Primary Soft Tissue Sarcoma Research Articles

Long-Term Follow-Up of Patients Receiving Neoadjuvant Treatment Modalties for Soft Tissue Sarcomas of the Extremities.

Simple SummaryNeoadjuvant radiotherapy has gained popularity as a treatment strategy for locally advanced soft tissue sarcoma of the extremities. However, little is yet known about the benefits and risks of adding preoperative chemotherapy. High-risk patients with soft tissue sarcomas of the extremities are treated with a combined preoperative radiochemotherapy at our institution. This study reports an analysis of patients treated either with primary surgery or with preoperative radiochemotherapy, followed by surgery. This kind of multimodal therapy leads to excellent oncological outcomes and is associated with low rates of severe postoperative complications in highly specialized centers.Background: Neoadjuvant treatment modalities in soft tissue sarcoma (STS) of the extremities have become more popular in recent years, but because of the rarity and heterogeneity of STS, there are yet few studies on the long-term impact of neoadjuvant treatment modalities, especially in terms of neoadjuvant radiochemotherapy. Methods: The study enrolled 136 patients with primary STS of the extremities who underwent surgery with curative intent or neoadjuvant therapy, followed by surgery in a 15-year period. Neoadjuvant treatment consisted of radiotherapy (RT) with 60 Gy and in most cases simultaneous chemotherapy (CTx) with ifosfamide (1.5 g/m2/d, d1–5, q28) and doxorubicine (50 mg/m2/d, d3, q28). We investigated the clinical, (post)-operative and histopathological data and the oncological follow-up as well. The median follow-up period was 82 months (range 6–202). Results: A total of 136 patients (M:F = 73:63) with a mean age of 62 years (range; 21–93) was observed. Seventy-four patients (54.4%) received neoadjuvant therapy (NT), 62 patients (45.6%) received primary surgery (PS). When receiving NT, patients with high-risk STS had a lower risk to develop distant metastasis (p = 0.025). Age, histological type, tumor size and surgical margins (R0 vs. R1) had no influence on any survival rates. There was an association between NT and the occurrence of postoperative complications (p = 0.001). The 5-year local recurrence free survival (LRFS), metastasis free survival (MFS), disease free survival (DFS) and overall survival (OS) rate of the whole cohort was 89.9%, 77.0%, 70.6% and 72.6%; whereas the 5-year LRFS, MFS, DFS and OS rate was 90.5%, 67.2%, 64.1% and 62.8% for the NT group and 89.5%, 88.3%. 78.4% and 83.8% for the PS group. Conclusions: Multimodal treatment strategies in patients with STS of extremities lead to excellent oncological outcomes. Patients with high-risk STS had a significantly better MFS when receiving NT than patients with low-risk STS. NT was associated with a higher probability of postoperative but well-manageable complications.

Radiation Therapy in Primary Soft Tissue Sarcoma of the Superficial Trunk.

Despite anatomical differences, truncal soft tissue sarcomas (STS) often are grouped with extremity sarcomas. We evaluated the clinical outcome of patients with truncal STS who underwent gross total resection (GTR) and radiation therapy (RT), with special emphasis on those treated with intensity modulated radiation therapy (IMRT). From January 1, 2001 to December 31, 2018, 64 patients received GTR and RT, where 48 patients were male, 35 patients were aged ≤ 60 years, and 48 patients had tumors ≤ 10 cm. Sixty-two tumors were high grade, 36 were in the chest wall, 7 in the abdominal wall, and 21 were paraspinal. During surgery, 7 received mesh reconstruction, and 6 received flap closure. R0 resection was achieved in 53 patients. Thirteen patients received chemotherapy. With a median follow-up of 57 months, the 5-year actuarial local control (LC) was 71%. In the IMRT subset (50/64, 78%), the 5-year LC for the chest/abdominal wall was 84%, and 69% for the paraspinal subsite. Grade 2+ radiation dermatitis was seen in 21 of 64 (33%) patients, 5 of 64 (8%) developed noninfectious wound complications, 5 of 64 (8%) developed infectious wound complications, and 1 of 64 (2%) developed grade 2 chest wall pain. No additional grade 2+ late toxicity was observed. Based on this study, achieving LC in truncal STS treated with GTR and RT remains challenging even with IMRT (5-year LC: 78%). While the use of IMRT was more promising for tumors of the chest/abdominal wall with 5-year LC of 84%, it was 69% for those located in the paraspinal subsite, indicating a need for further improvement.

