Sarcoma[JOURNAL]
Sun
200 papers
Time Trends and Prognostic Factors for Overall Survival in Myxoid Liposarcomas: A Population-Based Study.
Lansu J, Van Houdt WJ, Schaapveld M … +4 more , Walraven I, Van de Sande MAJ, Ho VKY, Haas RL
Sarcoma · 2020 · PMID 33029074 · Full text
BACKGROUND: The purpose of this study was to evaluate the overall survival (OS) and associated characteristics for patients with Myxoid Liposarcoma (MLS) over time in The Netherlands. METHODS: A population-based study wa...
BACKGROUND: The purpose of this study was to evaluate the overall survival (OS) and associated characteristics for patients with Myxoid Liposarcoma (MLS) over time in The Netherlands. METHODS: A population-based study was performed of patients with primary localized ( = 851) and metastatic ( = 50) MLS diagnosed in The Netherlands between 1989 and 2016, based on data from the National Cancer Registry. RESULTS: The median age of the MLS patients was 49 years, and approximately two-thirds was located in the lower limb. An association was revealed between age and the risk of having a Round Cell (RC) tumor. OS rates for primary localized MLS were 93%, 83%, 78%, and 66% after 1, 3, 5, and 10 years, respectively. The median OS for patients with metastatic disease at diagnosis was 10 months. Increasing age (Hazard Ratio (HR) 1.05, =0.00), a tumor size >5 cm (HR 2.18; =0.00), and tumor location (trunk HR 1.29; =0.09, upper limb HR 0.83; =0.55, and "other" locations HR 2.73; =0.00, as compared to lower limb) were independent prognostic factors for OS. The percentage of patients treated with radiotherapy (RT) increased over time, and preoperative RT gradually replaced postoperative RT. In contrast to patients with localized disease, significant improvement of OS was observed in patients with metastatic disease over time. CONCLUSIONS: In this large nationwide cohort, tumor size and tumor location were independent prognostic factors for OS. Furthermore, a higher probability of an RC tumor with increasing age was suggested. An increased use of RT over the years did not translate into improved OS for localized MLS.
Pharmacologic Inhibition of Ezrin-Radixin-Moesin Phosphorylation is a Novel Therapeutic Strategy in Rhabdomyosarcoma.
Proudfit A, Bhunia N, Pore D … +3 more , Parker Y, Lindner D, Gupta N
Sarcoma · 2020 · PMID 33005093 · Full text
Intermediate and high-risk rhabdomyosarcoma (RMS) patients have poor prognosis with available treatment options, highlighting a clear unmet need for identification of novel therapeutic strategies. Ezrin-radixin-moesin (E...
Intermediate and high-risk rhabdomyosarcoma (RMS) patients have poor prognosis with available treatment options, highlighting a clear unmet need for identification of novel therapeutic strategies. Ezrin-radixin-moesin (ERM) family members are membrane-cytoskeleton linker proteins with well-defined roles in tumor metastasis, growth, and survival. ERM protein activity is regulated by dynamic changes in the phosphorylation at a conserved threonine residue in their C-terminal actin-binding domain. Interestingly, ERM family member, ezrin, has elevated expression in the RMS tissue. Despite this, the translational scope of targeting ERM family proteins in these tumors through pharmacological inhibition has never been considered. This study investigates the inhibition of ERM phosphorylation using a small molecule pharmacophore NSC668394 as a potential strategy against RMS. Upon in vitro treatment with NSC668394, RMS cells exhibit a dose-dependent decrease in cell viability and proliferation, with induction of caspase-3 cleavage and apoptosis. siRNA-mediated knockdown of individual ERM protein expression revealed that each regulates RMS survival to a different degree. In vivo administration of NSC668394 in RMS xenografts causes significant decrease in tumor growth, with no adverse effect on body weight. Collectively, this study highlights the importance of the active conformation of ERM proteins in RMS progression and survival and supports pharmacologic inhibition of these proteins as a novel therapeutic approach.
Angiosarcoma of the Scalp and Face: A Dosimetric Comparison of HDR Surface Applicator Brachytherapy and VMAT.
Mitra D, Pei Y, Buzurovic I … +5 more , Devlin PM, Thornton K, Raut CP, Baldini EH, Lam MB
Sarcoma · 2020 · PMID 32908445 · Full text
PURPOSE: Angiosarcoma of the face and scalp is a rare disease with high rates of recurrence. The optimal treatment approach is not well defined. This study presents a dosimetric comparison of high-dose-rate surface appli...
