine the clinical significance of the pseudocapsule.     

Retroperitoneal sarcomas: the Yale experience and a review of the literature

Between 1964 and 1978, 23 patients were referred for radiation therapy for retroperitoneal sarcoma. Three patients had complete excision and negative resection margins and all survived for 5 years without recurrent disease. One patient had complete resection but positive margins, but had a local recurrence 3 1/2 years after radiation. One patient had partial excision with gross residual, and this patient survived only 17 months after an incomplete radiation treatment schedule. Ten cases had biopsy only and were then referred. We found that there were only four cases that survived longer than 1 year, and their average dose of radiation was 4,400 rads, as compared to only 2,691 rads for the remaining six. We conclude that for incompletely resected or only biopsied retroperitoneal sarcoma, doses of 4,000-5,000 rads should be given and then a reevaluation for complete resection should be done with CT scan and reexploration. For completely resected cases, we feel that postoperative radiation adds to the local control. We have reviewed the literature and presented the data in support of our conclusions.     

Preoperative chemo-radiation therapy for localised retroperitoneal sarcoma: A phase I–II study from the Italian Sarcoma Group

BackgroundTo study feasibility, safety and activity of the combination of high-dose long-infusion ifosfamide (HLI) and radiotherapy (RT) as preoperative treatment for resectable localised retroperitoneal sarcoma (RPS).MethodsPatients received three cycles of HLI (14 g/m2). RT was started in combination with second cycle and administered up to a total dose of 50.4 Gy. Surgery was scheduled 4–6 weeks after the end of RT. Primary end-point was 3-year relapse free survival (RFS). The trial is registered with ITASARC_1II_2004_003.FindingsBetween December 2003 and 2010, 83 patients were recruited. Main histological subtypes were well differentiated liposarcoma (19/83, 23%), dedifferentiated liposarcoma (26/83, 31%), leiomyosarcoma (14/83, 17%). Median tumour size was 120 mm (interquartile (IQ) range = 82–160). The overall preoperative treatment was completed in 60 patients. Chemotherapy (CT) was completed in 65, while RT in 73. Four patients progressed before surgery and were not operated. 79 patients underwent surgery. At a median follow-up of 4.8 years (IQ range = 3–6.1), 23 and 15 patients developed local recurrence (LR) and distant metastases (DM); 30 patients died of disease. 3 and 5-year RFS and overall survival were 0.56 (90% confidence interval (CI): 0.45, 0.65) and 0.44 (90% CI: 0.27, 0.48), and 0.74 (90% CI: 0.62, 0.81) and 0.59 (90% CI: 0.33, 0.58). Crude cumulative incidence of LR and DM at 5 years were 0.37 (standard error (SE): 0.06) and 0.26 (SE: 0.06).InterpretationThe combination of preoperative HLI and RT was feasible in two thirds of patients, while preoperative RT could be completed in most (73/83). Although a systemic coverage can be added to RT when this is felt to be appropriate, the ongoing international phase III trial is exploring the role of RT alone.FundingThis is a pure academic trial. No funding sources contributed to it.     

Diagnostic accuracy of biopsy after neoadjuvant treatment for well-differentiated and dedifferentiated retroperitoneal liposarcoma

PurposeAccurate histopathological grading of percutaneous biopsies is essential to guide adequate management of patients with suspected retroperitoneal liposarcoma. In this regard, however, limited reliability has been described. Therefore, we conducted a retrospective study to assess the diagnostic accuracy in retroperitoneal soft tissue sarcomas and simultaneously investigate its impact on patients' survival.Materials and methodsReports of an interdisciplinary sarcoma tumor board between 2012 and 2022 were systematically screened for patients with well-differentiated (WDLPS) and dedifferentiated retroperitoneal liposarcoma (DDLPS). Histopathological grading on pre-operative biopsy was correlated with corresponding postoperative histology. Additionally, patients' survival outcomes were examined. All analyses were performed in two subgroups: patients with primary surgery and patients with neoadjuvant treatment.ResultsA total of 82 patients met our inclusion criteria. Diagnostic accuracy of patients who underwent upfront resection (n = 32) was significantly inferior to patients with neoadjuvant treatment (n = 50) (66% versus 97% for WDLPS, p < 0.001; 59% versus 97% for DDLPS, p < 0.001). For patients with primary surgery, histopathological grading on biopsy and surgery was concordant in only 47% of cases. Sensitivity for detecting WDLPS was higher than for DDLPS (70% versus 41%). Higher histopathological grading in surgical specimens correlated with worse survival outcomes (p = 0.01).ConclusionHistopathological grading of RPS may no longer be reliable after neoadjuvant treatment. The true accuracy of the percutaneous biopsy may need to be studied in patients who do not receive neoadjuvant treatment. Future biopsy strategies should aim to improve identification of DDLPS to inform patient management.     

