Sarcoma[JOURNAL]
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200 papers
Oral Etoposide for Relapsed or Refractory Ewing Sarcoma in Adolescent and Adult Patients.
Kostos L, Rayson V, Desai J … +5 more , Orme L, Bae S, Hamilton A, Luen SJ, Lewin J
Sarcoma · 2024 · PMID 39713774 · Full text
Prognosis remains poor for patients with relapsed or refractory Ewing sarcoma, with limited treatment options after first-line therapy. Oral etoposide has efficacy in the paediatric setting; however, data are limited in...
Prognosis remains poor for patients with relapsed or refractory Ewing sarcoma, with limited treatment options after first-line therapy. Oral etoposide has efficacy in the paediatric setting; however, data are limited in adults. A retrospective analysis was conducted on 33 patients with relapsed or refractory Ewing sarcoma who completed at least one cycle of oral etoposide at the Peter MacCallum Cancer Centre from 2005 to 2020. The median age at diagnosis and first relapse was 21 and 23 years, respectively. All patients had prior exposure to intravenous etoposide. Nine patients (27%) had stable disease for at least 6 months, and six patients (18%) had a partial response. The clinical benefit rate was 45%. The median PFS was 3.6 months (95% CI: 1.7-5.5), and OS was 8.5 months (95% CI: 4.1-13.0). Despite prior exposure, oral etoposide demonstrated antitumour activity and durable responses in the relapsed or refractory setting for adult patients with Ewing sarcoma.
Incidence of Undifferentiated Pleomorphic Sarcoma (UPS) in the United States.
Ma J, Groisberg R, Shao C … +1 more , Zhong W
Sarcoma · 2024 · PMID 39502684 · Full text
The classification of undifferentiated pleomorphic sarcoma (UPS) has been evolving with advances in immunohistochemistry and genomic profiling over the past 20 years. There is a lack of current information on UPS inciden...
The classification of undifferentiated pleomorphic sarcoma (UPS) has been evolving with advances in immunohistochemistry and genomic profiling over the past 20 years. There is a lack of current information on UPS incidence. Due to the lack of designated histology codes for UPS in the Surveillance, Epidemiology, and End Results (SEERs) program, we estimated UPS incidence by three different definitions based on clinical opinions using the 2000-2020 data from 22 registries of the SEER program. The incidence varied widely across the three definitions with 0.06 per 100,000 persons for the least inclusive definition and 0.67 per 100,000 persons for the most inclusive definition in 2016-2020, making it challenging to estimate the exact incidence of UPS. Regardless, all the incidences decreased between 2000 and 2020. Guidelines in UPS diagnosis and classification need to be better implemented in the US.
Desmoplastic Small Round Cell Tumors: Clinical Presentation, Molecular Characterization, and Therapeutic Approach of Seven Patients.
Gaidzik VI, Mayer-Steinacker R, Wittau M … +15 more , Schultheiß M, V Baer A, Oehl-Huber K, Dahlum S, Fischer A, Gerstenmaier U, Seufferlein T, Buck A, Beer A, Thaiss W, Möller P, Döhner H, Siebert R, Marienfeld R, Barth TFE
Sarcoma · 2024 · PMID 39411551 · Full text
Desmoplastic small round blue cell tumor (DSRCT) is a highly aggressive fatal sarcoma without evidence-based therapeutic guidelines. We present here seven patients with DSRCT including immunohistochemistry combined with...
Desmoplastic small round blue cell tumor (DSRCT) is a highly aggressive fatal sarcoma without evidence-based therapeutic guidelines. We present here seven patients with DSRCT including immunohistochemistry combined with fluorescence in situ hybridization (FISH), next generation sequencing (NGS, = 6) as well as OncoScan array ( = 3) analyses and show consecutive therapeutic approaches. All seven DSRCT patients presented with an extended abdominal mass; median age at diagnosis was 24.8 years. NGS analyses revealed five class 4 or 5 sequence variants. Remarkably, OncoScan and targeted analyses by FISH identified genomic gains of in two cases. Cyclin D1 expression was present in all seven tumors as shown by immunohistochemical staining. Multimodal therapeutic concepts included systemic therapies, resection, and radiation. Six patients were treated as first-line therapy with conventional chemotherapy. All except one patient had a dismal therapy response. Subsequent therapy lines consisted of chemotherapeutic combinations followed by targeted therapies. Due to Cyclin D1 expression, the CDK4/6 inhibitor palbociclib was applied to four patients. The median therapy duration until disease progression in these patients was 4.5 months (range, 1.5-5 months). So, genomic gain and Cyclin D1 expression are common features pointing to cell-cycle deregulation as a possible therapeutic target.
ATRX and Its Prognostic Significance in Soft Tissue Sarcoma.
Cullen MM, Floyd W, Dow B … +6 more , Schleupner B, Brigman BE, Visgauss JD, Cardona DM, Somarelli JA, Eward WC
Sarcoma · 2024 · PMID 38741704 · Full text
PURPOSE: Recently, the association between ATRX and a more aggressive sarcoma phenotype has been shown. We performed a retrospective study of sarcomas from an individual institution to evaluate ATRX as a prognosticator i...
