Sarcoma Center Research Articles

Prophylactic Ureteral Catheter Placement Appears to Reduce Intraoperative Ureteric Injury During Resection of Primary Retroperitoneal Liposarcoma.

Background: Prophylactic ureteral catheters placement (PUCP) was advocated as an effective strategy for decreasing ureteral morbidities in colorectal surgeries. However, whether it should be routinely used prior to primary retroperitoneal liposarcoma (PRLS) surgeries remains unknown. Methods: It was a retrospective study, conducted at a tertiary sarcoma center. Medical records of patients with PRLS undergoing surgeries from January 2015 through December 2018 were reviewed. Primary endpoint was the rate of ureteral morbidities during and after retroperitoneal liposarcoma resection procedures. Univariate and multivariate analyses determined risk factors associated with ureteral injury (UI) in patients undergoing surgeries. Results: A total of 55 patients of PRLS were included. Fourteen (25.5%) patients underwent PUCP, with 1 UI (7.1%) identified. In 41 patients with no PUCP, 15 (36.6%) exhibited UIs during and post surgeries. There were significant improvements of UIs in group PUCP, compared with patients without PUCP (P < .05). Resection surgeries combined with colectomy and tumor-ureter relationship were 2 risk factors significantly associated to UIs (P < .01). Conclusions: PUCP might be an effective way of preventing UIs in patients with PRLS. It could be suggested especially in patients with ureter encased by tumor or anticipated colectomy during the surgical process.

Chronic expansive pelvic hematoma: atypical location of pseudotumor. A report of 2 cases.

Case: Chronic expansive pelvic hematoma is a very rare entity with a few cases published in the literature, being the most frequent locations the thorax and chest. The tumoral mass called pseudotumor produces a large osteolysis in the pelvis with a difficult radiological diagnosis, and not rarely misdiagnosed as sarcoma. The treatment of these type of lesions should be done at a specialized sarcoma centre, and under the supervision of a multidisciplinary committee, that it is enable the correct differential diagnosed and treatment. The pathological analysis is the key point to the diagnosis. We report two cases located in the pelvis, an extremely rare location. The resection should be wide to avoid recurrence. The reconstruction in the pelvic cases required “ice-cream cone” prosthesis that provides the restoration of the functionality of the hip. Conclusion: This rare phenomenon is suspicious for malignant tumour. The treatment of this entities should be evaluated in an expert centre in the treatment of sarcomas.

Preoperative Risk Factors for Fibrosarcomatous Transformation in Dermatofibrosarcoma Protuberans.

Dermat of ibrosarcoma protuberans (DFSP) is a soft-tissue sarcoma with a high risk of local recurrence, though typically never metastasizes. DFSP can transform into high-grade fibrosarcoma (DFSP-FS), which has a risk of metastasis. Currently, treatment for DFSP includes Moh's micrographic surgery (MMS); however, this is not recommended for DFSP-FS. Often, the transformation to DFSP-FS is not recognized until the final histological diagnosis. At that point, wide local excision (WLE) of a previous MMS site can be morbid. As such, we analyzed patient risk factors to allow identification of DFSP-FS transformation at presentation. We reviewed 368 (174 female, 194 male) patients with a mean age of 42 years from two sarcoma centers. A total of 319 (87%) patients had a history of DFSP and 49 (13%) had DFSP-FS. When comparing patients with a DFSP to those with a DFSP-FS, patients with a DFSP-FS were more likely (p<0.05) to be older, female and with larger tumors. A painful mass and rapidly enlarging mass were associated with DFSP-FS. Patients who presented with DFSP-FS were found to typically have a larger, painful, and growing mass. Patients with these features should be referred for WLE over MMS at presentation.

Exploring the Feasibility of a Mindfulness-Music Therapy Intervention to Improve Anxiety and Stress in Adolescents and Young Adults with Cancer