Preoperative radiation,surgical margins,and local control of extremity sarcomas of soft tissues

The relationship between status of the surgical margin and local control of soft tissue sarcoma of the extremities by preoperative radiation therapy has been assessed in 132 consecutive patients from 1974 to 1988. The 5-year actuarial local control rate was 94% for all patients; the rates were 97% and 82% for the 104 tumors with negative marigins and the 28 tumors with positive margins, respectively. The local control rate was independent of the extent of the negative margin. For primary sarcomas, the local control rates were 96%, 97%, and 100%, respectively, for margins that were negative at ≤1 mm or > 1 mm, or the specimens were negative for tumor; for positive margins the results was 83%. The overall local control rate was lower (not significant) for recurrent than for primary sarcomas: 88% vs. 94%. On the other hand, when stratifying by margin status, no difference was observed in local control results, i.e.: (1) 97% and 91% for primary and recurrent tumors with negative margins, respectively, and (2) 83% and 80% for primary and recurrent tumors with positive margins, respectively. Local control was not significantly higher in those patients who were treated by surgical resection (S), radiation (RT), and re-excision of the previous tumor bed than in those whose radiation therapy was started after the biopsy and followed by one surgical resection. For primary sarcomas that were resected with negative margins the results were: 100% of 15 patients treated by S-RT-S and 96% of 75 patients treated by RT-S. © 1993 Wiley-Liss, Inc.     

Prognostic significance of 18F-FDG PET at diagnosis in patients with soft tissue sarcoma and bone sarcoma; systematic review and meta-analysis

PurposeThe usefulness of ¹⁸F-fluorodeoxyglucose positron emission tomography (¹⁸F-FDG PET) for the survival prognosis in soft tissue sarcoma (STS) and bone sarcoma (BS) is controversial. The objective of this systematic review was to provide an up-to-date and unprecedented summary of the prognostic value of ¹⁸F-FDG PET at diagnosis in STS and BS.MethodsStudies evaluating pre-treatment ¹⁸F-FDG PET for overall survival of STS and BS were systematically searched for in MEDLINE, EMBASE, and Web of Science. Comparative analyses of the pooled hazard ratios (HR) of overall survival were performed between patients with high and low maximum standardised uptake value (SUVmax). The quality of study designs was evaluated using the Newcastle-Ottawa scale (NOS) for quality assessment of cohort studies. P < 0.05 was defined as statistically significant.ResultsA total of six studies comprising 514 patients with STS and BS were considered for the meta-analysis. The pooled HR for overall survival was 1.22 (95% confidence interval: 1.03–1.46), suggesting that high SUVmax predicts a significantly shorter overall survival period than low SUVmax (P = 0.03). Additional subgroup analyses using patients with STS alone showed that high SUVmax might predict poorer overall survival than low SUVmax (P = 0.004), although only two studies consisting of 96 patients were included. The overall quality of the included studies evaluated by the NOS assessment was adequate.Conclusion¹⁸F-FDG PET at diagnosis provides a very useful predictive tool for patients with STS and BS.     

Predictors of lung recurrence and disease-specific mortality after pulmonary metastasectomy for soft tissue sarcoma

BackgroundWe identified prognostic factors in a 30-year series of STS treated at a single Institution, using an advanced statistical approach.MethodsFrom June 1988 to July 2019, 164 patients were referred to Rizzoli Orthopedic Hospital, Bologna, Italy) for STS lung metastasectomy (LMTS). The endpoints were lung metastasis recurrence (LMR) and lung metastasis-specific mortality (LMSM).The analysis included directed acyclic graphs, cubic splines, and a competing risk model in order to minimize bias.ResultsThe 10- and 15- year LMR cumulative incidence were 0.77 (0.76–0.78) whereas 10- and 15- year freedom from LMSM were 0.60 [0.51–0.70] and 0.56 [0.47–0.67], respectively. The malignant peripheral nerve sheath tumor (MPNST) histotype (SHR 4.12 [2.05–8.27]), a disease-free interval (DFI) up to 68 months (HR from 2 [1.7–2.2] to 1.5 [1.1–1.9]) and a LM size ≥4 mm (3.1 [2.1–4.4]) predicted LMR.Myxofibrosarcoma (HR 2.52[1.64–3.86]), synovial sarcoma (2.53[1.22–5.23]), adjuvant chemotherapy (2.01[1.11–3.61]), DFI between 2 months and 20 months (HR from 1.5 [1.1–2.3] to 1.3 [1.1–1.7] and primary tumor size a primary tumor size comprised between 3.6 cm and 10 cm predicted LMSM. A sharp increase in LMSM was observed with a tumor size from ≥20 cm.ConclusionsOur analysis corrected by potential confounders allowed us to identify specific histotypes and DFI intervals as predictors of both LMR and LMSM. Tumor size adjuvant chemotherapy adversely affected LM-related survival. Our findings need to be confirmed by larger randomized studies.     