PURPOSE: Angiosarcoma of the face and scalp is a rare disease with high rates of recurrence. The optimal treatment approach is not well defined. This study presents a dosimetric comparison of high-dose-rate surface applicator (HDR-SA) brachytherapy to volumetric-modulated arc therapy (VMAT). METHODS: Between 2011 and 2018, 12 patients with primary or recurrent angiosarcoma of the face or scalp were treated with HDR-SA brachytherapy using CT-based planning at our institution. For comparison, deliverable VMAT plans for each patient were generated, and dose distribution was compared to the delivered HDR-SA brachytherapy plans. RESULTS: Both VMAT and HDR-SA brachytherapy plans delivered good coverage of the clinical target. However, the dose distribution of VMAT was significantly different from HDR-SA brachytherapy across a variety of parameters. Mean doses to the lacrimal gland, orbit, lens, and cochlea were significantly higher with HDR-SA brachytherapy vs. VMAT. Brain Dmax, V80%, and V50% were also significantly higher with HDR-SA brachytherapy. CONCLUSIONS: There may be dosimetric advantages to VMAT over HDR-SA brachytherapy for many patients. However, individual tumor location, patient anatomy, and treatment reproducibility may result in HDR-SA brachytherapy being the preferred technique in a subset of patients. Ultimately, a personalized approach is likely to be the optimal treatment plan.
Predicting Pathologic Bone Lesions Using Scout Computed Tomography (CT) Imaging.
Colello MJ, Pichiotino ER, Tanner SL … +2 more , Porter SE, Gurich RW
Sarcoma · 2020 · PMID 32848506 · Full text
The purpose of this study is to evaluate the benefit of reviewing scout CT images, obtained for routine oncologic surveillance, for the early identification of pathologic bony lesions. A retrospective review was conducte...
The purpose of this study is to evaluate the benefit of reviewing scout CT images, obtained for routine oncologic surveillance, for the early identification of pathologic bony lesions. A retrospective review was conducted on patients who previously underwent surgical treatment by two orthopedic oncology surgeons at a tertiary care institution from 2009-2019 for pathologic lesions or fractures of the humerus or femur. Radiographic records were reviewed to identify patients in this cohort who had available scout views from CT imaging prior to official diagnosis of the bony lesion or fracture. CT scout images were assessed by two independent reviewers to identify any pathologic lesions, and radiographic reports were reviewed to identify if the lesions were noted by radiology at the time of the initial scan interpretation. One hundred and forty-four patients were identified, and thirty-nine had an available scout CT image prior to official diagnosis of the lesion. Twenty-five patients (64.1%) had lesions identified by authors on scout CT versus only 9 (23.1%) who had lesions that were documented in the initial CT radiologic report. There was a total of 29 lesions identified by the study authors on scout CT, and 19 (65.5%) were not reported in the initial radiographic interpretation with an average interval between observation by authors and official diagnosis of 202 days. Of the impending fractures, three patients (16.7%) went on to complete fracture prior to referral to orthopedics with an average interval between these missed lesions on scout CT and their presentation with fracture of 68 days. This study advocates for the careful review of all scout CT imaging as an essential part of the work up for metastatic disease and encourages all practitioners to utilize this screening tool for the identification of pathologic bony lesions which may help expedite early treatment to reduce patient morbidity.
Assessing the Desmoid-Type Fibromatosis Patients' Voice: Comparison of Health-Related Quality of Life Experiences from Patients of Two Countries.
Timbergen MJM, van der Graaf WTA, Grünhagen DJ … +7 more , Younger E, Sleijfer S, Dunlop A, Dean L, Verhoef C, van de Poll-Franse LV, Husson O
Sarcoma · 2020 · PMID 32774131 · Full text
PURPOSE: Desmoid-type fibromatosis (DTF) is a rare, nonmetastasising soft tissue tumour. Symptoms, unpredictable growth, lack of definitive treatments, and the chronic character of the disease can significantly impact he...
PURPOSE: Desmoid-type fibromatosis (DTF) is a rare, nonmetastasising soft tissue tumour. Symptoms, unpredictable growth, lack of definitive treatments, and the chronic character of the disease can significantly impact health-related quality of life (HRQoL). We aimed at identifying the most important HRQoL issues according to DTF patients in two countries, in order to devise a specific HRQoL questionnaire for this patient group. METHODS: DTF patients and healthcare providers (HCPs) from the Netherlands and the United Kingdom individually ranked 124 issues regarding diagnosis, treatment, follow-up, recurrence, living with DTF, healthcare, and supportive care experiences, according to their relevance. Descriptive statistics were used to calculate priority scores. RESULTS: The most highly ranked issues by patients ( = 29) were issues concerning "tumour growth," "feeling that there is something in the body that does not belong there," and "fear of tumour growth into adjacent tissues or organs" with mean () scores of 3.0, 2.9, and 2.8, respectively (Likert scale 1-4). British patients scored higher on most issues compared to Dutch patients ( 2.2 vs. 1.5). HCPs ( = 31) gave higher scores on most issues compared to patients ( 2.3 vs. 1.8). CONCLUSION: This study identified the most relevant issues for DTF patients, which should be included in a DTF-specific HRQoL questionnaire. Additionally, we identified differences in priority scores between British and Dutch participating patients. Field testing in a large, international cohort is needed to confirm these findings and to devise a comprehensive and specific HRQoL questionnaire for DTF patients.