ContextAdolescent and young adults (AYAs) with cancer experience significant psychological distress due to cancer treatment that can persist long after treatment. However, little is known regarding optimal interventions to support the psychosocial needs of AYAs with cancer. ObjectiveThe overall objective of this single arm, longitudinal, pilot study was to determine the feasibility of implementing a mindfulness-based music therapy intervention to improve anxiety and stress in AYAs receiving cancer treatment. MethodsAYAs (15 – 39 years old) who were to receive cancer treatment for ≥ eight weeks were recruited from the pediatric, melanoma, sarcoma, breast, lymphoma, and leukemia oncology outpatient centers at Dana-Farber Cancer Institute. The music therapy intervention included four sessions of individual mindfulness-based music therapy in-person or using Zoom over twelve weeks. Prior to-and after the intervention period, participants completed the Patient-Reported Outcomes Measurement Information Anxiety 4a and Perceived Stress Scale. Changes in patient-reported outcomes are compared using Wilcoxon signed-rank tests. ResultsOver ∼14 months, 37 of 93 eligible AYAs were enrolled to the study (39.8% consent rate). Overall, 27 of 37 (73%) participants (Median age=32; 56.8% Female) completed at least two music therapy sessions and the baseline measures and end of study measures. Participation in the mindfulness-based music therapy sessions resulted in significant pre-to-posttest improvements in perceived stress (median change: -4.0, P = 0.013) and non-significant changes in anxiety (median change: -1.9, P = 0.20). Satisfaction and acceptability were highly rated. ConclusionsThe delivery of a four-session mindfulness-based music therapy intervention to AYAs receiving chemotherapy was feasible and significantly improved perceived stress. These preliminary findings should be confirmed in a randomized controlled trial. Trial RegistrationClinicalTrials.gov Identifier: NCT03709225

Osteosarcoma of the Pelvis: Clinical Presentation and Overall Survival.

Introduction Osteosarcoma is the most common sarcoma of bone. Pelvic osteosarcoma presents a significant therapeutic challenge due to potential late symptom onset, metastatic dissemination at diagnosis, and inherent difficulties of wide surgical resection secondary to the complex and critical anatomy of the pelvis. The rates of survival are well reported for osteosarcoma of the appendicular skeleton, but specific details regarding presentation and survival are less known for osteosarcoma of the pelvis. Methods The Surveillance, Epidemiology, and End Results (SEER) program was queried for primary osteosarcoma of the bony pelvis from 2004 to 2015. Cases with Collaborative Staging variables (available after 2004) were analyzed by grade, histologic subtype, surgical intervention, tumor size, tumor extension, and presence of metastasis at diagnosis. The 2-, 5-, and 10-year survival rates were assessed with respect to these variables. The SEER database was then queried for age, tumor size, surgical intervention, metastasis at time of presentation, and survivorship data for patients with primary osteosarcoma of the upper extremity, lower extremity, vertebrae, thorax, and face/skull, and rates for all anatomic locations were then compared to patients with primary pelvic osteosarcoma. Results A total of 292 cases of pelvic osteosarcoma were identified from 2004 to 2015 within the database, representing 9.8% of cases among all surveyed primary sites. The most common histologic subtype was osteoblastic osteosarcoma (69.9%), followed by chondroblastic osteosarcoma (22.3%). The majority of cases were high-grade tumors (94.3%), of size >8 cm (72.0%), and with extension beyond the originating bone (74.0%). For the entire pelvic osteosarcoma group, the 2-, 5-, 10-year survival rates were 45.6%, 26.5%, and 21.4%, respectively, which were the poorest among surveyed anatomic sites. The 5-year overall survival was an abysmal 5.3% for patients with metastatic disease at diagnosis, and 37.0% for non-metastatic pelvic osteosarcoma treated with surgery and chemotherapy. When compared to other locations, pelvic osteosarcoma had higher rates of metastatic disease at presentation (33.5%), larger median tumor size (11.0 cm), and older median age at diagnosis (47.5 years). While over 85% of patients with tumors at the extremities received surgery, only 47.4% of pelvic osteosarcomas in this cohort received surgical resection—likely influenced by larger tumor size, sacral involvement, frequency of metastasis, older age, or delayed referral to a sarcoma center. Conclusion This study clarifies presenting features and clinical outcomes of pelvic osteosarcomas, which often present with large, high-grade tumors with extracompartmental extension, high likelihood of metastatic disease at diagnosis, and a potential limited ability to be addressed surgically. The survival rates of primary osteosarcoma of the pelvis are poor and are lower than osteosarcomas from other anatomic locations. While acknowledging the influence of metastasis, tumor characteristics, and advanced age on the decision to undergo surgical excision of a pelvic osteosarcoma, the rates of surgical resection are low and highlight the importance of understanding appropriate conditions for oncologic resection of pelvic sarcomas.