Perioperative blood transfusion is not an independent predictor for worse outcomes in retroperitoneal sarcoma surgery

BackgroundSurgery for retroperitoneal soft tissue sarcoma (RPS) is technically challenging, often requiring perioperative red blood cell transfusion (PBT). In other cancers, controversy exists regarding the association of PBT and oncologic outcomes. No study has assessed this association in primary RPS, or identified factors associated with PBT.MethodsData was collected on all resected primary RPS between 2006 and 2020 at The Ottawa Hospital (Canada) and University Hospital Birmingham (United Kingdom). ‘PBT’ denotes transfusion given one week before surgery until discharge. Multivariable regression (MVA) identified clinicopathologic factors associated with PBT and assessed PBT association with oncologic outcomes. Surgical complexity was measured using resected organ score (ROS) and patterns of resection.Results192 patients were included with 98 (50.8%) receiving PBT. Median follow-up was 38.2 months. High tumour grade (OR 2.20, P = 0.048), preoperative anemia (OR 2.78, P = 0.020), blood loss >1000 mL (OR 4.89, P = 0.004) and ROS >2 (OR 2.29, P = 0.026) were associated with PBT on MVA. A direct linear relationship was observed between higher ROS and increasing units of PBT (β = 0.586, P = 0.038). Increasingly complex patterns of resection were associated with increasing odds of PBT. PBT was associated with severe post-operative complications (P = 0.008) on MVA. Univariable association between PBT and 5-year disease-free or overall survival was lost upon MVA.ConclusionsSurgical complexity and high tumour grade are potentially related to PBT. Oncologic outcomes are not predicted by PBT but are better explained by tumour grade which subsequently may increase surgical complexity. Strategies to reduce PBT should be considered in primary RPS patients.     

MRI surveillance for local recurrence in extremity soft tissue sarcoma

IntroductionThe role of MRI in surveillance for local recurrence (LR) remains uncertain in extremity soft tissue sarcoma (STS). The aims of this study were 1) to examine the usefulness of MRI in detecting LR, 2) to identify the characteristics of LR detected by MRI, and 3) to examine whether MRI surveillance is associated with oncologic outcome.Materials and Methods477 patients who had regular surveillance for LR after surgery for extremity STS were reviewed. Surveillance was performed by routine MRI in 325 patients or other imaging modalities in 152 patients.ResultsThe rate of MRI-detected LR, defined as clinically undetectable LR identified on MRI, was 10.5% in the MRI surveillance cohort. The detection rates of MRI-detected LR were significantly higher in the patients with high risk of LR. MRI-detected LRs were more commonly located in the thigh or buttock (p = 0.005), were smaller (p = 0.001) and had LRs without mass formation (p = 0.007) than non-MRI-detected LRs. On Kaplan-Meier analysis, patients with MRI-detected LR tended to have better post-LR survival (p = 0.104).ConclusionRoutine MRI surveillance can detect a significant number of clinically undetectable LRs in extremity STS especially for LRs in the thigh or buttock, small LRs or LRs without mass formation.     