PET-CT for the diagnosis and treatment of primary musculoskeletal tumors in Chinese patients - experience from 255 patients in a single center.

The current study was carried out to assess the value of positron emission tomography (PET)/CT on the diagnosis and staging of primary musculoskeletal tumors. PET-CT test results and histopathological study reports of all the patients with primary musculoskeletal tumors in our department from January 2006 to July 2015 were retrospectively reviewed. Maximum standardized uptake value (SUVmax) in these PET-CT reports were recorded and analyzed respectively for each type of sarcoma. A total of 255 patients were included in the final analysis. Sensitivity of SUVmax based diagnosis was 96.6% for primary malignant osseous sarcomas and 91.2% for soft tissue sarcomas. SUVmax of high-grade osseous sarcomas (average 8.4 ± 5.5) was significantly higher (p < 0.001) than low-grade osseous sarcomas (average 3.9 ± 1.8); based on current case series, SUVmax of high-grade soft tissue sarcomas (7.5 ± 5.1) was not significantly different (p = 0.229) from that of low-grade soft tissue sarcomas (5.3 ± 3.7). Significant decrease of SUVmax value after chemotherapy was associated with favorable prognosis in patients with osteosarcoma. Results of the current study indicate that, the SUVmax based application of PET-CT can be a valuable supplementary method to histopathological tests regarding the diagnosis and staging of primary musculoskeletal sarcomas. SUVmax based application of PET-CT is a highly sensitive method in diagnosis of primary osseous and soft tissue sarcomas in Chinese patients.

Inflammatory prognostic scoring systems are risk factors for surgical site infection following wide local excision of soft tissue sarcoma

IntroductionLimb-sparing surgery with negative margins is possible in most soft tissue sarcoma (STS) resections and focuses on maximising function and minimising morbidity. Various risk factors for surgical site infections (SSIs) have been reported in the literature specific to sarcoma surgery. The aim of this study is to determine whether systemic inflammatory response prognostic scoring systems can predict post-operative SSI in patients undergoing potentially curative resection of STS.MethodsPatients who had a planned curative resection of a primary STS at a single centre between January 2010 and December 2019 with a minimum follow-up of 6 months were included. Data were extracted on patient and tumour characteristics, and pre-operative blood results were used to calculate inflammatory prognostic scores based on published thresholds and correlated with risk of developing SSI or debridement procedures.ResultsA total of 187 cases were included. There were 60 SSIs. On univariate analysis, there was a statistically significant increased risk of SSI in patients who are diabetic, increasing specimen diameter, American Society of Anaesthesiology (ASA) grade 3, use of endoprosthetic replacement, blood loss greater than 1 L, and junctional tumour location. Modified Glasgow prognostic score, C-reactive protein/albumin ratio and neutrophil–platelet score (NPS) were statistically associated with the risk of SSI. On multivariate analysis, ASA grade 3, junctional tumour location and NPS were independently associated with the risk of developing a SSI.ConclusionThis study supports the routine use of simple inflammation-based prognostic scores in identifying patients at increased risk of developing infectious complications in patients undergoing potentially curative resection of STS.

Retroperitoneal soft tissue sarcoma: surgical management

Retroperitoneal soft tissue sarcomas are rare and heterogeneous tumors with high recurrence rates that require amultimodal treatment approach and asurgical resection strategy adapted to tumor localization and histological subtype. Based on current scientific data this article intends to provide an overview on subtype-specific features, prognostic factors and operative techniques in the surgical management of retroperitoneal soft tissue sarcomas. Areview of the literature addressing surgical management of retroperitoneal soft tissue sarcomas was performed. Current evidence and recommendations were summarized. Macroscopically complete tumor resection represents the sole curative treatment option for both primary and recurrent retroperitoneal soft tissue sarcomas. To minimize the probability of tumor-infiltrated resection margins, compartmental resection has become astandard treatment for retroperitoneal soft tissue sarcomas. This approach includes resection of all organs and structures adjacent to the tumor. Multivisceral resection is often associated with this approach and it is acceptable in terms of morbidity and mortality if performed at acenter with experience in retroperitoneal sarcoma surgery. Histologic subtype, tumor grading, and quality of initial surgical treatment are major prognostic factors for oncologic overall survival.

18F-FLT PET/CT as a Prognostic Imaging Biomarker of Disease-Specific Survival in Patients with Primary Soft-Tissue Sarcoma.