PURPOSE: Recently, the association between ATRX and a more aggressive sarcoma phenotype has been shown. We performed a retrospective study of sarcomas from an individual institution to evaluate ATRX as a prognosticator in soft tissue sarcoma. . 128 sarcomas were collected from a single institution and stained for ATRX. The prognostic significance of these markers was evaluated in a smaller cohort of primary soft tissue sarcomas ( = 68). Kaplan-Meier curves were created for univariate analysis, and Cox regression was utilized for multivariate analysis. RESULTS: High expression of ATRX was found to be a positive prognostic indicator for overall survival and metastasis-free survival in our group of soft tissue sarcomas both in univariate analysis and multivariate analysis (HR: 0.38 (0.17-0.85), =0.02 and HR: 0.49 (0.24-0.99), =0.05, respectively). CONCLUSIONS: High expression of ATRX is a positive prognostic indicator of overall survival and metastasis-free survival in patients with STS. This is consistent with studies in osteosarcoma, which indicate possible mechanisms through which loss of ATRX leads to more aggressive phenotypes. Future prospective clinical studies are required to validate the prognostic significance of these findings.
Factors Influencing the Outcome of Patients with Primary Ewing Sarcoma of the Sacrum.
Rechl V, Ranft A, Bhadri V … +22 more , Brichard B, Collaud S, Cyprova S, Eich H, Ek T, Gelderblom H, Hardes J, Haveman LM, Hartmann W, Hauser P, Heesen P, Jürgens H, Kanerva J, Kühne T, Raciborska A, Rascon J, Streitbürger A, Uhlenbruch Y, Timmermann B, Kersting J, Pham MT, Dirksen U
Sarcoma · 2024 · PMID 38524902 · Full text
BACKGROUND: Ewing sarcoma (EwS) is a rare and highly malignant bone tumor primarily affecting children, adolescents, and young adults. The pelvis, trunk, and lower extremities are the most common sites, while EwS of the...
BACKGROUND: Ewing sarcoma (EwS) is a rare and highly malignant bone tumor primarily affecting children, adolescents, and young adults. The pelvis, trunk, and lower extremities are the most common sites, while EwS of the sacrum as a primary site is very rare, and only few studies focusing on this location are published. Due to the anatomical condition, local treatment is challenging in sacral malignancies. We analyzed factors that might influence the outcome of patients suffering from sacral EwS. METHODS: We retrospectively analyzed data of the GPOH EURO-E.W.I.N.G 99 trial and the EWING 2008 trial, with a cohort of 124 patients with localized or metastatic sacral EwS. The study endpoints were overall survival (OS) and event-free survival (EFS). OS and EFS were calculated using the Kaplan-Meier method, and univariate comparisons were estimated using the log-rank test. Hazard ratios (HRs) with respective 95% confidence intervals (CIs) were estimated in a multivariable Cox regression model. RESULTS: The presence of metastases (3y-EFS: 0.33 vs. 0.68; < 0.001; HR = 3.4, 95% CI 1.7 to 6.6; 3y-OS: 0.48 vs. 0.85; < 0.001; HR = 4.23, 95% CI 1.8 to 9.7), large tumor volume (≥200 ml) (3y-EFS: 0.36 vs. 0.69; =0.02; HR = 2.1, 95% CI 1.1 to 4.0; 3y-OS: 0.42 vs. 0.73; =0.04; HR = 2.1, 95% CI 1.03 to 4.5), and age ≥18 years (3y-EFS: 0.41 vs. 0.60; =0.02; HR = 2.6, 95% CI 1.3 to 5.2; 3y-OS: 0.294 vs. 0.59; =0.01; HR = 2.92, 95% CI 1.29 to 6.6) were revealed as adverse prognostic factors. CONCLUSION: Young age seems to positively influence patients` survival, especially in patients with primary metastatic disease. In this context, our results support other studies, stating that older age has a negative impact on survival. Tumor volume, metastases, and the type of local therapy modality have an impact on the outcome of sacral EwS. Level of evidence: Level 2. This trial is registered with NCT00020566 and NCT00987636.
Exploring the Diagnostic Dilemma of Indeterminate Pulmonary Nodules in Patients with Primary Sarcoma of Bone.
Westlake B, Brown J, Hart J … +3 more , Skiby C, Jones K, Groundland J
Sarcoma · 2024 · PMID 38476458 · Full text
INTRODUCTION: Bone sarcomas are known to have a predilection for pulmonary metastasis. Surveillance protocols are thus focused on periodic chest imaging, typically with CT scan. Pulmonary nodules can be easily identified...