Immunohistochemical Analysis of CD99 and PAX8 in a Series of 15 Molecularly Confirmed Cases of Ewing Sarcoma.
Chinchilla-Tábora LM, Ortiz Rodríguez-Parets J, González Morais I … +2 more , Sayagués JM, Ludeña de la Cruz MD
Sarcoma · 2020 · PMID 32675940 · Full text
Ewing sarcomas are an uncommon group of malignant neoplasms. A multidisciplinary approach is highly recommended to reach a correct diagnosis, considering the clinical, radiological, and histopathological aspects. Since i...
Ewing sarcomas are an uncommon group of malignant neoplasms. A multidisciplinary approach is highly recommended to reach a correct diagnosis, considering the clinical, radiological, and histopathological aspects. Since in up to 90% of cases, the translocation t (11; 22) (q24; q12) occurs resulting in a chimeric fusion transcript EWSR1-FLI-1. The pathologist has several tools in addition to conventional techniques (hematoxylin and eosin), such as immunohistochemistry, which plays a very important role in the differential diagnosis. We present a series of 15 cases of molecularly confirmed ES, in which we found a sensitivity of 100% for CD99 and 80% for PAX8 by immunohistochemistry. This indicates a high sensitivity; however, it is known that both CD99 and PAX8 are also expressed in other tumours. Therefore, molecular confirmation should be performed in all cases.
Follow-Up in Bone Sarcoma Care: A Cross-Sectional European Study.
Goedhart LM, Leithner A, Ploegmakers JJW … +1 more , Jutte PC
Sarcoma · 2020 · PMID 32675939 · Full text
BACKGROUND: Follow-up of high-grade bone sarcoma patients with repeated radiological imaging aims at early detection of recurrent disease or distant metastasis. Repeated radiological imaging does expose (mostly young) pa...
BACKGROUND: Follow-up of high-grade bone sarcoma patients with repeated radiological imaging aims at early detection of recurrent disease or distant metastasis. Repeated radiological imaging does expose (mostly young) patients to ionising radiation. At this point, it is not known whether frequent follow-up increases overall survival. Additionally, frequent follow-up subjects patients and families to psychological stress. This study aims to assess follow-up procedures in terms of frequency and type of imaging modalities in bone tumour centres across Europe for comparison and improvement of knowledge as a first step towards a more uniform approach towards bone sarcoma follow-up. METHODS: Data were obtained through analysis of several follow-up protocols and a digital questionnaire returned by EMSOS members of bone tumour centres all across Europe. RESULTS: All participating bone tumour centres attained a minimum follow-up period of ten years. National guidelines revealed variations in follow-up intervals and use of repeated imaging with ionising radiation. A local and a chest X-ray were obtained at 47.6% of the responding clinics at every follow-up patient visit. CONCLUSIONS: Variations were seen among European bone sarcoma centres with regards to follow-up intervals and use of repeated imaging. The majority of these expert centres follow existing international guidelines and find them sufficient as basis for a follow-up surveillance programme despite lack of evidence. Future research should aim towards evidence-based follow-up with focus on the effects of follow-up strategies on health outcomes, cost-effectiveness, and individualised follow-up algorithms.
PET/CT Imaging as a Diagnostic Tool in Distinguishing Well-Differentiated versus Dedifferentiated Liposarcoma.
Parkes A, Urquiola E, Bhosale P … +16 more , Lin H, Watson K, Wang WL, Feig B, Torres K, Roland CL, Conley AP, Zarzour M, Livingston JA, Ratan R, Ludwig J, Araujo DM, Ravi V, Benjamin RS, Patel S, Somaiah N
Sarcoma · 2020 · PMID 32565716 · Full text
Distinguishing well-differentiated liposarcoma (WDLPS) from dedifferentiated liposarcoma (DDLPS) is essential given distinct treatment paradigms and chemosensitivity. Percutaneous biopsy has a low sensitivity for detecti...
