Sarcoma Center Research Articles

Management Strategies and Outcomes in Primary Liver Angiosarcoma.

Primary hepatic angiosarcoma is a rare tumor of the liver that originates from endothelial and fibroblastic tissue, with poor prognosis and lack of standardized treatment. We retrospectively analyzed the clinical characteristics and treatment outcomes of 23 patients with primary liver angiosarcoma treated at an academic sarcoma center. We screened all patients with primary liver angiosarcoma treated at Stanford between 2000 and 2022. Data was collected from EPIC electronic medical records and included patient demographics, tumor characteristics, treatment modalities, and patient outcomes. Statistical analysis was completed using Python 3.0, while survival curves were generated using the Kaplan-Meier method and Lifelines Packages. There were nearly equal numbers of males (11) and females (12) in our study, with most patients aged 70 to 79 at diagnosis. The median overall survival (OS) was 6 months (range 0.07 to 222.6 mo). The 2- and 5-year OS were both 38.6%. 71% of patients received systemic treatment with chemotherapy, while 29% received immunotherapy. Local treatment with surgery or radioembolization was performed in 14% of patients. Three patients in our study displayed particularly improved OS and received various treatments, which ranged from hepatic resection to ipilimumab/nivolumab. Our study demonstrated that primary liver angiosarcoma has poor outcomes despite treatment. Surgical resection with negative margins is the only curative modality. However, most patients present with advanced disease and are not surgical candidates. Further research is needed to identify more effective systemic therapy options for this devastating disease.

Association of chemotherapy dose intensity and age with outcomes in patients with Ewing's family sarcoma.

Ewing's family sarcoma (EFS) is an aggressive malignancy with a peak incidence in adolescents. Multimodal treatment involves surgery and/or radiotherapy, and chemotherapy typically with VDC/IE (vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide). There is a paucity of data for the treatment of adults, with protocols extrapolated from the pediatric setting. This study aimed to assess patterns of care, chemotherapy tolerability across age groups, and outcomes from four Australian sarcoma centers. ANZSA ACCORD sarcoma database and medical records were used to identify and collect data of patients aged≥10 years with EFS who received VDC/IE between 2010 and 2020. Survival outcomes were analyzed based on chemotherapy received dose intensity (RDI). Clinical predictors of RDI were explored using logistic regression. Of 146 patients with EFS, 76 received VDC/IE. The majority had localized disease (65%). Seventy-one percent completed scheduled chemotherapy, with some requiring dose reduction (29%), delay>7 days (65%), or cycle omission (4%). Hematological toxicity was the main reason for dose reduction/delay. Fifty-seven percent patients achieved an acceptable RDI ≥85%. Compared to those aged 10-19, the odds ratio for acceptable RDI aged 40-59 was 0.20 (95% CI 0.04-0.86, p=0.04). RDI was an independent prognostic factor for overall survival, after accounting for age, gender, Ewing's type, primary site, and stage (adjusted HR 0.25 [95% CI 0.10-0.63], p=0.004). Survival outcomes in EFS were associated with chemotherapy RDI. Older adults more commonly required dose reduction or early cessation of treatment due to toxicity. VDC/IE chemotherapy should be carefully tailored in adults>40 years.

Biopsies for Soft-Tissue Sarcoma of the Hand: Dangers, Hazards, and Outcomes.

Excisional biopsies for soft-tissue sarcoma (STS) of the hand are commonly done outside of sarcoma centers and can compromise incorporation of the biopsy site into standard limb salvage or amputation flaps. We sought to identify risk factors for these suboptimal biopsies. We analyzed prospective data on all patients (N = 109) who underwent definitive resection of primary STS of the hand between 1995 and 2019 at our institution. Biopsies were classified by type (excisional vs. incisional/needle), whether they were done before referral, and whether the incision could be incorporated into standard limb salvage or amputation flaps (ILS biopsies) or not (NILS biopsies). Analyses examined potential predictors of NILS biopsies and whether outcomes differed by biopsy type. Biopsies done before referral (N = 91) were more likely to be excisional (79% vs. 17%). Excisional biopsies were associated with smaller tumor size (median, 2.0 vs. 3.15 cm; P = 0.025) and longer time to first intervention (1.88 vs. 1.17 months; P = 0.001). Forty-eight percent of excisional and 29% of incisional biopsy sites required soft-tissue coverage at the time of definitive surgery ( P = 0.07). Biopsy type was not associated with Musculoskeletal Tumor Society score or need for amputation. Risk factors for NILS biopsies included larger tumor size, deep tumor, and excisional biopsy. High-risk areas for NILS biopsies included the carpal tunnel, volar wrist, first webspace, radial palm, and proximal thumb. NILS biopsies were associated with positive margins, need for soft-tissue coverage, and lower Musculoskeletal Tumor Society scores. This study informs referral guidelines for patients with STS of the hand. Patients with tumors that are deep, large, or in high-risk locations should be referred to a sarcoma center before biopsy. If that is not possible, incisional biopsy in line with standard resection incisions or radiology-guided core needle biopsy is preferable to excisional biopsy. Prognostic study. Level II.