INTRODUCTION: Bone sarcomas are known to have a predilection for pulmonary metastasis. Surveillance protocols are thus focused on periodic chest imaging, typically with CT scan. Pulmonary nodules can be easily identified with this modality, but smaller nodules are not readily biopsied and may not represent metastatic disease. These are called indeterminate. The natural history of indeterminate nodules in a bone sarcoma population and factors associated with progression to true metastatic disease are not clearly defined. METHODS: All bone sarcoma patients treated at a single institution from 2010 to 2020 were eligible for inclusion. We treated 327 patients over this period; 119 were excluded for age less than 16 years, 31 were excluded for evident metastatic disease at presentation, and 60 were excluded for incomplete clinical follow-up or CT chest imaging either at staging or in surveillance. We assessed chest CT images for presence of pulmonary nodules and selected variables both at the staging and on surveillance images. Nodules were considered metastatic if proven histologically with a biopsy or by clinical interpretation by the multidisciplinary sarcoma team. Clinical and imaging factors were assessed for the association of indeterminate nodule progression to true metastatic disease. RESULTS: Seventy three of the 117 patients had indeterminate nodules on their staging CT scan; 41.1% of those patients progressed to metastatic disease compared to 43.2% of the patients that did not have indeterminate nodules on staging CT. Fifty eight of the 117 patients developed indeterminate nodules on surveillance chest CT, and 55.2% of those patients progressed to metastatic disease. There were no clinical or imaging factors that predicted the development of metastatic disease in the group that had indeterminate nodules at presentation; however, the number and size of nodules did correlate with progression to metastasis in those that developed indeterminate nodules on surveillance. CONCLUSION: Indeterminate pulmonary nodules are common on staging CT scans in patients with a bone sarcoma. The presence or absence of these indeterminate nodules was not predictive of progression to true metastatic disease in this cohort. However, the development of indeterminate nodules on surveillance imaging was associated with progression to metastatic disease with the size and number of nodules being important factors.
Progressive Improvement in 5-Year Survival Rates for Extremity Soft Tissue Sarcomas from 1999 to 2019.
Zastrow RK, El Sayed M, LiBrizzi CL … +2 more , Jacobs AJ, Levin AS
Sarcoma · 2024 · PMID 38410124 · Full text
BACKGROUND: Extremity soft-tissue sarcoma (ESTS) is a group of rare, heterogeneous malignancies. Previous studies have demonstrated a progressive improvement in 5-year survival rate over time, but recent trends are unkno...
BACKGROUND: Extremity soft-tissue sarcoma (ESTS) is a group of rare, heterogeneous malignancies. Previous studies have demonstrated a progressive improvement in 5-year survival rate over time, but recent trends are unknown. Therefore, this study aimed to provide an update on the clinical characteristics and 5-year survival rate of ESTS from 1999 to 2019. METHODS: This retrospective cohort study used the Surveillance, Epidemiology, and End Results (SEER) database. Overall, 5,654 patients over the age of 15 years with primary ESTS diagnosed between 1999 and 2019 were included. Data on patient demographics, clinical characteristics, and survival were extracted. Patients were grouped by year of diagnosis: 1999-2005, 2006-2012, and 2013-2019. Kaplan-Meier and Cox proportional hazards regression analyses were performed. RESULTS: ESTS occurred primarily in the lower extremity (76.1%) and was frequently grade III (58.3%), >5 cm in size (69.9%), and without metastasis (77.9%) at diagnosis. Furthermore, there was a significant increase in the proportion of patients over age 60 ( < 0.001) and without metastasis ( < 0.001) over the study period. The 5-year survival rate successively improved, from 47% in 1999-2005, to 61% in 2006-2012, to 78% in 2013-2019. Similarly, in multivariate analysis, the mortality rate progressively declined from a hazard ratio (HR) of 3.4 in 1999-2005 to an HR of 2.1 in 2006-2012, with the 2013-2019 group having the best overall survival ( < 0.001). Age, tumor size, grade, and metastasis were negative prognostic factors for survival; radiation and surgery were positive prognostic factors. CONCLUSIONS: The 5-year overall survival rate for ESTS progressively improved over the 20-year study period, perhaps due to an increasing proportion of older patients diagnosed with local disease. These findings may also be related to earlier detection or more effective treatment over the study period.
Meningeal Solitary Fibrous Tumor: A Single-Center Retrospective Cohort Study.
Roohani S, Alberti Y, Mirwald M … +8 more , Ehret F, Stromberger C, Roohani SF, Bender K, Flörcken A, Märdian S, Zips D, Kaul D
Sarcoma · 2024 · PMID 38274845 · Full text
BACKGROUND: Meningeal solitary fibrous tumors (SFTs) are rare, malignant, mesenchymal tumors of the central nervous system. While surgical gross total resection is widely accepted as a positive prognostic factor for loca...
BACKGROUND: Meningeal solitary fibrous tumors (SFTs) are rare, malignant, mesenchymal tumors of the central nervous system. While surgical gross total resection is widely accepted as a positive prognostic factor for local control (LC), the role of postoperative radiotherapy (PORT) remains controversial. We sought to report our institutional experience with a particular focus on outcomes after PORT. MATERIALS AND METHODS: In this single-center, retrospective cohort study, 20 patients with the primary diagnosis of histopathologically confirmed meningeal SFT were analyzed. Data on patient characteristics, imaging, treatment modalities, histopathology, and oncological outcomes were collected. LC and overall survival (OS) were assessed using the Kaplan-Meier estimator. RESULTS: The median follow-up time was 95.8 months. After surgery only, 9 out of 11 patients (81.8%) developed a local recurrence while, after surgery and PORT, 3 out of 9 patients (33.33%) showed local failure. The 5- and 10-year LC rates were 50.5% and 40.4% in the surgery-only group and 80% at both time points in the surgery with the PORT group. In the surgery-only group, 4 out of 11 patients (36.4%) died, and 4 out of 9 patients (44.4%) died in the surgery and PORT group. OS rates after 5 and 10 years were 88.9% and 66.7% in the surgery-only group and 88.9% and 76.2% in the surgery with PORT group. CONCLUSIONS: Our findings suggest that PORT may improve LC in patients with meningeal SFT. The low incidence of meningeal SFT impedes prospective studies and requires further international collaborative efforts to exploit retrospective datasets and molecular analysis to improve patient outcomes.
