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Survey of Paediatric Oncologists and Pathologists regarding Their Views and Experiences with Variant Translocations in Ewing and Ewing-Like Sarcoma: A Report of the Children's Oncology Group. 儿科肿瘤学家和病理学家对尤因和尤因样肉瘤变异易位的看法和经验的调查:一份儿童肿瘤学组的报告。
Sarcoma Pub Date : 2020-12-05 eCollection Date: 2020-01-01 DOI: 10.1155/2020/3498549
Michael D Kinnaman, Chong Zhu, Daniel A Weiser, Sana Mohiuddin, Pooja Hingorani, Michael Roth, Jonathan Gill, Katherine A Janeway, Richard Gorlick, Stephen L Lessnick, Patrick J Grohar
{"title":"Survey of Paediatric Oncologists and Pathologists regarding Their Views and Experiences with Variant Translocations in Ewing and Ewing-Like Sarcoma: A Report of the Children's Oncology Group.","authors":"Michael D Kinnaman,&nbsp;Chong Zhu,&nbsp;Daniel A Weiser,&nbsp;Sana Mohiuddin,&nbsp;Pooja Hingorani,&nbsp;Michael Roth,&nbsp;Jonathan Gill,&nbsp;Katherine A Janeway,&nbsp;Richard Gorlick,&nbsp;Stephen L Lessnick,&nbsp;Patrick J Grohar","doi":"10.1155/2020/3498549","DOIUrl":"https://doi.org/10.1155/2020/3498549","url":null,"abstract":"<p><p>Advances in molecular diagnostics have identified subsets of Ewing and Ewing-like sarcomas driven by variant translocations with unique biology. It is likely that patients with these tumours will have different clinical features and therapeutic outcomes. Nevertheless, the management of these patients both locally and within cooperative group trials depends on the local pathological diagnosis. It is not known what molecular diagnostic approaches are employed by local pathologists or if the exact translocation is commonly determined. In addition, it is not known what therapeutic approaches are employed for these patients or what cooperative trials are deemed appropriate for these patients by expert consensus. To answer these questions, we performed an international survey of oncologists and pathologists to better understand the diagnostic approaches used to identify variant translocations and the influence the findings have on therapy and clinical trial eligibility. An online survey was distributed to oncologists and pathologists primarily in North America. A total of 141 surveys were completed, representing a 28% response rate. The majority of respondents considered EWSR1-ETS gene family translocations (range 61-96%) to be Ewing sarcoma and would include them on the primary arm of a Ewing sarcoma clinical trial. There was a lack of consensus on how to classify and stratify BCOR-CCNB3, CIC-DUX4, and EWSR1+ with non-ETS partner fusions. Most respondents were either unsure how their institution tested, or their institution did not perform the test. In cases with atypical Ewing morphology, most respondents favoured additional fusion transcript testing. There is a lack of consensus regarding the classification and stratification of rare molecular subtypes in Ewing sarcoma. It is not clear how these alternative translocations have impacted outcomes for past clinical studies. This suggests a need for molecular confirmation of diagnoses and centralized or minimum standardization of testing for future trial enrolment.</p>","PeriodicalId":21431,"journal":{"name":"Sarcoma","volume":"2020 ","pages":"3498549"},"PeriodicalIF":0.0,"publicationDate":"2020-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/3498549","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38854961","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 10
Potential Association between Kaposi Sarcoma and Gout: An Exploratory Observational Study. 卡波西肉瘤与痛风之间的潜在联系:一项探索性观察研究。
Sarcoma Pub Date : 2020-12-01 eCollection Date: 2020-01-01 DOI: 10.1155/2020/8844970
Assaf Moore, Idit Peretz, Lilach Yosef, Daniel A Goldstein, Hadar Goldvaser, Suzanna Horn, Yonatan Edel, Alona Zer