The purpose of this study was to evaluate 18F-FLT PET/CT as an early prognostic imaging biomarker of long-term overall survival and disease-specific survival (DSS) in soft-tissue sarcoma (STS) patients treated with neoadjuvant therapy (NAT) and surgical resection. Methods: This was a 10-y follow-up of a previous single-center, single-arm prospective clinical trial. Patients underwent 18F-FLT PET/CT before treatment (PET1) and after NAT (PET2). Posttreatment pathology specimens were assessed for tumor necrosis or fibrosis and for Ki-67 and thymidine kinase 1 expression. Maximally selected cutoffs for PET and histopathologic factors were applied. Survival was calculated from the date of subject consent to the date of death or last follow-up. Results: The study population consisted of 26 patients who underwent PET1; 16 of the 26 with primary STS underwent PET2. Thirteen deaths occurred during a median follow-up of 104 mo. In the overall cohort, overall survival was longer in patients with a low than a high PET1 tumor SUVmax (dichotomized by an SUVmax of ≥8.5 vs. <8.5: not yet reached vs. 49.7 mo; P = 0.0064). DSS showed a trend toward significance (P = 0.096). In a subanalysis of primary STS, DSS was significantly longer in patients with a low PET1 tumor SUVmax (dichotomized by an SUVmax of ≥8 vs. <8; P = 0.034). There were no significant 18F-FLT PET response thresholds corresponding to DSS or overall survival after NAT at PET2. Conclusion:18F-FLT PET may serve as a prognostic baseline imaging biomarker for DSS in patients with primary STS.

Primary Soft Tissue Sarcoma of the Heart: An Emerging Chapter in Cardio-Oncology

Primary malignant cardiac tumors are rare, with a prevalence of about 0.01% among all cancer histotypes. At least 60% of them are primary soft tissue sarcomas of the heart (pSTS-h) that represent almost 1% of all STSs. The cardiac site of origin is the best way to classify pSTS-h as it is directly linked to the surgical approach for cancer removal. Indeed, histological differentiation should integrate the classification to provide insights into prognosis and survival expectancy of the patients. The prognosis of pSTS-h is severe and mostly influenced by the primary localization of the tumor, the difficulty in achieving complete surgical and pharmacological eradication, and the aggressive biological features of malignant cells. This review aims to provide a detailed literature overview of the most relevant issues on primary soft tissue sarcoma of the heart and highlight potential diagnostic and therapeutic future perspectives.

Efficacy and Safety of Hypofractionated Preoperative Radiotherapy for Primary Locally Advanced Soft Tissue Sarcomas of Limbs or Trunk Wall.

Simple SummaryThe primary treatment of soft tissue sarcomas (STS) is radical resection. The use of adjuvant radiotherapy showed a significantly decreased incidence of local recurrence. In our previous study, we presented that preoperative hypofractionated radiotherapy is safe and efficient for treating an unselected group of patients with STS. This study aimed to assess the treatment scheme’s use in patients with primary STS treated at one institution. The preoperative radiotherapy (RT) scheme consisted of 5 Gy per fraction for a total dose of 25 Gy. Surgery was performed within 2–4 days from the last day of RT. We included 311 patients in this prospective study. In this group, with a significant percentage of patients with extensive, high-grade STS, hypofractionated preoperative radiotherapy was associated with similar local control compared to published studies dedicated to this population. The early tolerance was good, with a small number of late complications. Background: The use of adjuvant radiotherapy (RT) shows a significantly decreased incidence of local recurrence (LR) in soft tissue sarcomas (STS). This study aimed to assess the treatment scheme’s effect in patients with primary STS treated at one institution. Methods: In this phase 2 trial, 311 patients aged ≥18 years with primary, locally advanced STS of the extremity or trunk wall were assigned to multimodal therapy conducted at one institution. The preoperative RT scheme consisted of 5 Gy per fraction for a total dose of 25 Gy. Surgery was performed within 2–4 days from the last day of RT. The primary endpoint was LR-free survival (LRFS). Adverse events of the treatment were assessed. Results: We included 311 patients with primary locally advanced STS. The median tumor size was 11 cm. In total, 258 patients (83%) had high-grade tumors. In 260 patients (83.6%), clear surgical margins (R0) were obtained. Ninety-six patients (30.8%) had at least one type of treatment adverse event. LR was observed in 13.8% patients. The 5-year overall survival was 63%. Conclusion: In this group, with a significant percentage of patients with extensive, high-grade STS, hypofractionated preoperative RT was associated with good local control and tolerance.