Genomic Breakpoint Characterization and Transcriptome Analysis of Metastatic, Recurrent Desmoplastic Small Round Cell Tumor.
Magrath JW, Flinchum DA, Hartono AB … +5 more , Goldberg IN, Espinosa-Cotton M, Moroz K, Cheung NV, Lee SB
Sarcoma · 2023 · PMID 37457440 · Full text
Desmoplastic small round cell tumor (DSRCT) is a rare pediatric cancer caused by the fusion oncogene. Despite initial response to chemotherapy, DSRCT has a recurrence rate of over 80% leading to poor patient prognosis w...
Desmoplastic small round cell tumor (DSRCT) is a rare pediatric cancer caused by the fusion oncogene. Despite initial response to chemotherapy, DSRCT has a recurrence rate of over 80% leading to poor patient prognosis with a 5-year survival rate of only 15-25%. Owing to the rarity of DSRCT, sample scarcity is a barrier in understanding DSRCT biology and developing effective therapies. Utilizing a novel pair of primary and recurrent DSRCTs, we present the first map of DSRCT genomic breakpoints and the first comparison of gene expression alterations between primary and recurrent DSRCT. Our genomic breakpoint map includes the lone previously published DSRCT genomic breakpoint, the breakpoint from our novel primary/recurrent DSRCT pair, as well as the breakpoints of five available DSRCT cell lines and five additional DSRCTs. All mapped breakpoints were unique and most breakpoints included a 1-3 base pair microhomology suggesting microhomology-mediated end-joining as the mechanism of translocation fusion and providing novel insights into the etiology of DSRCT. Through RNA-sequencing analysis, we identified altered genes and pathways between primary and recurrent DSRCTs. Upregulated pathways in the recurrent tumor included several DNA repair and mRNA splicing-related pathways, while downregulated pathways included immune system function and focal adhesion. We further found higher expression of the upregulated gene set in the recurrent tumor as compared to the primary tumor and lower expression of the downregulated gene set, suggesting the fusion continues to play a prominent role in recurrent tumors. The identified pathways including upregulation of DNA repair and downregulation of immune system function may help explain DSRCT's high rate of recurrence and can be utilized to improve the understanding of DSRCT biology and identify novel therapies to both help prevent recurrence and treat recurrent tumors.
Fibroblast Activation Protein Expression in Sarcomas.
Crane JN, Graham DS, Mona CE … +12 more , Nelson SD, Samiei A, Dawson DW, Dry SM, Masri MG, Crompton JG, Benz MR, Czernin J, Eilber FC, Graeber TG, Calais J, Federman NC
Sarcoma · 2023 · PMID 37333052 · Full text
OBJECTIVES: Fibroblast activation protein alpha (FAP) is highly expressed by cancer-associated fibroblasts in multiple epithelial cancers. The aim of this study was to characterize FAP expression in sarcomas to explore i...
OBJECTIVES: Fibroblast activation protein alpha (FAP) is highly expressed by cancer-associated fibroblasts in multiple epithelial cancers. The aim of this study was to characterize FAP expression in sarcomas to explore its potential utility as a diagnostic and therapeutic target and prognostic biomarker in sarcomas. METHODS: Available tissue samples from patients with bone or soft tissue tumors were identified at the University of California, Los Angeles. FAP expression was evaluated via immunohistochemistry (IHC) in tumor samples ( = 63), adjacent normal tissues ( = 30), and positive controls ( = 2) using semiquantitative systems for intensity (0 = negative; 1 = weak; 2 = moderate; and 3 = strong) and density (none, <25%, 25-75%; >75%) in stromal and tumor/nonstromal cells and using a qualitative overall score (not detected, low, medium, and high). Additionally, RNA sequencing data in publicly available databases were utilized to compare FAP expression in samples ( = 10,626) from various cancer types and evaluate the association between FAP expression and overall survival (OS) in sarcoma ( = 168). RESULTS: The majority of tumor samples had FAP IHC intensity scores ≥2 and density scores ≥25% for stromal cells (77.7%) and tumor cells (50.7%). All desmoid fibromatosis, myxofibrosarcoma, solitary fibrous tumor, and undifferentiated pleomorphic sarcoma samples had medium or high FAP overall scores. Sarcomas were among cancer types with the highest mean FAP expression by RNA sequencing. There was no significant difference in OS in patients with sarcoma with low versus high FAP expression. CONCLUSION: The majority of the sarcoma samples showed FAP expression by both stromal and tumor/nonstromal cells. Further investigation of FAP as a potential diagnostic and therapeutic target in sarcomas is warranted.