Distinguishing well-differentiated liposarcoma (WDLPS) from dedifferentiated liposarcoma (DDLPS) is essential given distinct treatment paradigms and chemosensitivity. Percutaneous biopsy has a low sensitivity for detecting DDLPS. We sought to identify the diagnostic utility of positron emission tomography/computed tomography (PET/CT) in identifying WDLPS versus DDLPS. An independent radiologist reviewed PET/CT images to identify target lesions and determine the maximum standardized uptake value (SUVmax). An independent pathologist review confirmed WDLPS or DDLPS histology. A binary cutoff point of SUVmax was identified using a classification and regression trees (CART) algorithm. We identified 20 patients with WDLPS or DDLPS with 26 PET/CTs performed for separate recurrences that were followed by surgical sampling. Of the 26 records, 12 were DDLPS (46%) and 14 were WDLPS (54%). Patients with DDLPS had significantly higher SUVmax than those with WDLPS ( value = 0.0035). A SUVmax of 4 was identified as the cutoff point. Using this cutoff, the sensitivity of SUVmax identifying a case as DDLPS was 83.3% (95% CI: 51.6%, 97.9%) and the specificity was 85.7% (95% CI: 57.2%, 98.2%). PET/CT is a sensitive and specific diagnostic tool to identify the presence of dedifferentiation within the tumor.
Deep Functional and Molecular Characterization of a High-Risk Undifferentiated Pleomorphic Sarcoma.
Berlow NE, Grasso CS, Quist MJ … +10 more , Cheng M, Gandour-Edwards R, Hernandez BS, Michalek JE, Ryan C, Spellman P, Pal R, Million LS, Renneker M, Keller C
Sarcoma · 2020 · PMID 32565715 · Full text
Nonrhabdomyosarcoma soft-tissue sarcomas (STSs) are a class of 50+ cancers arising in muscle and soft tissues of children, adolescents, and adults. Rarity of each subtype often precludes subtype-specific preclinical rese...
Nonrhabdomyosarcoma soft-tissue sarcomas (STSs) are a class of 50+ cancers arising in muscle and soft tissues of children, adolescents, and adults. Rarity of each subtype often precludes subtype-specific preclinical research, leaving many STS patients with limited treatment options should frontline therapy be insufficient. When clinical options are exhausted, personalized therapy assignment approaches may help direct patient care. Here, we report the results of an adult female STS patient with relapsed undifferentiated pleomorphic sarcoma (UPS) who self-drove exploration of a wide array of personalized Clinical Laboratory Improvement Amendments (CLIAs) level and research-level diagnostics, including state of the art genomic, proteomic, live cell chemosensitivity testing, a patient-derived xenograft model, and immunoscoring. Her therapeutic choices were also diverse, including neoadjuvant chemotherapy, radiation therapy, and surgeries. Adjuvant and recurrence strategies included off-label and natural medicines, several immunotherapies, and N-of-1 approaches. Identified treatment options, especially those validated during the study, were not introduced into the course of clinical treatment but did provide plausible treatment regimens based on FDA-approved clinical agents.
Time to Treatment Initiation and Survival in Adult Localized High-Grade Bone Sarcoma.
Lawrenz JM, Featherall J, Curtis GL … +8 more , George J, Jin Y, Anderson PM, Shepard DR, Reith JD, Rubin BP, Nystrom LM, Mesko NW
Sarcoma · 2020 · PMID 32454786 · Full text
OBJECTIVE: Few studies have evaluated the prognostic implication of the length of time from diagnosis to treatment initiation in bone sarcoma. The purpose of this study is to determine if time to treatment initiation (TT...
OBJECTIVE: Few studies have evaluated the prognostic implication of the length of time from diagnosis to treatment initiation in bone sarcoma. The purpose of this study is to determine if time to treatment initiation (TTI) influences overall survival in adults diagnosed with primary bone sarcoma. METHODS: A retrospective analysis of the National Cancer Database identified 2,122 patients who met inclusion criteria with localized, high-grade bone sarcoma diagnosed between 2004 and 2012. TTI was defined as length of time in days from diagnosis to initiation of treatment. Patient, disease-specific, and healthcare-related factors were also assessed for their association with overall survival. Kruskal-Wallis analysis was utilized for univariate analysis, and Cox regression modeling identified covariates associated with overall survival. RESULTS: Any 10-day increase in TTI was not associated with decreased overall survival (hazard ratio (HR) = 1.00; =0.72). No differences in survival were detected at 1 year, 5 years, and 10 years, when comparing patients with TTI = 14, 30, 60, 90, and 150 days. Decreased survival was significantly associated ( < 0.05) with patient ages of 51-70 years (HR = 1.66; =0.004) and > 71 years (HR = 2.89; < 0.001), Charlson/Deyo score ≥2 (HR = 2.02; < 0.001), pelvic tumor site (HR = 1.58; < 0.001), tumor size >8 cm (HR = 1.52; < 0.001), radiation (HR = 1.81; < 0.001) as index treatment, and residing a distance of 51-100 miles from the treatment center (HR = 1.30; =0.012). Increased survival was significantly associated ( < 0.05) with chordoma (HR = 0.27; =0.010), chondrosarcoma (HR = 0.75; =0.002), treatment at an academic center (HR = 0.64; =0.039), and a private (HR = 0.67; =0.006) or Medicare (HR = 0.71; =0.043) insurer. A transition in care was not associated with a survival disadvantage (HR = 0.90; =0.14). CONCLUSIONS: Longer TTI was not associated with decreased overall survival in localized, high-grade primary bone sarcoma in adults. This is important in counseling patients, who may delay treatment to receive a second opinion or seek referral to a higher volume sarcoma center.