Surgery in soft tissue sarcoma: the thin line between a surgical or more conservative approach.

Surgery is the primary treatment for localized, clinically resectable soft tissue sarcoma (STS). Chemotherapy and radiotherapy, administered in the pre- or post-operative settings, have important ancillary roles in the multimodal management of primary STS. Some sarcoma centers also employ locoregional therapies such as isolated limb perfusion and deep wave hyperthermia in multimodal therapy. In advanced or metastatic STS, surgery is recommended when complete resection is feasible. Nevertheless, in certain situations there is thin line between a surgical or non-surgical approach, generally related to STS histological type/subtype, disease stageand technical considerations. In advanced STS, factors favoring surgery are isolated oligometastatic disease, long disease-free interval, suitable histology, response to chemotherapy and high probability of a complete resection.

Is bipolar hemiarthroplasty an appropriate type of hip articulation following proximal femoral or total femoral resections for musculoskeletal malignancies?

Different approaches are applied for reconstruction in patients with a musculoskeletal malignancy which require a proximal femoral or total femoral resection. We aimed to evaluate the treatment outcomes of patients who underwent a proximal femoral or total femoral resection due to bone and soft tissue tumors and had an endoprosthetic reconstruction by a bipolar hemiarthroplasty type of hip articulation. We retrospectively identified 133 patients who underwent a proximal femoral or total femoral endoprosthetic replacement after resection of a bone or soft tissue malignancy. There were 74 male and 59 female patients, with a mean age of 55.02 ± 16.92years (range 11-84years) and a median follow-up of 24.47 ± 24.45months (range 6-164months). Patient demographics, surgical, and oncological data were recorded. Acetabular wear was measured using the classification proposed by Baker. Functional assessment was performed using the Musculoskeletal Tumor Society (MSTS) functional score. There was no statistically significant difference among primary diagnostic groups in terms of gender, prosthesis type, trochanter major resection, local recurrence, complication/revision rate, and MSTS Score (p > 0.05, for each parameter). On the other hand, a statistically significant difference was detected in terms of degree of acetabular erosion among diagnostic groups (p < 0.001); the acetabular erosion rate (AER) was found to be lower in patients with metastatic carcinoma than in patients with a diagnosis of primary bone or soft tissue sarcoma. The univariable analysis revealed that the effect of age, primary diagnosis, localization, follow-up time, and presence and number of distant organ metastasis variables on AER were found to be statistically significant (p = 0.018, p = 0.035, p = 0.002, p = 0.007, p = 0.031, p = 0.040, respectively). In patients who undergo a proximal femoral or a total femoral resection due to a musculoskeletal tumor, bipolar hemiarthroplasty is an adequate type of hip articulation method, since it does not affect the revision requirements and functional outcomes of patients with acetabular erosion.

Evaluation of the Predictive Potential of 18F-FDG PET and DWI Data Sets for Relevant Prognostic Parameters of Primary Soft-Tissue Sarcomas

Simple SummaryIn this study, we assessed the potential of simultaneously acquired 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) and magnetic resonance imaging-derived quantitative imaging parameters to predict the tumor grade, metastatic status, and response to neoadjuvant therapy of primary soft-tissue sarcomas of the extremities. Based on the results of the present study, quantifications of the 18F-FDG PET uptake provide useful prognostic data for the evaluation of histopathological response to neoadjuvant treatment as well as the aggressiveness of high-risk sarcomas, whereas no correlation between the different outcome variables and the results for tumor size and diffusion-weighted imaging-derived apparent diffusion coefficient values was found. Accordingly, measurements of the metabolic activity of primary and untreated soft-tissue sarcomas could non-invasively deliver relevant information, that may be used for treatment planning and risk-stratification of sarcoma patients in a pretherapeutic setting.Background: To evaluate the potential of simultaneously acquired 18F-FDG PET- and MR-derived quantitative imaging data sets of primary soft-tissue sarcomas for the prediction of neoadjuvant treatment response, the metastatic status and tumor grade. Methods: A total of 52 patients with a high-risk soft-tissue sarcoma underwent a 18F-FDG PET/MR examination within one week before the start of neoadjuvant treatment. For each patient, the maximum tumor size, metabolic activity (SUVs), and diffusion-restriction (ADC values) of the tumor manifestations were determined. A Mann–Whitney-U test was used, and ROC analysis was performed to evaluate the potential to predict histopathological treatment response, the metastatic status or tumor grade. The results from the histopathological analysis served as reference standard. Results: Soft-tissue sarcomas with a histopathological treatment response revealed a significantly higher metabolic activity than tumors in the non-responder group. In addition, grade 3 tumors showed a significant higher 18F-FDG uptake than grade 2 tumors. Furthermore, no significant correlation between the different outcome variables and tumor size or calculated ADC-values could be identified. Conclusion: Measurements of the metabolic activity of primary and untreated soft-tissue sarcomas could non-invasively deliver relevant information that may be used for treatment planning and risk-stratification of high-risk sarcoma patients in a pretherapeutic setting.