Distal Humeral Replacement in Patients with Primary Bone Sarcoma: The Functional Outcome and Return to Sports

Distal humeral replacement (DHR) is a limb-salvage option for the endoprosthetic reconstruction of bone defects following the resection of a primary bone sarcoma. As primary bone sarcomas are only occasionally located around the distal humerus, there is a paucity of information regarding postoperative function, and patients’ resumption of sporting activities. With advances in diagnostics and in surgical and oncological treatment leading to an increased patient life expectancy and higher quality of life, patients’ functional outcome and return to sports activities are of increasing interest. Between 1997 and 2021, a total of 24 patients underwent DHR with a single-design modular implant at a tertiary sarcoma center. A total of 14 patients who died of their disease were excluded, leaving a study cohort of 10 patients, with a median age of 30 years on the day of surgery (IQR 20–37). At the last follow-up, after a median of 230 months (IQR 165–262), the median MSTS was 19 (IQR 13–24), the median TESS was 79 (IQR 66–87), the median SEV was 38% (IQR 24–53), the median TS was 6 (IQR 4–7), and the median WAS was 3 (IQR 1–8). Among the variables of gender, surgery on the dominant extremity, intraoperative nerve resection, extra-articular tumor resection, chemotherapy, radiotherapy, and revision surgeries, none were associated with a better/lower functional outcome score or return to sports activities. However, a higher level of sports performance prior to diagnosis (WAS > 10) was associated with a higher level of sports performance postoperatively (p = 0.044).

Editorial introductions.

Editorial introductions.

President Biden Chooses Monica M. Bertagnolli, MD, to Lead the NIH

President Biden Chooses Monica M. Bertagnolli, MD, to Lead the NIH

MRI radiomics-based machine learning for classification of deep-seated lipoma and atypical lipomatous tumor of the extremities

PurposeTo determine diagnostic performance of MRI radiomics-based machine learning for classification of deep-seated lipoma and atypical lipomatous tumor (ALT) of the extremities.Material and methodsThis retrospective study was performed at three tertiary sarcoma centers and included 150 patients with surgically treated and histology-proven lesions. The training-validation cohort consisted of 114 patients from centers 1 and 2 (n = 64 lipoma, n = 50 ALT). The external test cohort consisted of 36 patients from center 3 (n = 24 lipoma, n = 12 ALT). 3D segmentation was manually performed on T1- and T2-weighted MRI. After extraction and selection of radiomic features, three machine learning classifiers were trained and validated using nested fivefold cross-validation. The best-performing classifier according to previous analysis was evaluated and compared to an experienced musculoskeletal radiologist in the external test cohort.ResultsEight features passed feature selection and were incorporated into the machine learning models. After training and validation (74% ROC-AUC), the best-performing classifier (Random Forest) showed 92% sensitivity and 33% specificity in the external test cohort with no statistical difference compared to the radiologist (p = 0.474).ConclusionMRI radiomics-based machine learning may classify deep-seated lipoma and ALT of the extremities with high sensitivity and negative predictive value, thus potentially serving as a non-invasive screening tool to reduce unnecessary referral to tertiary tumor centers.

The Diagnosis and Treatment of Osteosarcoma and Ewing's Sarcoma in Children and Adolescents.