{"title":"Potential Association between Kaposi Sarcoma and Gout: An Exploratory Observational Study.","authors":"Assaf Moore,&nbsp;Idit Peretz,&nbsp;Lilach Yosef,&nbsp;Daniel A Goldstein,&nbsp;Hadar Goldvaser,&nbsp;Suzanna Horn,&nbsp;Yonatan Edel,&nbsp;Alona Zer","doi":"10.1155/2020/8844970","DOIUrl":"https://doi.org/10.1155/2020/8844970","url":null,"abstract":"<p><strong>Background: </strong>Kaposi sarcoma is a rare vascular mesenchymal neoplasm, associated with Human Herpes Virus 8 (HHV8). Gout is a condition clinically characterized by recurrent flares of arthritis and hyperuricemia. Following our clinical impression that patients with classical Kaposi sarcoma (CKS) have a high rate of gout, we explored this in a retrospective manner.</p><p><strong>Methods: </strong>All consecutive patients diagnosed with sarcoma or carcinosarcoma within a single tertiary center between 1/2012-12/2017 were identified through the pathology department database. A cohort of CKS patients was compared with the non-Kaposi sarcoma and carcinosarcoma cohort. Data were extracted from patients' electronic medical records. Patients younger than 18 and patients without clinical data available were excluded. Association between diagnosis of gout and CKS was assessed and adjusted for risk factors.</p><p><strong>Results: </strong>Three hundred and sixty-one patients were eligible for this analysis, 61 were diagnosed with CKS and 300 with other types of sarcoma. We found a higher incidence of gout in CKS patients, 11/61 (18%) patients, compared with 8/300 (2.6%) with other types of sarcoma, odds ratio (OR) 8.0 (<i>P</i> < 0.00001). This association persisted when adjusted for age >39 years (OR = 6.7, <i>P</i> < 0.00001), age and male sex (OR = 4.97, <i>P</i> < 0.0001), and when adjusting for multiple confounding factors and medical comorbidities.</p><p><strong>Conclusions: </strong>We have demonstrated a statistically significant association between gout and CKS. As risk factors for gout were accounted for, this association may be explained by HHV8 immune-related effects. This should be further explored in vitro and in population-based studies.</p>","PeriodicalId":21431,"journal":{"name":"Sarcoma","volume":"2020 ","pages":"8844970"},"PeriodicalIF":0.0,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/8844970","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25314495","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Clinical Characteristics, Management, and Outcomes of 19 Nonpediatric Patients with Desmoplastic Small Round Cell Tumor: A Cohort of Brazilian Patients. 19例非儿科结缔组织增生小圆细胞瘤患者的临床特征、处理和结局:一组巴西患者。
Sarcoma Pub Date : 2020-10-28 eCollection Date: 2020-01-01 DOI: 10.1155/2020/8713165
Fernando Campos, Daniel L Coutinho, Maria Letícia G Silva, Ademar Lopes, Antônio Nascimento, Samuel Aguiar Júnior, Ulisses R Nicolau, Maria Nirvana Formiga, Felipe D'Almeida Costa, Celso Mello
{"title":"Clinical Characteristics, Management, and Outcomes of 19 Nonpediatric Patients with Desmoplastic Small Round Cell Tumor: A Cohort of Brazilian Patients.","authors":"Fernando Campos,&nbsp;Daniel L Coutinho,&nbsp;Maria Letícia G Silva,&nbsp;Ademar Lopes,&nbsp;Antônio Nascimento,&nbsp;Samuel Aguiar Júnior,&nbsp;Ulisses R Nicolau,&nbsp;Maria Nirvana Formiga,&nbsp;Felipe D'Almeida Costa,&nbsp;Celso Mello","doi":"10.1155/2020/8713165","DOIUrl":"https://doi.org/10.1155/2020/8713165","url":null,"abstract":"<p><p>Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive mesenchymal malignancy, usually affecting young males. There is no consensus on the best therapeutic approach. We seek to characterize a cohort of nonpediatric patients with DSRCT treated at a large Brazilian cancer center. We performed a retrospective analysis of patients with histologically confirmed DSRCT referred to our institution (2007-2020). Clinical and imaging data were extracted and summarized with descriptive statistics. Survival analyses were conducted by the Kaplan-Meier method and compared with the log-rank test. We included 19 patients with DSRCT, the median age at diagnosis was 26 years (range: 15-41 years), and 68% were male. Ninety percent presented with abdominopelvic masses, and 32% had extra-abdominal metastasis at diagnosis. Eleven patients (58%) underwent surgery, four patients (21%) received whole abdominal adjuvant radiotherapy, and five patients (26%) had hyperthermic intraperitoneal chemotherapy. Median OS was 27 months (interquartile range: 18-51 m). The five-year OS rate was 12%. Our data confirm the aggressiveness of DSRCT despite intense multimodality treatment. Outcomes of patients treated in a reference cancer center in a developing country are similar to cancer centers in developed nations. Multicenter cooperation is urgent to the development of clinical trials and to improve diagnosis and treatment efficacy.