Long-Term Follow-up of a Randomized Study of Oral Etoposide versus Fermentatum Pini as Maintenance Therapy in Osteosarcoma Patients in Complete Surgical Remission after Second Relapse.
Longhi A, Cesari M, Serra M … +1 more , Mariani E
Sarcoma · 2020 · PMID 32398946 · Full text
BACKGROUND: In relapsed osteosarcoma, the 5-yr postrelapse disease-free survival (PRDFS) rate after the second relapse is <20%. In June 2007, a randomized study was started comparing oral etoposide vs fermentatum Pini (...
BACKGROUND: In relapsed osteosarcoma, the 5-yr postrelapse disease-free survival (PRDFS) rate after the second relapse is <20%. In June 2007, a randomized study was started comparing oral etoposide vs fermentatum Pini (an extract derived from the parasitic plant L., European mistletoe) as maintenance therapy in patients with metastatic osteosarcoma in complete surgical remission after the second relapse. The primary endpoint was the PRDFS rate at 12 months (compared to the historical control rate). This is a long-term updated result. . 10 patients received oral etoposide 50 mg/m daily for 21 days every 28 days for 6 months, and 9 patients received fermentatum Pini 3 times/wk subcutaneously for 1 year. The study closed early in July 2011 due to insufficient recruitment. Lymphocyte subpopulations were analyzed at T0, T3, T6, T9, and T12 months. RESULTS: On 30 June 2019, at a median follow-up ITT of 83 months (range 3-144 ms), a median PRDFS of 106 ms (2-144) was observed in the arm with 5/9 patients who never relapse vs a PRDFS of 7 months (3-134) in the etoposide arm (all patients in the Etoposide arm relapsed) (hazard ratio HR = 0.287, 95% CI: 0.076-0.884, =0.03). Model forecast 10-yr overall survival rates as 64% in the arm and 33% in the etoposide arm. Lymphocyte subpopulation counts (CD3, CD4, and CD56) showed an increase in the Viscum arm while a decrease was observed in the etoposide arm during treatment. CONCLUSIONS: After 12 years from the start of the trial, the patients in the arm continue to show a considerably longer PRDFS compared to oral etoposide, and a trend for an advantage in OS is evident even if the number of treated patients is too small to draw conclusions. as maintenance treatment after complete surgical remission in relapsed osteosarcoma should be further investigated and compared with other drugs.
Results of a Randomized, Double-Blinded, Placebo-Controlled, Phase 2.5 Study of Saracatinib (AZD0530), in Patients with Recurrent Osteosarcoma Localized to the Lung.
Baird K, Glod J, Steinberg SM … +19 more , Reinke D, Pressey JG, Mascarenhas L, Federman N, Marina N, Chawla S, Lagmay JP, Goldberg J, Milhem M, Loeb DM, Butrynski JE, Turpin B, Staddon A, Spunt SL, Jones RL, Rodler ET, Schuetze SM, Okuno SH, Helman L
Sarcoma · 2020 · PMID 32398945 · Full text
PURPOSE: Osteosarcoma is a rare cancer and a third of patients who have completed primary treatment will develop osteosarcoma recurrence. The Src pathway has been implicated in the metastatic behavior of osteosarcoma; ab...
PURPOSE: Osteosarcoma is a rare cancer and a third of patients who have completed primary treatment will develop osteosarcoma recurrence. The Src pathway has been implicated in the metastatic behavior of osteosarcoma; about 95% of samples examined express Src or have evidence of downstream activation of this pathway. Saracatinib (AZD0530) is a potent and selective Src kinase inhibitor that was evaluated in adults in Phase 1 studies. The primary goal of this study was to determine if treatment with saracatinib could increase progression-free survival (PFS) for patients who have undergone complete resection of osteosarcoma lung metastases in a double-blinded, placebo-controlled trial. . Subjects with recurrent osteosarcoma localized to lung and who had complete surgical removal of all lung nodules were randomized within six weeks after complete surgical resection. Saracatinib, or placebo, was administered at a dose of 175 mg orally, once daily, for up to thirteen 28-day cycles. RESULTS: Thirty-seven subjects were included in the analyses; 18 subjects were randomized to receive saracatinib and 19 to receive placebo. Intent-to-treat analysis demonstrated a median PFS of 19.4 months in the saracatinib treatment group and 8.6 months in the placebo treatment group (=0.47). Median OS was not reached in either arm. CONCLUSIONS: Although saracatinib was well tolerated in this patient population, there was no apparent impact of the drug in this double-blinded, placebo-controlled trial on OS, and Src inhibition alone may not be sufficient to suppress metastatic progression in osteosarcoma. There is a suggestion of potential clinical benefit as evidenced by longer PFS in patients randomized to saracatinib based on limited numbers of patients treated.