Osteosarcoma and Ewing's sarcoma in children and adolescents require age-specific interdisciplinary diagnosis and treatment to achieve optimal therapeutic outcomes. The diagnosis and treatment of malignant bone tumors in childhood and adolescence are presented in the light of publications retrieved by a selective search, pertinent guidelines, and the authors' extensive experience in an interdisciplinary cancer center. Bone sarcomas make up approximately 5% of all malignancies in children and adolescents; the most common types are Ewing's sarcoma and osteosarcoma. Patients are often not referred to a specialized center until long after the onset of symptoms, as they and their physicians rarely consider the possibility of a bone tumor, and the symptoms are often trivialized. Bone pain of unknown origin, swelling, and functional limitations should be investigated with conventional x-rays. Lesions of unclear origin should be biopsied after a meticulous clinical and radiologic evaluation. Multimodal treatment consists of neo - adjuvant chemotherapy, limb-preserving resection if possible, and radiotherapy where indicated. In multicenter studies, patients with osteosarcoma achieve event-free survival in 64% of cases if their disease is localized, and 28% if it is metastatic; the corresponding figures for patients with Ewing's sarcoma are 80% and 27%, respectively. With implementation of the current treatment recommendations, most children and adolescents with malignant bone tumors can be treated successfully with curative intent. These patients should be referred to a sarcoma center for diagnosis and treatment.

Racial and ethnic disparities in presentation and outcomes of patients with gastrointestinal stromal tumors (GIST) in the era of targeted therapy.

e18573 Background: GIST is the most common malignant mesenchymal tumor of the gastrointestinal tract and is a compelling model for targeted therapies. Racial and ethnic minorities are underrepresented in clinical research, and information regarding disparities in GIST is limited. Our sarcoma center is located within a large and diverse population of Hispanic, LatinX, and African American/African Caribbean patients (pts). We aimed to identify racial and ethnic disparities among pts with GIST. Methods: We retrospectively reviewed all pts with primary or recurrent GIST between January 2003 and October 2022 in our institution. All data were retrieved from electronic medical records. SPSS Statistics was utilized, Fisher exact test and log-rank test were used for analysis. A two-sided p < 0.05 was considered statistically significant. Results: A total of 1150 pts had a diagnosis of GIST during the study period. Information regarding race and ethnicity was available in 711 pts. There were 42.4% White Non-Hispanic/LatinX (WN), 33.2% Hispanic/LatinX (HL), and 24.4% Black Non-Hispanic/LatinX (BN). The stomach was the most common primary site in all three groups, especially in the BN population (80.9%) vs WN (53%) and HL (61.9%), followed by the small intestine (p < 0.001). The WN pts more commonly presented with metastatic disease on diagnosis (16.2%) vs BN (12.7%) and HL (8.9) (p = 0.042) and had higher grade tumors (37.7%) vs BN (24.9%) and HL (23.7%) (p < 0.001). Median disease-free survival (mDFS) after radical resection was significantly lower in BN population vs WN and HL (p = 0.014). Disease-specific survival (DSS) in BN was also significantly reduced compared to the other groups (p = 0.024). A total of 445 pts had some molecular testing done, and BN were significantly less tested (38.5%) (p < 0.001). The most common primary driver mutation was KIT exon 11 throughout all groups, especially in the BN group corresponding to 80% of the cases tested (p = 0.008). The median overall survival (mOS) among pts with metastatic disease was 7.1 years (95% CI = 5.6-8.6). The HL population had a trend toward the longest mOS (8.8 years), followed by the WN (7.7 years), followed by the BN population (5.8 years) (p = 0.073). A total of 75 of these patients were tested for secondary mutations, with no statistical difference among the groups. Conclusions: We found in our study the WN population was significantly more likely to present with metastatic disease and higher-grade tumors than BN or HL pts. Nonetheless, the BN population were substantially less likely to undergo mutation testing and had a trend toward a worse mOS by more than 1 year. All pts had their care within the same healthcare system, implicating access to mutation testing and socioeconomic barriers playing a role in inferior outcomes. Further studies are needed to understand molecular differences and decrease the existing gap among these populations.

Risk stratification tools to help decide on adjuvant chemotherapy usage in resected soft tissue sarcomas: A 10 year review of an Irish sarcoma centre experience.