Oncological outcome after hyperthermic isolated limb perfusion for primarily unresectable versus locally recurrent soft tissue sarcoma of extremities

IntroductionThe management of locally advanced extremity soft tissue sarcomas, particularly in terms of a limb salvage strategy, represents a challenge, especially in recurrent tumors. In the context of a patient-tailored multimodal therapy, hyperthermic isolated limb perfusion (ILP) is a promising limb-saving treatment option. We report the outcome of patients with primarily irresectable and locally recurrent soft tissue sarcoma (STS) treated by ILP.Patients and methodsData about patient demographics, clinical und histopathological characteristics, tumor response, morbidity and oncological outcome of all patients with STS, who underwent an ILP at our institution in a 10-year period, were retrospectively detected and analyzed.ResultsThe cohort comprised 30 patients. Two patients were treated with ILP for palliative tumor control, 13 patients because of a local recurrent soft tissue sarcoma (rSTS) and 15 patients because of primarily unresectable soft tissue sarcoma (puSTS). 25 of the 28 patients with curative intention received surgery after ILP (11 pts with rSTS and 14 pts with puSTS). Histopathologically we observed complete response in 6 patients (24%) and partial responses in 19 patients (76%) with a significant better remission in patients with puSTS (p = 0,043). Limb salvage rate was 75%. Mean follow-up was 69 months [range 13–142 months]. Seven (7/11; 64%) patients with rSTS and one (1/14; 7%) patient with puSTS developed local recurrence after ILP and surgery, whereas eight (8/13; 62%) rSTS patients and seven (7/15; 47%) puSTS patients developed distant metastasis. During follow-up, eight patients (28.5%) died of disease (5/13; 38%) rSTS and 3/15 (20%) puSTS. ILP in the group of previously irradiated sarcoma patients (n = 13) resulted in a limb salvage rate of 69% and was not associated in an increased risk for adverse events.DiscussionILP for advanced extremity STS is a treatment option for both puSTS and rSTS resulting in good local control and should be considered in multimodal management. ILP is also a good option for patients after radiation history.     

Dedifferentiated liposarcoma in the extremity and trunk wall: A multi-institutional study of 132 cases by the Japanese Musculoskeletal Oncology Group (JMOG)

BackgroundDedifferentiated liposarcoma occurs predominantly in the retroperitoneum. Given the paucity of cases, information on the clinical characteristics of this entity in the extremities and trunk wall is quite limited. In particular, the significance of preoperative evaluation and principles of intraoperative management of the different components, i.e., well-differentiated and dedifferentiated areas, are still to be defined.MethodsClinical characteristics, treatment outcomes, and risk factors for poor oncological outcomes in cases of dedifferentiated liposarcoma in the extremity or trunk wall were analyzed by a retrospective, multicentric study.ResultsA total of 132 patients were included. The mean duration from the initial presentation to dedifferentiation was 101 months in dedifferentiation-type cases. The 5-year local recurrence-free survival, metastasis-free survival, and disease-specific survival rates were 71.6%, 75.7%, and 84.7%, respectively. Among 32 patients with metastasis, 15 presented with extrapulmonary metastasis. A percentage of dedifferentiated area over 87.5%, marginal/intralesional margin, and R1/2 resection in the dedifferentiated area were independent risk factors for local recurrence. Dedifferentiated areas over 36 cm², French Federation of Cancer Centers Sarcoma Group grade III, and intralesional or marginal resection were independent risk factors for metastasis. A dedifferentiated area over 77 cm² and lung metastasis were independent risk factors for disease-specific mortality.ConclusionsThe typical clinical characteristics of dedifferentiated liposarcoma in the extremity and trunk wall were reconfirmed in the largest cohort ever. The evaluation of the dedifferentiated area in terms of grade, extension, and pathological margin, together with securing adequate surgical margins, was critical in the management of this entity.     

The impact of postoperative radiological surveillance intensity on disease free and overall survival from primary retroperitoneal,abdominal and pelvic soft-tissue sarcoma

AimThis observational study aimed to evaluate the impact of intensity of radiological surveillance on survival following resection of retroperitoneal sarcoma.MethodRetrospective cohort study of patients undergoing primary resection of soft tissue sarcoma arising in the retroperitoneum, abdomen or pelvis at a single, high-volume sarcoma centre. Intensity of follow-up regimes up to 5 postoperative years were categorized as ‘European Society for Medical Oncology (ESMO) compliant’ (intense), or ‘non-ESMO compliant’ (less-intense). The primary outcome measure was overall survival (OS). The secondary outcome measures were disease-free survival (DFS) and reoperation rate. Analyses were stratified by high (grade 2 or 3) or low (grade 1) tumour grade.ResultsOf 168 patients, 67.1% had high-grade and 32.9% had low-grade disease. Overall, 40.0% of patients had ESMO-compliant radiological follow-up (high-grade:25.7%, low-grade:66.7%). 41.7% of patients died and 48.2% suffered local or distant recurrence by cessation of follow up. Upon univariable analysis for high-grade tumours, ESMO compliance reduced DFS (p = 0.066) but had no impact on OS. There was no significant difference in the reoperation rate in patients with ESMO-compliant and non-compliant follow-up (p = 0.097). In low-grade tumours, ESMO compliance significantly reduced DFS (p < 0.001), but without effecting OS. In risk-adjusted models for high-grade tumours, ESMO compliant follow-up was associated with reduced OS (HR:3.47, 1.40–8.61, p = 0.007) and no difference in DFS. In low-grade tumours, there was no association between overall ESMO compliance and OS or DFS.ConclusionThis study did not find a benefit for high-intensity radiological surveillance and overall survival in patients undergoing primary resection for high or low-grade retroperitoneal sarcoma.     