AB009. Pitfalls in mediastinal masses diagnosis

: Mediastinum is a complex anatomic space in which different malignancies can occur, originating from different cell types, such as epithelial thymic, lymphoid, germinal cell and mesenchimal cell. Their diagnosis requires dedicated knowledge and expertise, due to heterogeneous features. Indeed, there is also a frequent overlap between different histologies leading to uncertainty in the diagnostic process. Here, we present an uncommon mediastinal mass challenging in its characterization. A thirty-year-old woman performed thoracic CT scan for dyspnea and persistent cough. Imaging showed a solid mass of 14×11 cm involving the left thorax with mediastinal deviation to the right side. Patient underwent resection en bloc of left endothoracic mass and segmental resection of the left upper lung lobe, and of the 3rd, 4th and 5th ribs. Initial histological examination was suggestive for B3 thymoma/thymic carcinoma due to the positivity for p63, CD99, CD117 and vimentin. Pathological stage pT1b. Patient was then referred to our rare tumor Reference Center. Histological review showed small-medium sized tumor cells. The immunohistochemical panel showed negativity for cytokeratins (CKMNF116, CKAE1/AE3, CK19), as well as PAX8, CD56 NSE and CD117; while CD99 was positive, excluding the diagnosis of thymic/thymoma neoplasms; c-kit and other thymoma related genes resulted negative for mutations at a next generation sequencing (NGS) analysis. On the other hand, the negativity of different types of cytokeratins and the elevated mitotic index associated with the sporadic positivity for p63 was not specific for an epithelial neoplasm. Morphological pattern and positivity for CD99 led to a diagnosis of undifferentiated sarcoma. A third revision, was performed at the National Sarcoma Center and included the assessment of markers for sarcoma cluster differentiation. Tumor cells were characterized by the positivity for CD99 and NKX2.2 consistent with a diagnosis of Ewing’s sarcoma, despite the negativity for ETV4. The evaluation of expert centers was critical to establish a correct diagnosis in this complex case. Taking into account the time lasting from the diagnosis and the aggressiveness of this kind of neoplasm, the patient was candidate for chemo-radiotherapy after a multidisciplinary discussion.

Measuring functional outcome in upper extremity soft-tissue sarcoma: Validation of the Toronto extremity salvage score and the QuickDASH patient-reported outcome instruments

Interest in functional outcome (FO) and health-related quality of life (HRQL) in extremity soft-tissue sarcoma (STS) patients has increased. The aim of this study was to validate two FO questionnaires for upper extremity STS patients: the Toronto Extremity Salvage Score (TESS) and short version of the Disability of Arm, Shoulder and Hand (QuickDASH), based on Finnish population data. A multi-center study was conducted at two academic sarcoma centers. Surgically treated upper extremity STS patients were invited to participate. Patients completed the TESS and the QuickDASH with HRQL questionnaires the 15D and the QLQ-C30. The scores were analyzed and compared. Fifty-five patients with a mean follow-up period of 4.7 years were included. Mean age was 63 years (standard deviation [SD] 14.6). The mean score for TESS was 88.5 (SD 15.1) and for QuickDASH 17.8 (SD 19.6). The QuickDASH had a statistically significantly better score coverage. A ceiling effect was noted, 27% and 20% for TESS and QuickDASH, respectively. The TESS and QuickDASH scores were strongly correlated (r= -0.89). The TESS score strongly correlated with the QLQ-C30 (r=0.79) and the 15D score (r=0.70). The QuickDASH score correlated strongly with the QLQ-C30 score (r=-0.71) and moderately with the 15D score (r= -0.56). The TESS score had a statistically significantly stronger correlation with the 15D score than QuickDASH (p<0.005). Both the TESS and the QuickDASH provide reliable scores for assessing FO in upper extremity STS patients. The QuickDASH has a better coverage, whereas TESS showed a stronger correlation to HRQL scores.

Factors associated with complications after resection of soft tissue sarcomas of the groin

IntroductionSoft tissue sarcomas (STS) are rare malignant tumors that require regimented treatment at designated cancer centers. The surgical care of groin tumors is difficult because of frequent local complications. Few studies have been done on prognostic factors and complications. This led us to conduct a retrospective study to: (1) identify factors associated with local postoperative complications during the surgical care of primary groin STS; (2) identify the factors associated with delayed adjuvant radiation therapy; (3) define the optimal surgical treatment strategy to allow adjuvant treatments to start as early as possible, if applicable. HypothesisWe hypothesized that certain patients presenting with an STS of the groin or inguinal area are at higher risk of complications. Materials and MethodsThis retrospective single-center study included all the patients admitted to our referral sarcoma center between 1995 and 2016 for the resection of a primary STS of the groin. Major complications were defined as surgical revision, an invasive procedure, or prolonged dressing use. ResultsOf the 55 included patients, 13 suffered major complications (24%) of which 10 were surgical revisions, two were repeated aspirations and one was prolonged dressing use. Among the 10 surgical revisions, there were two pedicled salvage flaps. The patients who suffered major complications were significantly more likely to be smokers than the patients who did not have major complications (31% vs 2% (p=0.002)). Obesity and surgical bone exposure were most often associated with complications but not significantly (23% vs 5%, p=0.053 and 38% vs 14% (p=0.057), respectively). Of the 39 patients (71%) who needed postoperative radiation therapy, 5 patients (13%) had it delayed, and 3 patients (8%) did not receive any at all due to major complications. ConclusionIn our study, smoking was associated with the occurrence of major complications after groin STS resection and there was a strong trend for obesity and surgical bone exposure. Major complications were associated with a delay in starting postoperative radiation therapy. Thus, we recommend flap coverage after tumor resection in patients who have factors known to contribute to complications. Level of evidenceIV, Retrospective study.