Diagnostic Value of TLE1 in Synovial Sarcoma: A Systematic Review and Meta-Analysis.
El Beaino M, Jupiter DC, Assi T … +4 more , Rassy E, Lazar AJ, Araujo DM, Lin PP
Sarcoma · 2020 · PMID 32322158 · Full text
BACKGROUND: Synovial sarcoma can present morphologically in multiple forms, including biphasic and monophasic subtypes. As a result, the histological diagnosis can sometimes be challenging. Transducin-Like Enhancer 1 (TL...
BACKGROUND: Synovial sarcoma can present morphologically in multiple forms, including biphasic and monophasic subtypes. As a result, the histological diagnosis can sometimes be challenging. Transducin-Like Enhancer 1 (TLE1) is a transcriptional corepressor that normally is involved in embryogenesis and hematopoiesis but is also expressed in certain tumors. This systematic review examines the potential role of TLE1 as a diagnostic biomarker for the synovial sarcoma. . A literature review and meta-analysis were conducted using the electronic databases Pubmed, the Cochrane Library, and Google Scholar. Thirteen studies met our eligibility criteria and were selected for in-depth analysis. RESULTS: The mean sensitivity and specificity of TLE1 in detecting synovial sarcoma were 94% (95% CI 91%-97%) and 81% (95% CI 72%-91%), respectively, when all studies were aggregated together. The mean positive predictive value (PPV) of TLE1 was 75% (95% CI 62%-87%), whereas the negative predictive value (NPV) was 96% (95% CI 93%-98%). CONCLUSION: TLE1 is a sensitive and specific marker for synovial sarcoma that can aid in its diagnosis. Due to its involvement in several relevant signaling pathways, TLE1 might have direct relevance to the pathophysiology of the disease.
Clinical Outcomes of Osteoarticular Extracorporeal Irradiated Autograft for Malignant Bone Tumor.
Takenaka S, Araki N, Ueda T … +7 more , Kakunaga S, Imura Y, Hamada KI, Outani H, Naka N, Myoui A, Yoshikawa H
Sarcoma · 2020 · PMID 32317858 · Full text
METHODS: We retrospectively reviewed 33 patients who underwent osteoarticular ECIA after bone tumor resection from 1988 to 2014. We investigated complications, radiographic changes by the International Society of Limb Sa...
METHODS: We retrospectively reviewed 33 patients who underwent osteoarticular ECIA after bone tumor resection from 1988 to 2014. We investigated complications, radiographic changes by the International Society of Limb Salvage graft evaluation criteria, and functional outcomes according to the Musculoskeletal Tumor Society scoring system. RESULTS: Fifteen patients were reoperated upon due to infection ( = 9), protruding fixation implant ( = 4), or fracture of the grafted bone ( = 2). The average radiographic evaluation score was 66.4%, and the median functional score was 23 (77%). The radiographic score for the proximal humerus or proximal tibia was lower than that for the other locations. The functional score was not different among the autograft sites but was related to the radiographic score. CONCLUSION: Although osteoarticular ECIA is one of the reasonable surgical options for patients with tumors for which reliable prostheses are not available, we do not recommend osteoarticular ECIA as a routine procedure because of high complication rate.
Current Approaches for Personalized Therapy of Soft Tissue Sarcomas.
Kirsanov KI, Lesovaya EA, Fetisov TI … +3 more , Bokhyan BY, Belitsky GA, Yakubovskaya MG
Sarcoma · 2020 · PMID 32317857 · Full text
Soft tissue sarcomas (STS) are a highly heterogeneous group of cancers of mesenchymal origin with diverse morphologies and clinical behaviors. While surgical resection is the standard treatment for primary STS, advanced...
Soft tissue sarcomas (STS) are a highly heterogeneous group of cancers of mesenchymal origin with diverse morphologies and clinical behaviors. While surgical resection is the standard treatment for primary STS, advanced and metastatic STS patients are not eligible for surgery. Systemic treatments, including standard chemotherapy and newer chemical agents, still play the most relevant role in the management of the disease. Discovery of specific genetic alterations in distinct STS subtypes allowed better understanding of mechanisms driving their pathogenesis and treatment optimization. This review focuses on the available targeted drugs or drug combinations based on genetic aberration involved in STS development including chromosomal translocations, oncogenic mutations, gene amplifications, and their perspectives in STS treatment. Furthermore, in this review, we discuss the possible use of chemotherapy sensitivity and resistance assays (CSRA) for the adjustment of treatment for individual patients. In summary, current trends in personalized management of advanced and metastatic STS are based on combination of both genetic testing and CSRA.