11575 Background: Soft tissue sarcoma (STS) is comprised of 80 pathologic subtypes based on a combination of distinctive morphological, immunohistochemical and molecular features. Adult-type soft tissue and visceral sarcomas are rare, with an estimated incidence of 4-5/100,000/year in Europe. The National Comprehensive Cancer Network (NCCN) guidelines recommend consideration of neo-adjuvant or adjuvant systemic treatment in resectable stage III (high grade tumours which are >5cm) and also in those who need to undergo an amputation or radical resection with adverse functional outcomes. The Sarculator risk prediction tool has identified a threshold of risk above which the administration of chemotherapy may provide an overall survival benefit. This can be applied to the most common histologic subtypes. Patients affected by these subtypes and with a 10-year predicted OS likelihood <60% are considered as high risk and therefore, should be considered for adjuvant chemotherapy. Alternatively, tumours considered at high risk of recurrence based on size >5cm and high grade histology, may benefit from adjuvant chemotherapy. Therefore, the aim of this project is to review the outcomes of resected extremity/trunk soft tissue sarcoma and assess the prognostic accuracy of these risk prediction methods. Methods: All new patients with resected STS discussed in the STS MDT in Cork University Hospital between Jan 2012 – Dec 2021 were identified. The histology and imaging of the identified patients were reviewed. Data regarding demographics, histology, treatment and outcomes was collected. Risk assessment using AJCC and Sarculator score was done on all patients with an extremity or trunk sarcoma. Overall survival was recorded and assessed including Kaplan Meier method for time to event analysis. Results: 200 patients were identified as having an STS resected - 134 of these were located on the trunk or extremities representing 24 different histological subtypes. Sarculator score was calculated for 60 of these (well differentiated liposarcomas, desmoid tumours, and dermatofibrosarcoma protuberans were excluded). 3 patients received adjuvant chemotherapy and 4 patient neoadjuvant chemotherapy. Overall survival data is presented below. Conclusions: Our cohort is representative of the broad histological subtypes expected in sarcoma. Sarculator score results correlate with international outcomes, with higher scores associated with increased mortality. In our cohort, sarculator score was more predictive of outcome than tumour grade/size alone. [Table: see text]

Exploring the Potential of Electronic Patient-Reported Outcome Measures to Inform and Assess Care in Sarcoma Centers: A Longitudinal Multicenter Pilot Study.

Electronic patient-reported outcome measures (ePROMs) are useful tools to assess care needs of patients diagnosed with cancer and to monitor their symptoms along the illness trajectory. Studies regarding the application of ePROMs by advanced practice nurses (APNs) specialized in sarcoma care and the use of such electronic measures for care planning and assessing quality of care are lacking. To explore the potential of ePROMs in clinical practice for assessing the patient's quality of life, physical functionality, needs, and fear of progression, as well as distress and the quality of care in sarcoma centers. A multicenter longitudinal pilot study design was chosen. Three sarcoma centers with and without APN service located in Switzerland were included. The instruments EQ-5D-5L, Pearman Mayo Survey of Needs, the National Comprehensive Cancer Network Distress Thermometer, PA-F12, and Toronto Extremity Salvage Score were used as ePROMs. Data were analyzed descriptively. Overall, 55 patients participated in the pilot study; 33 (60%) received an intervention by an APN, and 22 (40%) did not. Patients in sarcoma centers with APN service reported overall higher scores in quality of life and functional outcome. The number of needs and distress level were lower in sarcoma centers with APN service. No differences were found with respect to patients' fear of progression. Most of the ePROMs proved to be reasonable in clinical practice. PA-F12 has shown low clinical relevance. Using ePROMs appears to be reasonable to obtain clinically relevant patient information and to evaluate the quality of care in sarcoma centers.

Desmoid Tumors in Familial Adenomatous Polyposis Patients: Favorable Outcomes with Multidisciplinary Management.