The diagnosis of pulmonary metastases on chest computed tomography in primary bone sarcoma and musculoskeletal soft tissue sarcoma

The lungs are the commonest site of metastasis for primary high-grade bone and soft tissue sarcoma, but current guidelines on the management of pulmonary nodules do not specifically cater for this group of patients. The current article reviews the literature from the past 20 years that has reported the CT features of pulmonary metastases in the setting of known primary bone and soft tissue sarcoma, with emphasis on osteosarcoma, chondrosarcoma, and trunk and extremity soft tissue sarcoma, the aim being to aid radiologists who report chest CT of musculoskeletal sarcoma patients in deciding which lesions should be considered metastatic, which lesions are indeterminate and require follow-up, and which lesions are of no concern.

The role of ifosfamide-doxorubicin chemotherapy in the treatment histology-specific, high grade, locally advanced soft tissue sarcoma: A 14-year experience.

e23529 Background: To compare long-term outcomes of high grade, locally advanced, primary soft tissue sarcoma (STS), using Ifosfamide-Doxorubicin vs local therapy alone, in histology-specific sarcomas. Methods: Retrospective analysis was performed on 127 patients from 2005 to 2018, who had STS of extremity or trunk, &gt; 5cm, that were either Synovial Cell, Dedifferentiated Liposarcoma (DDL), Myxofibrosarcoma, Round Cell Liposarcoma (RCLS), Undifferentiated Pleomorphic Sarcoma (UPS), or Undifferentiated Sarcoma not otherwise specified (NOS), with central pathology review. Ifosfamide-doxorubicin was generally given neoadjuvant, followed by radiation and wide excision, with chemotherapy given in 38 patients, while 89 received local therapy alone. Multi-variable analysis of prognostic factors was performed, and local-recurrence-free-survival (LRFS), distant-metastases-free-survival (DMFS), disease-specific-survival (DSS), and overall-survival(OS) were estimated using Kaplan-Meier. Results: Median follow-up was 4.5 years. Younger age (p &lt; 0.0001) and Synovial histology (p = 0.0002) had significantly higher rates of receiving chemotherapy. Size &gt; 10cm and trunk location were poor prognostic features on multivariable analysis (MVA) affecting DMFS, DSS, and OS, while DDL histology had a more favorable effect; although size, trunk location, and DDL histology were not significantly different between treatment groups. Ifosfamide-Doxorubicin vs local therapy alone improved 5-year DMFS at 70.2% vs 49.5% ,p = 0.02, DSS 83.5% vs 57.9%, p = 0.009, and OS 80.6% vs 53.8%, p = 0.002. Sub-analysis of non-synovial histologies still showed a significant improvements in favor of chemotherapy in DMFS,(p = 0.04), DSS (p = 0.02), and OS (p = 0.003). Conclusions: Ifosfamide-Doxorubicin chemotherapy benefits younger patients with &gt; 5cm, high grade, STS of the trunk or extremity, with histologies of Synovial Cell, DDL, Myxofibrosarcoma, RCLS, UPS, or Undifferentiated Sarcoma NOS.

The impact of multimodality therapies in marginally inoperable soft tissue sarcomas (STS): The Toronto Sarcoma Program (TSP) experience.