Incidence of unplanned excisions of soft tissue sarcomas in the Netherlands: A population-based study

Timely recognition of soft tissue sarcomas (STS) remains challenging, potentially leading to unplanned excisions (also known as 'whoops procedures'). This population-based study charted the occurrence of unplanned excisions and identified associated patient, tumour, and treatment-related characteristics. Furthermore, it presents an overview of the outcomes and clinical management following an unplanned excision. From the Netherlands Cancer Registry (NCR) database, information was obtained on 2187 adult patients diagnosed with STS in 2016-2019 who underwent surgery. Tumours located in the mediastinum, heart or retroperitoneum were excluded, as well as incidental findings. Differences between patients with planned and unplanned excisions were assessed with chi-square tests and a multivariable logistic regression model. Overall, unplanned excisions comprise 18.2% of all first operations for STS, with a quarter of them occurring outside a hospital. Within hospitals, the unplanned excision rate was 14.4%. Unplanned excisions were more often performed on younger patients, and tumours unsuspected of being STS prior to surgery were generally smaller (≤5cm) and superficially located. Preoperative imaging was omitted more frequently in these cases. An unplanned excision more often resulted in positive margins, requiring re-excision. Patients who had an unplanned excision outside of a sarcoma centre were more often discussed at or referred to a sarcoma centre, particularly in case of residual tumour. Potential improvement in preventing unplanned excisions may be achieved by better compliance to preoperative imaging and referral guidelines, and stimulating continuous awareness of STS among general surgeons, general practitioners and private practices.

Abstract 11127: Clinical Heart Failure Events After Doxorubicin Treatment in High Grade Osteosarcoma Patients

Introduction: Osteosarcoma patients are at high risk of developing an anthracycline-induced cardiomyopathy (AC-CMP) due to their cancer treatment. The purpose of the study was to evaluate incidence of heart failure (HF) in this specific population and to identify predictors of HF. Methods: We conducted a retrospective cohort study at a tertiary sarcoma center (timeframe: 1982-2017) among 400 high grade osteosarcoma patients whom underwent surgery and received chemotherapy. Relevant data on demographics, cardiovascular risk profile, medications, osteosarcoma diagnosis and treatment was acquired from the electronic healthcare records and local oncology clinical registry. The primary endpoint was the composite of initiation of HF medication, hospitalization for HF and ICD implantation as primary prevention of sudden cardiac death in reduced left ventricular function. Results: The median age at sarcoma diagnosis in our cohort was 19 [p25-p75: 15-32] years with a median follow up time of 6 [2-14] years. At baseline, few patients used any medication or had cardiovascular risk factors: resp. 5 (1.3%) and 15 (3.8%). The endpoint occurred in 5.8% (23 patients). In 10 patients (2.5%) an admission for decompensated HF was the first presentation of AC-CMP, and ICD-implantation due to a reduced left ventricular ejection fraction occurred in 4 patients (1%). When sarcoma was diagnosed after 30 years of age, patients had near 5-fold increased risk of reaching the primary endpoint within 10 years after diagnosis (competing risk regression with competing risk of death due to malignancy: SHR 5.8 [2.0-16.9], p =0.001) and the time to a first HF event was shorter than younger patients: 3.4 [0.8-7.9] vs. 11.7 [5.2-16.0] years, p =0.027. Conclusions: The risk of developing HF in sarcoma patients is substantial, which warrants close monitoring during treatment and long term follow-up. Older age at diagnosis is associated with higher risk of developing HF and a shorter duration to events.