In this study, we aimed to describe the clinical features, management, and outcomes of desmoid tumors (DTs) in familial adenomatous polyposis (FAP) patients at a high-volume sarcoma center. Consecutive patients with FAP and DTs were identified from our institutional databases (1985-2021). Patient demographics, treatment, and outcomes were described. Categorical data were compared using Fisher's exact test, and Kaplan-Meier curves were used to estimate progression-free survival (PFS). Forty-five patients with 67 DTs were identified: 39 mesenteric or retroperitoneal (58.2%), 17 abdominal wall (25.4%), 4 extremity (6%), 4 breast (6%) and 3 back (4.4%). Severe DT symptoms were present in 12 patients (26.7%). Initial treatments per tumor were observation in 30 (44.8%) DTs, chemotherapy in 15 (22.4%) DTs, surgery in 10 (14.9%) DTs, and other systemic therapies in 10 (14.9%) DTs. The majority of DTs remained stable with observation or a single intervention (77.8%). Median PFS was 23.4 years (95% confidence interval 7.6-39.2). In the 12 severely symptomatic patients, four patients required more than two interventions for DT control. At a median follow-up of 6.0 years (range 0.7-35.8 years), 33 (73.3%) patients were alive with disease, 7 (15.6%) were alive without disease, and 5 (11.1%) died of other causes. No patients died of DT-related complications. The majority of DTs in FAP patients remained stable with observation or a single intervention. There were no DT-related deaths; however, 12 of 45 patients (26.7%) experienced significant tumor morbidity and required more interventions for disease control. Further studies on quality of life are required.

Symptomatic Neuroma Development following En Bloc Resection of Skeletal and Soft-Tissue Tumors: A Retrospective Analysis of 331 Cases.

Although symptomatic neuroma formation has been described in other patient populations, these data have not been studied in patients undergoing resection of musculoskeletal tumors. This study aimed to characterize the incidence and risk factors of symptomatic neuroma formation following en bloc resection in this population. The authors retrospectively reviewed adults undergoing en bloc resections for musculoskeletal tumors at a high-volume sarcoma center from 2014 to 2019. The authors included en bloc resections for an oncologic indication and excluded non-en bloc resections, primary amputations, and patients with insufficient follow-up. Data are provided as descriptive statistics, and multivariable regression modeling was performed. The authors included 231 patients undergoing 331 en bloc resections (female, 46%; mean age, 52 years). Nerve transection was documented in 87 resections (26%). There were 81 symptomatic neuromas (25%) meeting criteria of Tinel sign or pain on examination and neuropathy in the distribution of suspected nerve injury. Factors associated with symptomatic neuroma formation included age 18 to 39 [adjusted OR (aOR), 3.6; 95% CI, 1.5 to 8.4; P < 0.01] and 40 to 64 (aOR, 2.2; 95% CI, 1.1 to 4.6; P = 0.04), multiple resections (aOR, 3.2; 95% CI, 1.7 to 5.9; P < 0.001), preoperative neuromodulator requirement (aOR, 2.7; 95% CI, 1.2 to 6.0; P = 0.01), and resection of fascia or muscle (aOR, 0.5; 95% CI, 0.3 to 1.0; P = 0.045). The authors' results highlight the importance of adequate preoperative optimization of pain control and intraoperative prophylaxis for neuroma prevention following en bloc resection of tumors, particularly for younger patients with a recurrent tumor burden. Risk, III.

A Retrospective Multi-Institutional Cohort Analysis of Clinical Characteristics and Outcomes in Dedifferentiated Chondrosarcoma.

Dedifferentiated chondrosarcoma (DDCS) is a rare subset of chondrosarcoma. It is an aggressive neoplasm characterized by a high rate of recurrent and metastatic disease with overall poor outcomes. Systemic therapy is often used to treat DDCS; however, the optimal regimen and timing are not well defined, with current guidelines recommending following osteosarcoma protocols. We conducted a multi-institutional retrospective analysis of clinical characteristics and outcomes of patients with DDCS. Between 1 January 2004 and 1 January 2022, the databases from five academic sarcoma centers were reviewed. Patient and tumor factors, including age, sex, tumor size, site, location, the treatments rendered, and survival outcomes, were collected. Seventy-four patients were identified and included in the analysis. Most patients presented with localized disease. Surgical resection was the mainstay of therapy. Chemotherapy was used predominantly in the metastatic setting. Partial responses were low (n = 4; 9%) and occurred upon treatment with doxorubicin with cisplatin or ifosfamide and single-agent pembrolizumab. For all other regimens, stable disease was the best response. Prolonged stable disease occurred with the use of pazopanib and immune checkpoint inhibitors. DDCS has poor outcomes and conventional chemotherapy has limited benefit. Future studies should focus on defining the possible role of molecularly targeted therapies and immunotherapy in the treatment of DDCS.