Accuracy of Time to Treatment Initiation Data in Musculoskeletal Soft Tissue Sarcoma.
Lawrenz JM, Vega JF, George J … +9 more , Curtis GL, Gordon J, Maggiotto A, Tullio K, Shepard DR, Reith JD, Schwartz HS, Nystrom LM, Mesko NW
Sarcoma · 2023 · PMID 37261268 · Full text
BACKGROUND: Time to treatment initiation (TTI) is a quality metric in cancer care. The purpose of this study is to determine the accuracy of TTI data from a single cancer center registry that reports to the National Canc...
BACKGROUND: Time to treatment initiation (TTI) is a quality metric in cancer care. The purpose of this study is to determine the accuracy of TTI data from a single cancer center registry that reports to the National Cancer Database (NCDB) for sarcoma diagnoses. METHODS: A retrospective analysis of a single Commission on Cancer (CoC)-accredited cancer center's tumor registry between 2006 and 2016 identified 402 patients who underwent treatment of a musculoskeletal soft tissue sarcoma and had TTI data available. Registry-reported TTI was extracted from the tumor registry. Effective TTI was manually calculated by medical record review as the number of days from the date of tissue diagnosis to initiation of first effective treatment. Effective treatment was defined as oncologic surgical excision or initiation of radiation therapy or chemotherapy. Registry-reported TTI and effective TTI values were compared for concordance in all patients. RESULTS: In the entire cohort, 25% (99/402) of patients had TTI data discordance, all related to surgical treatment definition. For patients with a registry-reported value of TTI = 0 days, 74% (87/118) had a diagnostic surgical procedure coded as their first treatment event, with 73 unplanned incomplete excision procedures and 14 incisional biopsies. In these patients, effective TTI was on average 59 days ( < 0.001). For patients with a registry-reported value of TTI >0 days, only 4% (12/284) had discordant TTI values. CONCLUSIONS: Nearly three-fourths of patients with a registry-reported value of TTI = 0 days in a large, CoC-accredited cancer center registry had a diagnostic procedure coded as their first treatment event, though their effective treatment had not yet started. These data suggest that TTI is likely longer than what is reported to the NCDB. Redefinition of what constitutes surgical treatment should be considered to improve the accuracy of data used in measuring TTI in sarcoma.
Postoperative Complication and Reoperation Rates Following Resection of Soft Tissue vs. Bone Malignancies Based on Anatomic Location in the Inpatient Setting.
Ballatori AM, Shahrestani S, Ton A … +6 more , Chen XT, Yamout T, Gettleman BS, Heckmann ND, Menendez LR, Christ AB
Sarcoma · 2023 · PMID 36937506 · Full text
INTRODUCTION: Surgical excisions of upper and lower extremity malignancies are increasing annually, due in part to the rising incidence of sarcomas. The purpose of this study is to compare readmissions, reoperation rate,...
INTRODUCTION: Surgical excisions of upper and lower extremity malignancies are increasing annually, due in part to the rising incidence of sarcomas. The purpose of this study is to compare readmissions, reoperation rate, and complications following surgical excision of soft/connective tissue vs bone malignancies of the upper and lower extremities. METHODS: The Nationwide Readmissions Database (NRD) was queried from 2016-2017 to conduct a retrospective analysis of 16,435 patients diagnosed with malignant neoplasms of the long bone (ULLB, = 1,433) and soft tissue (ULST, = 2,049) of the upper limb and malignant neoplasms of the long bone (LLLB, = 5,422) and soft tissue (LLST, = 7,531) of the lower limb. Patients who underwent surgical excision of their neoplasms were included. Binomial multivariate logistic regression was used to compare complications, nonelective readmission rates, and reoperation rates between the two groups at 30 and 90 days. RESULTS: Average age of the ULST group was 61.88, with 36% female. Average age of the ULLB group was 44.97, with 41.90% female. Average age of the LLST group was 60.96, with 46.90% female. Average age of the LLLB group was 43.09, with 42.60% female. The ULST group had lower odds of readmission within 30 days (=0.263), which became significant within 90 days of surgery (=0.045). The LLST group had significantly higher odds of infection, reoperation within 30 to 90 days of the index surgery compared to the LLLB group ( < 0.0001). The LLST group had significantly lower odds of readmission within 30 (=0.04) and 90 days (=0.015) of the index surgery. CONCLUSION: Patients in the ULST group had significantly lower odds of 90-day readmission compared to the ULLB group. There were also significantly lower odds of 30- and 90-day readmission in the LLST group compared to the LLLB group. However, the LLST group had significantly higher odds of infection and reoperation within 30 and 90 days compared to the LLLB group.
Soft-Tissue Sarcomas in Adults in Ecuador in the Period 2010-2020.
Sánchez X, Moreno Roca A, Jimbo-Sotomayor R … +2 more , Armijos Acurio L, Viloria Cestari A
Sarcoma · 2022 · PMID 36600805 · Full text
BACKGROUND: Soft-tissue sarcomas (STSs) are rare tumors; they represent 1% of all tumors in adults. There are new diagnostic techniques to differentiate tumor types, and surgery continues to be the most important treatme...