Impact of the national sarcoma guidelines on the prevalence and outcome of inadvertent excisions of soft tissue sarcomas: An observational study from a UK tertiary referral centre

ObjectivesThis study aims to investigate the impact of the national guideline on the prevalence and outcome in patients with soft-tissue sarcoma (STS) who had undergone inadvertent excisions. MethodsA total of 2336 patients were referred to a tertiary sarcoma centre from six regions (North East, North West, East Midlands, West Midlands, Wales, and South West) in the United Kingdom with a diagnosis of STS between 1996 and 2016, of whom 561 patients (24.0%) had undergone inadvertent excisions. Patients were categorised into two groups of 10-year periods pre and post the National Institute for Health and Clinical Excellence (NICE) guideline implementation in 2006. ResultsThe proportion of inadvertent excisions decreased after the NICE guideline implementation: 27.2% (pre-NICE) versus 19.8% (post-NICE) (p = 0.001). A substantial regional variation (17.4%–34.5%) in the proportion of inadvertent excisions in the pre-NICE era was reduced in the post-NICE era (14.3%–22.4%). The 5-year disease-specific survival was 77.7% (pre-NICE) versus 75.6% (post-NICE) (p = 0.961) and there was a trend toward lower incidence of local recurrence in the post-NICE era; 13.5% (pre-NICE) versus 10.5% (post-NICE) (p = 0.522). Multivariate analyses revealed that residual tumours in re-resection specimens were independently associated with an increased risk of disease-specific mortality (HR, 3.35; p < 0.001) and local recurrence (HR, 1.99; p = 0.017), which was significantly reduced after the NICE guideline implementation (53.2% versus 42.0%; p = 0.022). ConclusionsThe NICE guideline implementation reduced the proportion of patients with STS who had undergone inadvertent excisions and residual tumour in re-resection specimens, indicating an improved pre-referral management of STSs.

Accuracy and Safety of Ultrasound-Guided Core Needle Biopsy of Soft Tissue Tumors in an Outpatient Setting: A Sarcoma Center Analysis of 392 Consecutive Patients

Simple SummaryIn patients with unclear soft tissue lesions, treatment planning largely depends on histology. Core needle biopsy is the diagnostic standard in these cases. The aim of this retrospective study was to investigate accuracy and safety of ultrasound guided core needle biopsy at a tertiary referral center. We show that ultrasound guided biopsy was feasible as a one stop shop procedure in an outpatient setting in 87.6% of the cases. The rate of conclusive biopsies was 88.5%. After surgical resection, the dignity, tumor type and histopathological grading of the biopsy matched one of the resection specimens in 97.2%, 92.7% and 92.5% of the cases, respectively. Major complications occurred in 0.8% of the cases. With this study, we confirm that ultrasound guided core needle biopsy is safe, effective and can be performed at the first outpatient presentation to speed up the diagnostic and therapeutic cascade in sarcoma patients.Background: The aim of this study was to investigate diagnostic accuracy, safety and histologic results of ultrasound guided core needle biopsy (CNB) in patients with soft tissue lesions (STL) at a tertiary referral center. Methods: A retrospective analysis of all consecutive patients undergoing ultrasound guided CNB for STL at our sarcoma outpatient service between January 2015 and August 2020 was performed. Results: A total of 392 patients were identified. Main histologic entities were sarcomas, lipomas and desmoid tumors. Biopsy was performed in an outpatient setting in 87.6% of the cases. Conclusive biopsies were obtained in 88.5% of the cases. In patients who underwent surgical resection after CNB, the concordance of dignity, tumor entity and histopathological grading between biopsy and resection specimen were 97.2%, 92.7% and 92.5% respectively. The risk of inconclusive CNB was highest in intraabdominal or retroperitoneal tumors (19.5%) and lowest in lesions at the lower extremity (4.4%). Major complications after CNB occurred in three cases (0.8%). No case of biopsy tract seeding was observed during the study period. Conclusions: Ultrasound guided CNB for STL at first presentation in a dedicated surgical outpatient setting is a safe procedure and yields a high diagnostic accuracy.

Preoperative Radiation Performed at a Nonsarcoma Center May Lead to Increased Wound Complications Following Resection in Patients With Soft Tissue Sarcomas.