11548 Background: The mainstay therapy of operable STS remains surgery, which may include (neo)adjuvant therapies. Within the TSP, marginally inoperable STS are often treated with sequential chemo (CTX) and radiation (RT) therapy, followed by surgery (SX). Herein we present our experience of multi-modality therapies for marginally inoperable STS patients (pts). Methods: This was a dual-center, single program, retrospective review. Pts were included if deemed to have marginally inoperable primary or recurrent STS, as determined at the TSP tumor board. Pts included must have had CTX with the intent of having RT and SX after. Pts demographics, treatment details and clinical outcomes data were collected. Relapse free survival (RFS) and overall survival (OS) were estimated using the Kaplan-Meier method. Multivariate analysis of the influence of disease characteristics and treatment on outcomes was assessed using Cox regression. Results: From June 2005 to May 2019, 75 pts were identified. Median age was 52 years (range 16-72). Pts were predominantly male (55%). Histological subtypes included dedifferentiated liposarcoma (29%), leiomyosarcoma (27%), synovial sarcoma (19%) and others (25%). Primary tumor was located in the retroperitoneum (48%), extremity (23%), pelvis (12%), thorax (9%), and other sites (8%). All pts had doxorubicin and ifosfamide CTX (median 4 cycles; range 1-6), while RT dose delivered was 50.4Gy/28 fractions in 58 (77%) of cases. Twenty three pts (31%) achieved partial response, 40 pts (53%) had stable disease and 12 pts (16%) had progression of disease (PD) on CTX, of which half (8%) did not undergo further treatment. Nine pts (12%) underwent CTX followed by SX due to significant response, 9 pts (12%) underwent CTX and RT without SX due to persistent tumor unresectability or PD. The final 50 pts (67%) completed multi-modality treatment (CTX, RT &amp; SX). Overall, 59 pts (79%) had SX; negative margins were achieved in 53 (71%). 19 pts (25%) had postoperative complications, causing death in 2 pts (2.7%). With a median follow-up of 72 months, median RFS and OS were 26.9 months (95% CI: 0-86.0), and 65 months (95% CI: 13.5-116.4). Extremity location was associated with superior RFS (median not reached [NR], HR 0.28 95% CI 0.09-0.83, p = 0.022), and OS (median NR, HR 0.29 95% CI 0.09-0.90, p = 0.032). Receipt of RT was associated with superior RFS (median NR, HR 0.23 95% CI 0.10-0.52, p &lt; 0.001); and OS (median NR, HR 0.21 95% CI 0.09-0.50, p &lt; 0.001). Pts who had PD after CTX were associated with poor outcomes - RFS (median 4.7 months, HR 2.03 95% CI 0.61-6.76, p = 0.24); and OS (median 21.9 months, HR 2.48 95% CI 0.73-8.47, P = 0.144). Conclusions: Multi-modality approach resulted in successful resection for most pts with marginally inoperable STS. Extremity location and RT administration were associated with better RFS and OS, while progression on CTX confers worse survival outcomes.

Refining the approach to patients with primary soft tissue sarcoma of the extremities and trunk wall (ESTSTS): Outcome improvement over 30 years at a single institution.

e23548 Background: In 2011 we reported the improved outcome of ESTS pts treated between 1987 and 2007 at Istituto Nazionale Tumori (INT), Milan (Italy). We updated the analysis at a later follow-up (FU) to assess possible changes in survival of ESTSTS. Outcomes according to histology were also explored. Methods: Pts with primary localized adult-type ESTSTS surgically treated at INT between 1987 and 2017 were retrospectively reviewed and divided into 6 groups according to the date of primary surgery. Crude cumulative incidence (CCI) of sarcoma-specific mortality (SSM), local relapse (LR), distant metastases (DMs) were calculated for each group in a competing risks framework. DM free survival (DMFS) was calculated for each group taking into account all DMs (occurred as first or later). PostDM survival and all outcomes according to histology were calculated before and after 2002. Results: 2384 pts were included. At a 104-mo m-FU, median post-DM FU was 76 mos. Clinicopathologic characteristics were comparable among groups. The use of preoperative chemo (ChT) and radiotherapy (RT) increased from 18% (1987-92) to 29% (2013-17) and from 1.6% to 26%. The rate of preoperative combined ChT-RT also increased from 0.8% to 14.3%. 1st event before SSM was LR in 17.9% pts, DM in 72.2% pts and synchronous LR-DM in 9.3% pts. Table shows 7-yrs outcomes incidence estimates for each period. CCI-SSM (p &lt; 0.001), CCI-LR (p &lt; 0.001) and DMFS (p = 0.040) improved significantly since 2003. 7-yrs postDM survival before vs. after 2003 increased from 15% to 23.6% (p = 0.012). Outcomes by histology were comparable to overall results. Conclusions: In the time frame analyzed, SSM improved by over 15%. An equal improvement in DMFS, the 50% decrease in CCI-LR (1 pts out of 5 had LR as 1st event before death), and the significant postDM increase might explain SSM improvement. Maximum change was seen since 2003 and was paralleled by an increase in the use of preoperative ChT and RT, that might have contributed to a better local and distant control. Of note, the increase in DMFS was not accompanied by a decrease in CCI-DM. The increase in DMFS might be attributable to combined modalities’ ability to offset the negative prognostic impact of positive surgical margins, while no effect was observed on DMs occurred as first event.[Table: see text]