BACKGROUND: Soft-tissue sarcomas (STSs) are rare tumors; they represent 1% of all tumors in adults. There are new diagnostic techniques to differentiate tumor types, and surgery continues to be the most important treatment for STS. METHODS: This cross-sectional study analyzed the morbidity and mortality caused by STS in adults between 2010 and 2020 using national databases. RESULTS: A total of 8,393 patients hospitalized due to STS were reported. The total number of deaths in Ecuador due to STS was 7,088 over the last decade, the provinces of Pichincha and Guayas registered the highest number of cases, and the mortality rate was 1.2 to 2.0 per 100,000 people. It is noteworthy that the lowest point of registered cases was in 2012. CONCLUSION: Soft-tissue sarcomas are rare tumors in Ecuador. The morbidity and mortality caused by these tumors have not changed in the last decade. National studies are needed to determine the prevalence of this illness and study intervention to lower mortality.
Clinical Outcomes of Soft Tissue Sarcoma around the Elbow Joint: A Retrospective Single Institution Study.
Azurin ES, Yamamoto N, Hayashi K … +10 more , Takeuchi A, Tada K, Miwa S, Igarashi K, Higuchi T, Yonezawa H, Morinaga S, Asano Y, Saito S, Tsuchiya H
Sarcoma · 2022 · PMID 36573098 · Full text
BACKGROUND: We report a retrospective case series analysis of clinical outcomes of patients with soft tissue sarcoma around the elbow. METHODS: Twenty-two patients underwent surgical tumor excision between January 1999 a...
BACKGROUND: We report a retrospective case series analysis of clinical outcomes of patients with soft tissue sarcoma around the elbow. METHODS: Twenty-two patients underwent surgical tumor excision between January 1999 and May 2017, with a mean follow-up of 85.2 months. RESULTS: Six tumors were localized in the upper arm, nine in the elbow, and seven in the forearm. Sixteen tumors were deep-seated, and six were superficially located. Fifteen patients underwent wide excision, including one amputation, and 18 achieved (81.8%) negative margins histologically. Two local recurrences and four distant metastases developed. The mean Musculoskeletal Tumor Society score was 92.0% (range, 33.3-100). The 5-year local recurrence-free survival rate, metastasis-free survival rate, and overall survival rate were 90.0%, 77.0%, and 79.7%, respectively. CONCLUSIONS: Local control and limb function can have favorable outcomes when the tumor excised has a histologically negative margin without sacrificing the major structure.
Analysis of Negative Reviews of Orthopedic Oncology Surgeons: An Investigation of Reviews from Healthgrades, Vitals, and Google.
Qubain L, Richman EH, Eaton V … +2 more , Brinkman JC, Goulding KM
Sarcoma · 2022 · PMID 36536691 · Full text
BACKGROUND: Physician review websites (PRWs) are increasing in usage and popularity. Our purpose is to characterize one-star reviews of orthopedic oncology surgeons to understand factors in healthcare that contribute to...
BACKGROUND: Physician review websites (PRWs) are increasing in usage and popularity. Our purpose is to characterize one-star reviews of orthopedic oncology surgeons to understand factors in healthcare that contribute to patient satisfaction. METHODS: Orthopedic oncology surgeons were randomly selected from the Musculoskeletal Tumor Society. A search for one-star reviews was performed on Google Reviews, Healthgrades, and Vitals.com. Reviews were classified as clinical or nonclinical. Statistical analyses were performed regarding the frequency of reviews and complaints for each category. RESULTS: Of the 7,733 reviews discovered, 908 (11.7%) were identified as one-star reviews. Of 907 usable complaints, 362 (40.8%) were clinical and 545 (59.2%) were nonclinical. The most common nonclinical complaints included bedside manner (65%) and limited time with providers (19%). The most common clinical complaints included complications (26%) and disagreements with the treatment plan (26%). There were 120 surgical and 221 nonsurgical reviews. Surgical patients had a higher rate of clinical complaints. Nonsurgical patients had a higher rate of total complaints. CONCLUSION: To the best of our knowledge, this is the first study examining PRWs regarding orthopedic oncology surgeons. Most one-star reviews were due to nonclinical complaints from nonsurgical patients. The most common factors are bedside manner, limited time with provider, phone communication issues, and rude/unprofessional conduct.
Patient-Specific Instruments for Forearm Sarcoma Resection and Allograft Reconstruction in Children: Results in 4 Cases.
Paulmier A, Raad M, Verhelle CE … +2 more , Paul L, Docquier PL
Sarcoma · 2022 · PMID 36353598 · Full text
For pediatric malignant bone tumors located in the limbs, limb salvage surgery is the gold standard, but it requires adequate resection margins to avoid local recurrence. Primitive bone sarcomas of the forearm (radius or...