Preoperative radiation therapy (RT) followed by wide-local excision with or without chemotherapy is widely accepted as management for soft tissue sarcomas (STS). Although studies have demonstrated excellent local control with this technique, there can be significant morbidity with the development of wound complications. It has been shown that sarcoma resections performed at a high-volume center lead to improved survival and functional outcomes. It is unclear, however, if radiation performed in a high-volume center leads to improved outcomes especially related to morbidity. The goal of this study was to determine whether preoperative RT performed at an academic cancer center have lower rates of wound complication compared with RT performed in community cancer centers. A total of 204 patients with STS were treated with preoperative RT±chemotherapy followed by limb-sparing resection. Of these, 150 patients had preoperative RT performed at an academic sarcoma center. wound complication were defined as those requiring secondary operations or prolonged wound care for 4 months following surgery. Predictors for wound complication were evaluated using a Fisher exact test for univariate analysis and logistic regression for multivariate analysis. The overall incidence of wound complication was 28.3%. Significant predictors for wound complication include tumor location and radiation delivered at a community hospital. The postoperative incidence of wound complication was 21% when the preoperative RT was performed at an academic cancer center versus 39% when performed at a community cancer center (P=0.009). On multivariate analysis, both tumor location (P=0.0012, 95% confidence interval: 0.03-0.45, odds ratio: 0.13) and RT performed at a community cancer center (P=0.02, 95% confidence interval: 1.13-4.48, odds ratio: 2.25) remained significant in correlation with postoperative wound complication. Preoperative RT performed at an academic cancer center led to lower rates of postoperative wound complication. This may support the recommendation that preoperative RT and resection of STS be performed at an experienced sarcoma center.

Preoperative diagnostics and typing of abdominal soft tissue sarcomas

Abdominal sarcomas are aheterogeneous group of rare soft tissue tumors and can be localized intraperitoneally or retroperitoneally. Apretherapeutic differentiated subtyping is essential for planning an individual, multimodal treatment concept in an interdisciplinary team of experts. The central aspects of histology acquisition, imaging diagnostics and (molecular) pathological subtyping of abdominal soft tissue sarcomas are described in detail. Imaging and pathological diagnostics are depicted based on the German S3 guidelines on adult soft tissue sarcomas, acurrent literature search and personal experiences at the Sarcoma Center at the National Center for Tumor Diseases in Dresden (NCT/UCC). Preoperative imaging and (molecular) pathological subtyping of abdominal soft tissue sarcomas place high demands on surgeons, radiologists and pathologists. Genome analyses of sarcomas have the potential to identify points of attack for individualized treatment options. The limitations of resectability can only be assessed by experienced sarcoma surgeons at specialized centers. The treatment of abdominal soft tissue sarcomas at an experienced center is associated with abetter prognosis. Even at the first suspicion of an abdominal sarcoma, areferral to an experienced center should be made in order to guarantee optimal expertise in diagnostics and treatment.

Incidence, Treatment and Outcome of Patients with Retroperitoneal Soft-Tissue Sarcoma in Switzerland 2005\u20132015: A Population-Based Analysis

BackgroundReports on the epidemiology and mortality of retroperitoneal soft tissue sarcoma (RSTS) in Switzerland are scarce. This study investigates the incidence and outcomes of surgically treated RSTS inpatients in Switzerland depending on the hospital type and size.MethodsData from the Swiss Federal Statistical Office were used to conduct a retrospective analysis of all RSTS inpatients and hospitalizations in Switzerland between 2005 and 2015. RSTS was identified by the code C48.x of the International Classification of Diseases (ICD-10). Sarcoma centers were identified by the annual total number of sarcoma patients (> 50 patients/year). The analysis of yearly incidence, age distribution as well as in-hospital complication and mortality was performed for non- and surgical-treated patients. A centralization of treating sarcoma patients was analyzed by the trend of hospitalizations in sarcoma centers and high-volume hospitals.ResultsDuring 2005–2015, 2.801 hospitalizations (1651 patients) were admitted to Swiss hospitals with the primary diagnosis of a RSTS. The yearly number of RSTS patients and the incidence (1.91/100.000) stayed constant within these 11 years. There were five sarcoma centers. We saw a clear trend of RSTS patients being treated (especially surgically) in centers over the 11 years. The complication rate of surgical-treated patients was higher in sarcoma centers (55% vs. 40%), though the overall mortality rate was lower (3.2% vs. 9.1%).ConclusionCentralization of RSTS treatment to certified sarcoma centers leads to a lower overall mortality rate and thus is highly recommended.

Postnephrectomy outcomes following en bloc resection of primary retroperitoneal sarcoma: multicentre study.

This article reports on postoperative renal function in the largest series of patients with primary retroperitoneal sarcoma undergoing nephrectomy at three high-volume sarcoma centres. Although half of patients develop chronic kidney disease of stage 3 or higher, rarely do patients develop acute renal failure requiring renal replacement therapy.