Personalizing surgical margins in retroperitoneal sarcomas: an update

Introduction: Tumor biology, as well as completeness of surgical resection, are two important prognostic factors when treating retroperitoneal sarcoma (RPS). A frontline extended surgical approach is associated with improved local control and possibly improved survival. However, this approach has to be tailored to each histological subtype, as the patterns of growth and recurrence risks vary significantly among them.

Areas covered: We provide a review of the literature in RPS, describing the behavior of each of the five main histologic subtypes: well-differentiated liposarcoma (WDLPS), dedifferentiated liposarcoma (DDLPS), leiomyosarcoma (LMS), solitary fibrous tumor (SFT) and malignant peripheral nerve sheath tumor (MPNST). The prognostic factors relevant to oncologic outcomes of RPS, the role of margins and the importance of local control are discussed. Finally, a histologic specific surgical approach to RPS is provided in detail.

Expert opinion: While tumor-related factors are paramount, the only intervenable predictive factor is extent and quality of surgery. The extended surgical approach has been advocated for previously and again we describe it in more detail, tailored specifically to the tumor subtype. The aim of this approach is to maximize the possibility of achieving a complete resection through a standardized approach based on histologic behavior and site of origin.     

Radiosensitivity translates into excellent local control in extremity myxoid liposarcoma

BACKGROUND:

Myxoid liposarcoma has been reported to be more radiosensitive compared with other soft tissue sarcomas (STS). The authors report the results of multidisciplinary treatment of extremity myxoid liposarcoma compared with a contemporary cohort of other STS subtypes with an emphasis on the role of radiotherapy (RT) in improving local control.

METHODS:

Between 1989 and 2004, 691 patients were identified from a prospective STS database who underwent combined management for localized extremity STS and were followed for a minimum of 12 months or until death. All patients underwent surgery together with pre or postoperative RT, depending on their presenting characteristics and resection margins. Demographics and outcomes were compared between patients with myxoid liposarcoma and other STS subtypes (other‐STS).

RESULTS:

Of 691 patients, 88 patients had myxoid liposarcoma and 603 had other STS subtypes (other‐STS). Median age was 48 and 60 years for the myxoid liposarcoma and other‐STS groups, respectively. Median follow‐up was 86 and 61 months, respectively. For myxoid liposarcoma and other‐STS groups, preoperative RT was used in 57% versus 61% of patients and postoperative RT in 43% versus 39%, respectively. The 5‐year local recurrence‐free survival was 97.7% for patients with myxoid liposarcoma compared with 89.6% for patients with other‐STS tumors (P = .008). High‐grade tumors were present in 7% and 59% of myxoid liposarcoma and other‐STS patients, respectively (P = .0003). Two myxoid liposarcoma patients with local recurrence had positive resection margins, whereas only 33% of patients in the other‐STS group who developed a local recurrence had positive resection margins. No patients with myxoid liposarcoma required amputation as primary management, whereas 8 (1.3%) required amputation as primary management in the other‐STS group. Systemic disease control was superior in myxoid liposarcoma compared with other‐STS patients, with 5‐year overall and metastasis‐free survival rates of 93.9% versus 76.4% (P = .0008) and 89.1% versus 66.0% (P = .0001) respectively. Of 12 myxoid liposarcoma patients with distant metastases, 7 appeared in nonpulmonary sites. In comparison, 205 (34%) patients with other‐STS tumors developed systemic disease but 78% had pulmonary metastases.

CONCLUSIONS:

Multidisciplinary management of extremity STS achieves high rates of local control. Myxoid liposarcoma is associated with higher rates of local control compared with other STS subtypes, after combined surgery and radiation, suggesting a particular radiosensitivity that can be exploited to improve oncologic outcome in appropriate cases. Cancer 2009. © 2009 American Cancer Society.