Experiences of patients and their relatives of postoperative radiological surveillance and surveillance intensity following primary resection of a soft tissue sarcoma and its impact on their quality of life: a systematic review protocol

IntroductionPostoperative radiological surveillance following primary resection of a soft tissue sarcoma (sarcoma of the retroperitoneum, abdomen, pelvis, trunk or extremities) is standard of care in all international high-volume sarcoma centres...

44. Symptomatic Neuroma Formation following Skeletal and Soft Tissue Tumor Resection

PURPOSE: While symptomatic neuroma formation has been described in other patient populations, these data have not been studied in patients undergoing resection of musculoskeletal tumors. This study aims to characterize the incidence and risk factors of symptomatic neuroma formation following en bloc resection in this population. METHODS: We retrospectively reviewed adults undergoing en bloc resections for musculoskeletal tumors at a high-volume sarcoma center from 2014-2019. We included en bloc resections for an oncologic indication and excluded non-en bloc resections, primary amputations, and patients with insufficient follow-up. Data are provided as descriptive statistics and multivariable regression modeling was performed. RESULTS: We included 231 patients undergoing 331 en bloc resections (46% female; mean age 52 years). Nerve transection was documented in 87 resections (26%). There were 81 symptomatic neuromas (25%) meeting criteria of Tinel sign or pain on exam and neuropathy in the distribution of suspected nerve injury. Factors associated with symptomatic neuroma formation included age 18-39 (aOR, 3.6; 95% CI, 1.5-8.4; p < 0.01) and 40-64 (aOR, 2.2; 95% CI, 1.1-4.6; p = 0.04), multiple resections (aOR, 3.2; 95% CI, 1.7-5.9; p < 0.001), preoperative neuromodulator requirement (aOR, 2.7; 95% CI 1.2-6.0; p = 0.01) and resection of fascia or muscle (aOR, 0.5; 95% CI, 0.3-1.0; p = 0.045). CONCLUSION: Our results highlight the importance of adequate preoperative optimization of pain control and intraoperative prophylaxis for neuroma prevention following en bloc resection of tumors, particularly for younger patients with a recurrent tumor burden.

Does Multidisciplinary Therapy at a Sarcoma Center Improve the Prognosis of Patients With Soft Tissue Sarcoma? A Retrospective Case Study at a Single Institute.

The effect of multidisciplinary therapy conducted at the sarcoma center of our hospital was examined to determine whether therapy undertaken here improved the prognosis of patients with soft-tissue sarcoma. The clinical findings and prognoses of patients treated before the establishment of the sarcoma center (72 patients from April 2016 to March 2018) and those treated after (155 patients from April 2018 to March 2021) were compared. The mean number of patients increased from 36.0 to 51.7 per year after the establishment of the sarcoma center. The proportion of patients with stage IV disease also increased from 8.3% to 12.9% after establishment of the sarcoma center. The 3-year survival rate of patients, considering all stages, decreased from 80.0% to 78.3% after establishment of the sarcoma center rather than showing an increase. The 3-year survival rate of patients with stage II and III disease increased from 78.6% to 84.7%, and that of stage III patients with retroperitoneal sarcoma increased from 70.0% to 86.7% after establishment of the sarcoma center. However, no statistically significant difference was observed in the survival curves. The establishment of a sarcoma center has contributed to centralizing treatment for soft-tissue sarcoma. Multidisciplinary therapy at sarcoma centers may improve the prognosis of patients with soft-tissue sarcomas.

Flap Reconstruction Outcome Following Surgical Resection of Soft Tissue and Bone Sarcoma in the Setting of (Neo)adjuvant Therapy: A Sarcoma Center Experience.

Soft tissue and bone sarcomas are heterogeneous groups of malignant tumors. The shift in their management, with an emphasis on limb salvage, has deemed the involvement of reconstructive surgeons an integral part of their multidisciplinary treatment. We present our experience with free and pedicled flaps in the reconstruction of sarcomas at a tertiary referral university hospital and major sarcoma center. All patients undergoing flap reconstruction after sarcoma resection over a 5-year period have been included in the study. Patient-related data and postoperative complications were collected retrospectively, ensuring a minimum follow-up of 3 years. A total of 90 patients underwent treatment with 26 free flaps and 64 pedicled flaps. Postoperative complications occurred in 37.7% of patients, and the flap failure rate was 4.4%. Diabetes, alcohol consumption and male gender were associated with increased early necrosis of the flap. Preoperative chemotherapy significantly increased the occurrence of early infection and late dehiscence, while preoperative radiotherapy was associated with a higher incidence of lymphedema. Intraoperative radiotherapy was associated with late seromas and lymphedema. Reconstructive surgery with either pedicled or free flaps is reliable, but it can be demanding in the setting of sarcoma surgery. A higher complication rate is to be expected with neoadjuvant therapy and with certain comorbidities.