For pediatric malignant bone tumors located in the limbs, limb salvage surgery is the gold standard, but it requires adequate resection margins to avoid local recurrence. Primitive bone sarcomas of the forearm (radius or ulna) are very rare and the reconstruction remains challenging. We describe a method to ensure minimal but adequate resection bone margins with precision in four consecutive patients with primitive bone sarcomas of the forearm. During the preoperative planning, magnetic resonance imaging (MRI) was used to delineate the tumor and the tumor volume was transferred to computerized tomography (CT) by image fusion. A patient-specific instrument (PSI) was manufactured by 3D printing to allow the surgeon to perform the surgical cuts precisely according to the preoperative planning. The first PSI was used for the resection of the tumor, which adopted a unique position at the bony surface. A second PSI was intended for the cutting of the bone allograft so that it fitted perfectly with the bone defect. In all four cases, the safe margin obtained into the bone was free of tumor (R0: microscopically margin-negative resection). The functional result was very good in all four patients. This limb salvage surgical technique can be applied in forearm bone sarcoma and improves surgical precision while maintaining satisfactory local tumor control. It can also reduce the surgical time and allow a stable osteosynthesis.
Risk of Malignant Transformation of Giant Cell Tumors of Bone Is 8 Times Lower with Megavoltage vs. Orthovoltage Radiation Therapy.
Musharbash FN, Edelstein A, El Abiad JM … +3 more , Levin AS, Alcorn SR, Morris CD
Sarcoma · 2022 · PMID 36311815 · Full text
BACKGROUND: The first-line treatment for most giant cell tumors (GCTs) of bone is surgical; radiotherapy (RT) is reserved for inoperable or refractory cases. While RT techniques have undergone a dramatic change over the...
BACKGROUND: The first-line treatment for most giant cell tumors (GCTs) of bone is surgical; radiotherapy (RT) is reserved for inoperable or refractory cases. While RT techniques have undergone a dramatic change over the past few decades, with the higher energy megavoltage RT replacing orthovoltage RT, concerns for high rates of malignant transformation following RT have limited its use. Evidence suggests a lower incidence of secondary malignancy after treatment with megavoltage compared with orthovoltage RT, but this has not been studied in GCTs. Our main purpose was to compare the incidence of malignant transformation of GCTB between patients treated with orthovoltage vs. megavoltage RT. METHODS: A literature review was performed to identify studies reporting GCTBs treated with RT from 01/1900 through 12/2019. Studies that did not report RT modality or separate orthovoltage and megavoltage results were excluded. Included in the analysis were 6 patients from our institution. Primary outcome was the incidence of malignant transformation; secondary outcomes were time to transformation and incidence of local recurrence. Fisher's exact tests and independent sample -tests were used, and significance was set at < 0.05. RESULTS: Twenty-two studies were included, which reported on 168 GCTBs treated with orthovoltage and 393 treated with megavoltage RT. Transformation incidence was 14% ( = 24) for orthovoltage and 1.8% ( = 7) for megavoltage RT, an 8-fold difference (odds ratio (OR) 9.1, 95% confidence interval (CI) 3.9-22, < 0.001). Mean time to transformation was 8.7 years for orthovoltage and 11.2 years for megavoltage RT (=0.28). Incidence of local recurrence was 38% (63/167) for orthovoltage and 17% (66/393) for megavoltage RT (OR 3.3, 95% CI 2.0-4.6, < 0.001). CONCLUSIONS: The risk of developing a malignancy after RT of GCTB is 8 times lower with megavoltage than with orthovoltage. Malignant transformation with megavoltage, while not zero, is lower than that in historical series. Use of modern RT techniques in inoperable or refractory GCTB may be appropriate.
Adjuvant Radiation after Primary Resection of Atypical Lipomatous Tumors of the Extremity Reduces Local Recurrence but Increases Complications: A Multicenter Evaluation.
Lawrenz JM, Johnson SR, Zhu K … +15 more , McKeon M, Moran CP, Vega J, Hajdu KS, Norris JP, Luo LY, Shinohara ET, Cates JMM, Rubin BP, Reith JD, Halpern JL, Mesko NW, Schwartz HS, Nystrom LM, Holt GE
Sarcoma · 2022 · PMID 36046749 · Full text
BACKGROUND: Radiation after resection of an atypical lipomatous tumor (ALT) is controversial. This study evaluates local control and complications after the first resection of ALTs of the extremity with or without adjuva...