Safe-margin surgery by plastic reconstruction in extremities or parietal trunk soft tissue sarcoma: A tertiary single centre experience

IntroductionTertiary centers recruit a large proportion of locally advanced or recurrent soft tissue sarcomas (STSs) that may have been preoperatively irradiated. The objective of this study was to evaluate the results of oncoplastic surgery (OPS) for patients affected by extremities or parietal trunk STS. Materials and methodsThis retrospective study includes patients who underwent a flap reconstruction after sarcoma resection between January 2018 and December 2020 at Institut Curie. The primary endpoint was the evaluation of the impact of OPS on the quality of surgical margins. The secondary endpoint was to quantify the morbidity of OPS and identify predictive factors for wound complications. ResultsOf 211 patients, 89 (42.2%) had a flap reconstruction. Surgery was realized on an irradiated field in 56 (62.9%) patients. Without OPS, all patients were candidates either for amputation (n = 9,10.1%) due to vessels/nerve infiltration, or R1/R2 resection (n = 80,89.9%). Seventy-two (80.0%) pedicle flaps and 18 (20.0%) free flaps were used. No R2 resections were performed. R0 and R1 margins were achieved in 82 (92.1%) and 7 (7.9%), respectively. The median closest margin was 3 mm (IQR 1–6 mm). Among R1 patients, 5 had positive margins along a preserved critical structure, 2 patients had well-differentiated liposarcomas. The surgical morbidity rate was 33.3% (30/90 flaps). The reoperation rate was 15.7% (14/89 patients). ConclusionsIn a referral sarcoma center, the collaboration between the surgical oncologist and the plastic surgery team should be considered upfront in the surgical plan, allowing the most adequate wide oncological resection with acceptable postoperative morbidity.

Treatment of 120 adult osteosarcoma patients with metachronous and synchronous metastases: A retrospective series of the French Sarcoma Group.

Treatment options for metastatic osteosarcomas are scarce. Following failure of standard first line therapy, patients who relapse present a challenging treatment dilemma, and have a poor prognosis. Surgical removal of all metastases is essential. A retrospective analysis of patients with metastatic osteosarcomas was conducted in 15 French Sarcoma Group centers. From January 2009 to December 2018, we identified 120 adult patients; 36 with synchronous and 84 with metachronous metastases with 74 males and 46 females. Mean age was 30 years (18-53). Metastatic sites were lung, bone and other in 91, 11 and 24 patients, respectively. Mean time to first metachronous metastases was 22 months (4-97). All patients except 13 (10.8%) with metachronous metastases received a first line systemic treatment for relapse, and 39 patients (32.5%) were included in a clinical trial. Eighty-one patients (67.5%) had local treatment of distant metastases. Median progression free survival (PFS) and overall survival (OS) were 5.5 (95% CI 4.6-6.4) and 20.5months (95% CI 13.2-27.7) respectively for the overall group. In multivariate analysis, more than five metastases, time to first metastases <24 months, were statistically significant negative prognostic factors for OS and PFS (P= .002, ≤.001 and P= .006, ≤.001, respectively). Surgery of metastases was associated with better prognosis on OS and PFS (P= .001 and .037, respectively). The presence of bone metastases was a negative prognostic factor on OS but not on PFS (P= .021). In reference sarcoma centers, relapsed osteosarcoma patients with more than one metastasis commonly receive more than one line of systemic therapy, and are included in clinical trial if available.