Induction of Myogenic Differentiation Improves Chemosensitivity of Chemoresistant Cells in Soft-Tissue Sarcoma Cell Lines.
Dawson LE, D'Agostino L, Hakim AA … +5 more , Lackman RD, Brown SA, Sensenig RB, Antonello ZA, Kuzin II
Sarcoma · 2020 · PMID 32300280 · Full text
Rhabdomyosarcoma (RMS) and rhabdoid tumors (RT) are rare soft-tissue malignancies with the highest incidence in infants, children, and adolescents. Advanced, recurrent, and/or metastatic RMS and RT exhibit poor response...
Rhabdomyosarcoma (RMS) and rhabdoid tumors (RT) are rare soft-tissue malignancies with the highest incidence in infants, children, and adolescents. Advanced, recurrent, and/or metastatic RMS and RT exhibit poor response to treatment. One of the main mechanisms behind resistance to treatment is believed to be intratumoral heterogeneity. In this study, we investigated the myogenic determination factor 1 (MYOD1) and Noggin (NOG) markers in an embryonal RMS (ERMS) cell line and an RT cell line and the differential response of the MYOD1 and NOG expressing subpopulations to chemotherapy. Importantly, we found that these markers together identify a subpopulation of cells (MYOD1+ NOG+ cells) with primary resistance to Vincristine and Doxorubicin, two commonly used chemotherapies for ERMS and RT. The chemoresistant MYOD1+ NOG+ cells express markers of undifferentiated cells such as myogenin and ID1. Combination of Vincristine with TPA/GSK126, a drug combination shown to induce differentiation of RMS cell lines, is able to partially overcome MYOD1/NOG cells chemoresistance.
Prognostic Factors for Development of Subsequent Metastases in Localized Osteosarcoma: A Systematic Review and Identification of Literature Gaps.
Basile P, Greengard E, Weigel B … +1 more , Spector L
Sarcoma · 2020 · PMID 32300279 · Full text
AIM: To investigate prognostic factors in pediatric and young adult patients with localized osteosarcoma that could predict the development of subsequent pulmonary metastases and lead to an ability to risk-stratify thera...
AIM: To investigate prognostic factors in pediatric and young adult patients with localized osteosarcoma that could predict the development of subsequent pulmonary metastases and lead to an ability to risk-stratify therapy. We performed a systematic review of the literature published since January 1990 to establish common evidence-based prognostic factors. METHODS: PubMed and Embase searches (Jan 1990-Aug 2018) were performed. Two reviewers independently selected papers for patients with localized osteosarcoma with subsequent metastatic development and then reviewed for quality of methods and prognostic factors. RESULTS: Database searches yielded 216 unique results. After screening, 27 full-text articles were studied in depth, with 9 items fulfilling predetermined inclusion and exclusion criteria. Age, tumor location, tumor size/volume, and histologic response carried independent prognostic value in the majority of the studies. CONCLUSIONS: Several prognostic factors seemed to be consistent amongst the studies, but the heterogeneity and smaller sizes of the study populations made pooling of results difficult. Standardization of larger patient populations and consistent definitions/cutoffs for prognostic factors are needed to further assess for consistent prognostic factors and potential predictive models to be developed.
Axillary Lymph Node Dissection in Angiosarcomas of the Breast: An Asian Institutional Perspective.
Ragavan S, Lim HJ, Tan JW … +8 more , Hendrikson J, Chan JY, Farid M, Chia CS, Tan GHC, Soo KC, Teo MCC, Ong CJ
Sarcoma · 2020 · PMID 32300278 · Full text
Angiosarcomas of the breast (ASB) are rare, making up to less than 8% of all angiosarcomas. The surgical management for this disease continues to vary throughout centres worldwide due to the current limited evidence. We...
Angiosarcomas of the breast (ASB) are rare, making up to less than 8% of all angiosarcomas. The surgical management for this disease continues to vary throughout centres worldwide due to the current limited evidence. We aim to examine the necessity of axillary lymph node dissection in this pathology through a retrospective study of axillary metastasis and recurrence patterns in patients treated at our institution. A retrospective review of a prospectively-maintained database was performed. All adult patients with a histologically confirmed diagnosis of ASB seen at the National Cancer Centre Singapore between 2006 and 2019 were identified. Axillary lymph node status, treatment, survival, and recurrence data were collated. Thirteen patients were identified with a confirmed diagnosis of ASB, of which there were 11 primary and 2 secondary angiosarcoma cases. Eight patients had some form of axillary lymph node dissection and 5 did not. No positive nodes were found in any examined axillary nodes despite high median number of nodes harvested (13, range 8-24). 5/13 patients had disease progression, of whom none had locoregional recurrence to the axilla. ASB continues to be rare and recurrent and presents as a challenge to treat. Axillary lymph node involvement is most likely not present in a majority of patients. Prophylactic removal is unwarranted in patients presenting without lymph node involvement due to the lack of axillary metastasis.
Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis.
Schöffski P, Timmermans I, Hompes D … +14 more , Stas M, Sinnaeve F, De Leyn P, Coosemans W, Van Raemdonck D, Hauben E, Sciot R, Clement P, Bechter O, Beuselinck B, Woei-A-Jin FJSH, Dumez H, Nafteux P, Wessels T
Sarcoma · 2020 · PMID 32300277 · Full text
BACKGROUND: Solitary fibrous tumor (SFT) is a rare variant of soft tissue sarcoma (STS). . We reviewed SFT patients (pts) treated at our institution between 12/1990 and 09/2017. RESULTS: We identified 94 pts with a media...
BACKGROUND: Solitary fibrous tumor (SFT) is a rare variant of soft tissue sarcoma (STS). . We reviewed SFT patients (pts) treated at our institution between 12/1990 and 09/2017. RESULTS: We identified 94 pts with a median follow-up (mFU) of 4.7 years (range: 0.1-21.53). Primary sites were the chest (33%), abdomen (21.3%), brain (12.8%), and extremities (9.6%); 6.4% of pts presented with synchronous metastasis. Median overall survival (mOS) from the first diagnosis was 56.0 months (m) (0.3-258.3). Doege-Potter syndrome was seen in 2.1% of pts. Primary resection was performed in 86 pts (91.5%). Median progression-free survival was 34.1 m (1.0-157.1), and 43% of pts stayed SFT-free during FU. Local recurrence occurred in 26.7% after a mFU of 35.5 m (1.0-153.8), associated with an OS of 45.1 m (4.7-118.2). Metachronous metastasis occurred in 30.2% after a mFU of 36.0 m (0.1-157.1). OS in metastatic pts was 19.0 m (0.3-149.0). Systemic therapy was given to 26 pts (27.7%) with inoperable/metastatic disease. The most common (57.7%) upfront therapy was doxorubicin, achieving responses in 13.3% of pts with a PFS of 4.8 m (0.4-23.8). In second line, pts were treated with ifosfamide or pazopanib, the latter achieving the highest response rates. Third-line treatment was heterogeneous. CONCLUSION: SFT is an orphan malignancy with a highly variable clinical course and a considerable risk of local failure and metachronous metastasis. Surgery is the only curative option; palliative systemic therapy is used in inoperable/metastatic cases but achieves low response rates. The highest response rates are seen with pazopanib in second/third line.
Treatment Patterns and Healthcare Resource Utilization among Patients with Advanced or Metastatic Soft Tissue Sarcoma in US Community Practices.
Nadler E, Aguilar K, Wentworth C … +6 more , Boyd M, Amirian ES, Barker S, French P, Wilson T, Hess LM
Sarcoma · 2020 · PMID 32256184 · Full text
INTRODUCTION: This study was designed to describe demographic and clinical characteristics of patients diagnosed with advanced or metastatic soft tissue sarcoma (STS) and to examine treatment and healthcare resource util...
INTRODUCTION: This study was designed to describe demographic and clinical characteristics of patients diagnosed with advanced or metastatic soft tissue sarcoma (STS) and to examine treatment and healthcare resource utilization patterns of this patient population in a United States (US) community-based oncology practice setting over time. METHODS AND MATERIALS: A retrospective observational study was conducted within the US Oncology Network (USON). Patients were eligible if they were diagnosed with advanced or metastatic STS and were treated at a USON site between 01 July 2015 and 31 August 2018. Demographic, clinical, and treatment characteristics were described for the overall study population. Comparisons between patients by time period (prior to and after October 2016) were evaluated using the test for continuous variables and chi-squared test for categorical variables. Data were available for analysis through 31 August 2018. RESULTS: Demographic and clinical characteristics of the eligible study cohort ( = 376) were similar between patients who initiated treatment before and after October 2016 (all > 0.05). Forty-three unique regimens were observed in the first-line setting, with the predominant regimen (gemcitabine + docetaxel) received by 33.2% ( = 125) patients. Prior to October 2016, 45.4% of patients received first-line gemcitabine + docetaxel, while 29.0% received this regimen after October 2016. CONCLUSIONS: While demographic and clinical characteristics were similar, treatment patterns changed in 2016. Future research should evaluate the impact of changing drug approvals and clinical trial results on future treatment patterns.
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