BACKGROUND: Radiation after resection of an atypical lipomatous tumor (ALT) is controversial. This study evaluates local control and complications after the first resection of ALTs of the extremity with or without adjuvant radiation. METHODS: A dual institution, retrospective review of patients treated from 1995 to 2020 with first-time resection of an ALT in the extremity was performed. In total, 102 patients underwent adjuvant radiation (XRT group) and 68 patients were treated with surgery alone (no-XRT group). The median follow-up time was 4.6 years (interquartile range (IQR) 2.0-7.3 years). The median radiation dose was 60 Gy (IQR 55-66 Gy). Univariable and multivariable analyses evaluated the association of patient, tumor, and treatment variables with recurrence and complications. Kaplan-Meier analysis evaluated local recurrence-free survival (LRFS) and time to complication. RESULTS: The overall incidence of local recurrence was 1% (1/102) in the XRT group and 24% (16/68) in the no-XRT group ( < 0.001). The median time-to-recurrence was 8.2 years (IQR 6.5-10.5 years). In the XRT and the no-XRT groups, 5-yr LRFS was 98% and 92% (=0.21) and 10-yr LRFS was 98% and 41% ( < 0.001), respectively. The absence of radiation (HR = 23.63, 95% CI (3.09-180.48); < 0.001) and R2 surgical resection margins (HR = 11.04, 95% CI (2.07-59.03); < 0.001) incurred a 23-fold and 11-fold increased risk of local recurrence, respectively, while tumor size, depth, location, and neurovascular involvement were not found to be independent predictors of recurrence. The complication rate was 37% (38/102) in the XRT group and 10% (7/68) in the no-XRT group ( < 0.001). Eight patients (8/102, 8%) required surgical management for complication in the XRT group compared with two patients (2/68, 3%) in the no-XRT group (=0.10). Higher radiation dose had a modest correlation with increased severity of complication (=0.24; =0.02). CONCLUSIONS: Adjuvant radiation after first-time resection of an ALT of the extremity was associated with a significantly reduced risk of local recurrence but a three-fold increase in complication rate. These data support a 10-year follow-up for these patients and inform a notable clinical trade-off if considering adjuvant radiation for this tumor with recurrent potential.
Malignant Transformation of Giant Cell Tumor of Bone and the Association with Denosumab Treatment: A Radiology and Pathology Perspective.
van Langevelde K, Cleven AHG, Navas Cañete A … +4 more , van der Heijden L, van de Sande MAJ, Gelderblom H, Bovée JVMG
Sarcoma · 2022 · PMID 35814640 · Full text
OBJECTIVE: Malignancy in giant cell tumor of bone (mGCTB) is categorized as primary (concomitantly with conventional GCTB) or secondary (after radiotherapy or other treatment). Denosumab therapy has been suggested to pla...
OBJECTIVE: Malignancy in giant cell tumor of bone (mGCTB) is categorized as primary (concomitantly with conventional GCTB) or secondary (after radiotherapy or other treatment). Denosumab therapy has been suggested to play a role in the etiology of secondary mGCTB. In this case series from a tertiary referral sarcoma center, we aimed to find distinctive features for malignant transformation in GCTB on different imaging modalities. Furthermore, we assessed the duration of denosumab treatment and lag time to the development of malignancy. METHODS: From a histopathology database search, 6 patients were pathologically confirmed as having initial conventional GCTB and subsequently with secondary mGCTB. RESULTS: At the time of mGCTB diagnosis, 2 cases were treated with denosumab only, 2 with denosumab and surgery, 1 with multiple curettages and radiotherapy, and 1 with surgery only. In the 4 denosumab treated patients, the mean lag time to malignant transformation was 7 months (range 2-11 months). Imaging findings suspicious of malignant transformation related to denosumab therapy are the absence of fibro-osseous matrix formation and absent neocortex formation on CT, and stable or even increased size of the soft tissue component. CONCLUSION: In 4 patients treated with denosumab, secondary mGCTB occurred within the first year after initiation of treatment. Radiotherapy-associated mGCTB has a longer lag time than denosumab-associated mGCTB. Close clinical and imaging follow-up during the first months of denosumab therapy is key, as mGCTB tends to have rapid aggressive behavior, similar to other high-grade sarcomas. Nonresponders should be (re) evaluated for their primary diagnosis of conventional GCTB.
Oncological and Functional Outcomes after Hemicortical Resection and Biological Reconstruction Using Allograft for Parosteal Osteosarcoma of the Distal Femur.
Savvidou OD, Goumenos S, Trikoupis I … +5 more , Kaspiris A, Melissaridou D, Gavriil P, Georgoulis J, Papagelopoulos PJ
Sarcoma · 2022 · PMID 35692235 · Full text
BACKGROUND: Parosteal osteosarcoma (PAOS) is a surface osteosarcoma. Treatment options include wide excision and endoprosthetic or allograft. However, due to the low local recurrence and metastasis rate, when it appears...
BACKGROUND: Parosteal osteosarcoma (PAOS) is a surface osteosarcoma. Treatment options include wide excision and endoprosthetic or allograft. However, due to the low local recurrence and metastasis rate, when it appears in the posterior surface of the distal femur, the lesion can be managed with hemicortical wide resection and biological reconstruction with hemicortical allograft. The purpose of this study is to evaluate the oncological and functional outcomes of patients with parosteal osteosarcoma (PAOS) of the posterior cortex of the distal femur who underwent biological reconstruction after hemicortical resection. METHODS: Eleven patients who underwent wide tumor resection and defect reconstruction of the posterior surface of the distal femur using hemicortical allograft were retrospectively studied. Local recurrence, metastasis, complications, and the functional outcome using the Musculoskeletal Tumor Society (MSTS) scoring system were evaluated. RESULTS: The average postoperative follow-up period was 53.64 months (range, 30 to 84 months). At the latest follow-up, all patients had no evidence of disease without metastases. One patient with local recurrence underwent revision surgery with fibula autograft reconstruction. The mean MSTS score was 93.45 ± 3.56. CONCLUSIONS: Treatment of patients with PAOS of the posterior aspect of the distal femur with hemicortical resection and allograft reconstruction has satisfactory oncological and functional outcome and low complication rates.
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