Impact of interdisciplinary sarcoma board evaluation on treatment outcome of primary soft-tissue sarcoma patients.

e23540 Background: Early presentation of soft-tissue sarcoma (STS) patients to a specialized sarcoma center including discussion in the interdisciplinary sarcoma board (ISB) prior to surgery is essential to the treatment of sarcomas. This approach significantly improves patient survival and guideline coherence. However, there exists only limited information on the adherence to the recommendations of the ISB. Accordingly, we decided to analyze a STS cohort at a large German sarcoma center focusing on outcome parameters and adherence to quality parameters defined by the German Cancer Society (Deutsche Krebsgesellschaft). Methods: In a retrospective data analysis, we identified n = 230 adult patients presented at the ISB of the Charité–Universitätsmedizin Berlin in Germany from January 2015 until December 2019. Inclusion criteria were as follows: newly diagnosed STS, presentation during first-line therapy and recommendation of at least one treatment modality such as surgery, chemotherapy, radiotherapy, regional hyperthermia or follow-up by the ISB. Clinical and follow up data was collected by using the hospital information system and the outpatient network. Results: Our patient cohort included 53% male and 47% female patients with a median age of 58 years (range 19-96). The majority (86%) showed a localized tumor stage, while 14% already had metastases. In 24% of the cases the sarcomas were classified as low-grade, in 76% as high-grade. Surgery was recommended for 66% of the cases, for 80% of them combined with chemotherapy, radiotherapy or hyperthermia. 14% of the patients received a recommendation for all 4 modalities. For 9% of the patients, chemotherapy alone was recommended. Both overall survival and progression-free survival was significantly higher in the group with complete adherence to the recommendations of the ISB (p &lt; 0.001). The worst prognosis was found in patients unable to adhere to the recommendations due to rapid progression or complications of the therapy (HR for death 15.06, 95%CI 7.94-32.22). If one recommended modality was not carried out, there also was a higher risk of death (HR 4.38, 95%CI 1.75-10.96). Most common reasons were patient refusal or individual decision by the treating physician. A metastasized tumor stage was associated with an increased risk of death (HR 2.62, 95%CI 1.45-4.75). In contrast, neither the histological grading (low vs. high) nor age did influence the mortality significantly. Conclusions: In our cohort of STS patients, survival depends significantly on adherence to the recommendations of the ISB. Our analysis at a German sarcoma center is in line with previous international reports demonstrating the importance of interdisciplinary decisions and therapeutic adherence. We hereby underline the essential role of interdisciplinary approaches in care of STS patients and the effort to implement defined quality parameters.

Management of Soft Tissue Sarcomas in Extremities: Variation in Treatment Recommendations and Surveillance According to Specialty and Continent

BackgroundThis study aimed to provide an insight into clinical decision-making and surveillance strategy of sarcoma specialists for patients with primary soft tissue sarcoma of the extremities (eSTS). The secondary aim was to quantify the role of patient- and tumor-specific factors in the perioperative management.MethodsMembers of sarcoma societies were sent a Web-based 21-item survey about eSTS management. The survey concerned only primary resectable high-grade eSTS in adults.ResultsThe study enrolled 396 respondents. The majority of the surgical specialists thought the evidence for perioperative chemotherapy (CTX) for high-grade eSTS was insufficient. Radiotherapy (RTX) was less frequently offered in Asia than in North America and Europe. The specialties and continents also differed regarding the importance of patient and tumor characteristics influencing RTX and CTX recommendation. For surveillance after initial treatment outpatient visits, chest computed tomography (CT) scans, and magnetic resonance images of the extremity were the methods primarily used. The specialists in North America preferred chest CT scan over chest x-ray, whereas those in Asia and Europe had no clear preference.DiscussionSpecialty and continent are important factors contributing to the variation in clinical practice, treatment recommendations, and surveillance of patients with primary resectable high-grade eSTS.