PULMONARY EPITHELIOID HEMANGIOENDOTHELIOMA: A RARE ENTITY WITH INSIDIOUS ONSET

TOPIC: Lung Cancer TYPE: Medical Student/Resident Case Reports INTRODUCTION: Sarcomas are rare, malignant tumors of connective tissue origin. Epithelioid hemangioendothelioma (EHE) is an exceedingly rare vascular sarcoma of endothelial origin, with an incidence of less than one per one million. Patients are typically asymptomatic or manifest symptoms related to organ involvement. We present a case of an asymptomatic woman with an incidental discovery of pulmonary EHE. CASE PRESENTATION: A 26 year old woman with no smoking history presented with vague abdominal pain. Her exam and initial labs were unremarkable. Abdominal imaging revealed nodules at the lung bases, and subsequent thoracic imaging revealed innumerable nodules throughout the lung parenchyma without pleural involvement. CT-guided transthoracic biopsy revealed filling and distension of alveolar spaces by a neoplastic proliferation of small, bland cells with perinuclear vacuoles within a hyalinized stroma. Immunohistochemical staining showed the neoplastic cells to be positive for ERG and CD31. These imaging and pathology findings were consistent with stage IV metastatic pulmonary EHE. She was referred to a sarcoma center of excellence, and surveillance was recommended. DISCUSSION: EHE is a vascular subtype of sarcoma of low-grade malignancy most commonly involving the liver and lungs. Pulmonary EHE can be asymptomatic or present with non-specific symptoms of dyspnea, cough, hemoptysis, or weight loss. Diagnosis is made with histopathologic epithelioid morphology and immunohistochemical vascular characteristics. Our patient's biopsy tissue stained positive for markers of vascular differentiation. CD34 and Factor VIII-related antigen are additional markers that may be used to identify vascular features of EHE. Disease course is unpredictable, and factors associated with a poor prognosis include pleural effusion and hemoptysis. Disease rarity precludes established treatment guidelines. Surgical resection should be considered for solitary lesions. Past reports have shown some responsiveness to taxane, anthracycline, or IFN-2a therapy. Anti-VEGF therapies show promise for eliciting partial response or as maintenance therapy. Still, many cases are indolent and a surveillance strategy may be reasonable. In our patient, surveillance was supported by her asymptomatic disease without poor prognostic factors. The development of significant symptoms or progressive disease would prompt further consideration of treatment. CONCLUSIONS: EHE is an extremely rare sarcoma subtype most commonly affecting the liver or lungs. Diagnosis is made on immunohistopathology and treatment options include surgical resection, surveillance, or anti-angiogenic agents. It is an important, albeit uncommon diagnosis to consider for diffuse pulmonary nodules seen on imaging. REFERENCE #1: Semenisty V, Naroditsky I, Keidar Z, Bar-Sela G. Pazopanib for metastatic pulmonary epithelioid hemangioendothelioma—a suitable treatment option: case report and review of anti-angiogenic treatment options. BMC Cancer. 2015;15(1):402. doi:10.1186/s12885-015-1395-6 REFERENCE #2: Chevreau C, Le Cesne A, Ray-Coquard I, et al. Sorafenib in patients with progressive epithelioid hemangioendothelioma: A phase 2 study by the French Sarcoma Group (GSF/GETO). Cancer. 2013;119(14):2639-2644. doi:10.1002/cncr.28109 REFERENCE #3: Agulnik M, Yarber JL, Okuno SH, et al. An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas. Ann Oncol. 2013;24(1):257-263. doi:10.1093/annonc/mds237 DISCLOSURES: No relevant relationships by Mohammad Ahmed, source=Web Response No relevant relationships by Gustavo Cumbo-Nacheli, source=Web Response No relevant relationships by Connor Kerndt, source=Web Response No relevant relationships by Ranuka Sinniah, source=Web Response

A Highly Reliable Convolutional Neural Network Based Soft Tissue Sarcoma Metastasis Detection from Chest X-ray Images: A Retrospective Cohort Study.

Simple SummarySoft tissue sarcomas are relatively rare malignant diseases. Part of the diagnosis and follow-up includes medical imaging of the thorax for detection of lung metastases. A Python script was created and trained using a set of lung X-rays and concordant CT scans from a high-volume German-speaking sarcoma center. It is capable of detecting malignant metastasis in the lung with a precision of 71.2%, specificity of 90.5%, sensitivity of 94% and accuracy of 91.2%. Furthermore, the program was able to detect even small nodules with a size <1 cm in conventional X-rays of the thorax. This algorithm was implemented into our daily clinical practice alongside with the radiologists’ findings. With this tool we aim to improve the quality of our service and reduce the expenditure of time.Introduction: soft tissue sarcomas are a subset of malignant tumors that are relatively rare and make up 1% of all malignant tumors in adulthood. Due to the rarity of these tumors, there are significant differences in quality in the diagnosis and treatment of these tumors. One paramount aspect is the diagnosis of hematogenous metastases in the lungs. Guidelines recommend routine lung imaging by means of X-rays. With the ever advancing AI-based diagnostic support, there has so far been no implementation for sarcomas. The aim of the study was to utilize AI to obtain analyzes regarding metastasis on lung X-rays in the most possible sensitive and specific manner in sarcoma patients. Methods: a Python script was created and trained using a set of lung X-rays with sarcoma metastases from a high-volume German-speaking sarcoma center. 26 patients with lung metastasis were included. For all patients chest X-ray with corresponding lung CT scans, and histological biopsies were available. The number of trainable images were expanded to 600. In order to evaluate the biological sensitivity and specificity, the script was tested on lung X-rays with a lung CT as control. Results: in this study we present a new type of convolutional neural network-based system with a precision of 71.2%, specificity of 90.5%, sensitivity of 94%, recall of 94% and accuracy of 91.2%. A good detection of even small findings was determined. Discussion: the created script establishes the option to check lung X-rays for metastases at a safe level, especially given this rare tumor entity.