Primary retroperitoneal sarcoma: A comparison of survival outcomes in specialist and non-specialist sarcoma centres

BackgroundConsensus guidelines outline that patients with primary retroperitoneal sarcoma (RPS) should be managed within specialist sarcoma centres (SSC). There is, however, a paucity of population-based data detailing incidence and outcomes in these patients. Hence, we aimed to evaluate patterns of care among RPS patients in England and compare outcomes for those undergoing surgery in high-volume specialist sarcoma centres (HV-SSC), low-volume SSC (LV-SSC), and non-SSC (N-SSC). MethodsData on patients diagnosed with primary RPS between 2013 and 2018 were extracted from NHS Digital’s National Cancer Registration and Analysis Service using the national cancer registration dataset. Diagnostic pathways, treatment, and survival outcomes were compared between HV-SSC, LV-SSC, and N-SSC. Uni- and multivariate analyses were calculated. ResultsOf 1878 patients diagnosed with RPS, 1120 (60%) underwent surgery within 12 months of diagnosis, with 847 (76%) operated on at SSC; of these, 432 patients (51%) were operated on in HV-SSC, and 415 (49%) in LV-SSC. One- and 5-year estimated overall survival (OS) rates for patients undergoing surgery in N-SSC were 70.6% (95% confidence interval [CI]: 64.8–75.7) and 42.0% (CI: 35.9–47.9), compared to 85.0% (CI: 81.1–88.1) and 51.7% (CI: 46.6–56.6) in LV-SSC (p < 0.01), and 87.4% (CI: 83.9–90.2) and 62.8% (CI: 57.9–67.4) in HV-SSC, (p < 0.01). After adjusting for patient- and treatment-related factors, patients treated in HV-SSC were found to have significantly longer OS than those treated at LV-SSC, with an adjusted hazard ratio of 0.78 (CI: 0.62–0.96, p < 0.05). ConclusionPatients with RPS undergoing surgery in HV-SSC have significantly better survival outcomes than those treated in N-SSC and L-SSC.

Chondrosarcoma of the Proximal Humerus: Does the Margin Affect Survival?

Chondrosarcoma (CS) is the second most common primary malignant bone tumour and, in the absence of reliable chemotherapy and radiotherapy, is effectively a surgical disease. Overall disease specific survival (DSS) is affected by tumour grade, whilst resection margin contributes to local recurrence free survival (LRFS). The aim of this study was to investigate factors that affect the local and systemic prognoses for conventional central CSs arising from the proximal humerus. A multi-centre, retrospective study from three international collaborative sarcoma centres identified 110 patients between 1995 and 2020 undergoing treatment for a conventional central CS of the proximal humerus; 58 patients (53%) had a grade 1 tumour, 36 (33%) had a grade 2 tumour, and 16 patients (13%) had a grade 3 CS. The mean age of patients was 50 years (range 10-85). The incidence of local recurrence (LR) was 9/110 (8.2%), and the disease specific mortality was 6/110 (5.5%). The grade was a statistically significant factor for LRFS (p < 0.001). None of the grade 1 tumours developed LR. The DSS was affected by the grade (p < 0.001) but not by the LR (p = 0.4). Only one patient with a grade 2 tumour died from the disease. The proximal humeral grade 1 CS behaved as a benign tumour, having no cases of LR nor death due to disease. Grade 2 CSs of the proximal humerus behaved in a more indolent way when compared with comparable grade tumours elsewhere in the appendicular skeleton, being locally aggressive with a higher LR rate than grade 1 CSs but still having very low mortality and a high rate of DSS. The LR in grade 2 CSs did not affect the DSS; therefore, surgical management in proximal humeral grade 2 CSs should have a greater emphasis on preserving function whilst maintaining an adequate margin for resection. The proximal humeral grade 3 CS was, as elsewhere in the skeleton, an aggressive, high-grade tumour. Therefore, surgical management should include en bloc resection with clear margins to avoid LR.