Does the primary soft-tissue sarcoma configuration predict configuration of recurrent tumors on magnetic resonance imaging?

Soft-tissue sarcomas (STS) are rare malignancies of the soft tissue. To assess whether the magnetic resonance imaging (MRI) configuration of primary STS can predict the configuration of a recurring tumor and whether the MRI configuration of multiple recurrences differs in one and the same patient. Thirty-nine patients with histologically proven recurrent STS were included in this retrospective study and underwent pre- and post-treatment MRI. Three main configurations of primary and recurrent tumors were identified: polycyclic/multilobulated; ovoid/nodular; and streaky. Sixty recurrent lesions were detected: 34 ovoid/nodular; 15 polycyclic/multilobulated; and 11 streaky. Five recurrences were multifocal and eight were bifocal. Of 39 patients, 28 (71.8%) presented one recurrence within the MRI follow-up period (P = 0.006); in 10 patients (25.6%), up to three different configurations of recurring STS were identified in one patient. Recurrences of polycyclic/multilobulated primaries were mostly ovoid/nodular (48%; P = 0.003) or polycyclic/multilobulated (37%; P = 0.014), and recurring ovoid/nodular STS significantly most often showed the same configuration as the primary tumor (85%; P < 0.001). Primary STS with a streaky configuration recurred in all three configurations in roughly equal proportions. Homogeneity/heterogeneity and tumor borders are significantly associated with the configuration of recurrences. Primary STS configuration may help predict recurrent tumor configuration when the primary STS had a polycyclic/multilobulated or ovoid/nodular configuration. However, recurrent STS configuration can also differ from primary STS configuration, especially when the primary STS had a streaky configuration, rendering recurrent STS difficult to predict. Different configurations of recurrent STS in one and the same patient are common.

Primary alveolar soft tissue sarcoma of the brain and lung: report of two cases

腺泡状软组织肉瘤（alveolar soft part sarcoma，ASPS）是一种少见的软组织恶性肿瘤，多发生于下肢深部软组织，生长缓慢，无疼痛，不引起明显功能障碍，易发生肺、骨、脑转移，脑及肺原发性ASPS极为少见，远期预后差。该文报道2例发生于脑抑或肺内原发的ASPS病例。.

Complete pathological response to neoadjuvant treatment is associated with better survival outcomes in patients with soft tissue sarcoma: Results of a retrospective multicenter study

BackgroundLocally advanced soft tissue sarcoma (STS) management may include neoadjuvant or adjuvant treatment by radiotherapy (RT), chemotherapy (CT) or chemoradiotherapy (CRT) followed by wide surgical excision. While pathological complete response (pCR) to preoperative treatment is prognostic for survival in osteosarcomas, its significance for STS is unclear. We aimed to evaluate the prognostic significance of pCR to pre-operative treatment on 3-year disease-free survival (3y-DFS) in STS patients. MethodsThis is an observational, retrospective, international, study of adult patients with primary non-metastatic STS of the extremities and trunk wall, any grade, diagnosed between 2008 and 2012, treated with at least neoadjuvant treatment and surgical resection and observed for a minimum of 3 years after diagnosis. The primary objective was to evaluate the effect of pCR. (≤5% viable tumor cells or ≥95% necrosis/fibrosis) on 3y-DFS. Effect on local recurrence-free survival (LRFS), distant recurrence-free survival (MFS) overall survival (OS) at 3 years was also analyzed. Statistical univariate analysis utilized chi-square independence test and odds ratio confidence interval (CI) estimate, multivariate analysis was performed using LASSO. ResultsA total of 330 patients (median age 56 years old, range:19–95) treated by preoperative RT (67%), CT (15%) or CRT (18%) followed by surgery were included. pCR was achieved in 74/330 (22%) of patients, of which 56/74 (76%) had received RT. 3-yr DFS was observed in 76% of patients with pCR vs 61% without pCR (p < 0.001). Multivariate analysis showed that pCR is statistically associated with better MFS (95% CI, 1.054–3.417; p = 0.033), LRFS (95% CI, 1.226–5.916; p = 0.014), DFS (95% CI, 1.165–4.040; p = 0.015) and OS at 3 years (95% CI, 1.072–5.210; p = 0.033). ConclusionsIn a wide, heterogeneous STS population we showed that pCR to preoperative treatment is prognostic for survival.