</p>","PeriodicalId":21431,"journal":{"name":"Sarcoma","volume":"2020 ","pages":"8713165"},"PeriodicalIF":0.0,"publicationDate":"2020-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/8713165","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38606789","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 6
Striving towards Normality in Daily Life: A Qualitative Study of Patients Living with Metastatic Gastrointestinal Stromal Tumour in Long-Term Clinical Remission. 在日常生活中努力走向正常:转移性胃肠道间质瘤患者长期临床缓解的定性研究。
Sarcoma Pub Date : 2020-10-06 eCollection Date: 2020-01-01 DOI: 10.1155/2020/1814394
Lena Fauske, Ivar Hompland, Geir Lorem, Kirsten Sundby Hall, Hilde Bondevik
{"title":"Striving towards Normality in Daily Life: A Qualitative Study of Patients Living with Metastatic Gastrointestinal Stromal Tumour in Long-Term Clinical Remission.","authors":"Lena Fauske,&nbsp;Ivar Hompland,&nbsp;Geir Lorem,&nbsp;Kirsten Sundby Hall,&nbsp;Hilde Bondevik","doi":"10.1155/2020/1814394","DOIUrl":"https://doi.org/10.1155/2020/1814394","url":null,"abstract":"<p><strong>Background: </strong>This study explored how patients with metastatic gastrointestinal stromal tumour (GIST) experience the psychosocial challenges associated with their disease and its treatment, as well as how that experience influenced their practical, relational, vocational, and existential life.</p><p><strong>Methods: </strong>This qualitative study has an explorative design and applied a phenomenological and hermeneutical approach. We conducted in-depth, semistructured interviews with 20 patients with metastatic GIST in long-term clinical remission. The gathered data were interpreted using a thematic analysis.</p><p><strong>Results: </strong>Living with metastatic GIST, as well as the side effects of the required medication, led to changes that limited the participants' daily life. They expressed how tiredness, impaired memory, and physical challenges were among the detrimental impacts of the disease on their family life, vocational life, social life, and leisure time. Adjustments were necessary to ensure they had sufficient energy to cope with the practical and relational aspects of everyday life. Feelings of uncertainty stemming from drug resistance, disease progression, and the possibility of early death were also experienced as challenging. Half the participants stated that it was difficult to keep negative mental health issues at bay, and all of them considered the time spent waiting for their scheduled follow-up scan to be burdensome.</p><p><strong>Conclusions: </strong>It is important to focus increased attention on how the daily practical and psychosocial life of patients with chronic cancer, including metastatic GIST, is affected by their disease. Doing so might provide health-care workers with clues regarding how best to guide and support such patients throughout their emotional journey and, therefore, to improve their quality of life. As new medical treatments can also prolong survival and induce long-term clinical remission in relation to several other forms of metastatic cancer, the findings concerning GIST reported in this study might have widespread implications.</p>","PeriodicalId":21431,"journal":{"name":"Sarcoma","volume":"2020 ","pages":"1814394"},"PeriodicalIF":0.0,"publicationDate":"2020-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/1814394","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38606588","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 6
Diagnostic Differences in Expert Second-Opinion Consultation Cases at a Tertiary Sarcoma Center. 第三期肉瘤中心专家第二意见咨询病例的诊断差异。
Sarcoma Pub Date : 2020-09-29 eCollection Date: 2020-01-01 DOI: 10.1155/2020/9810170
Asha Rupani, Magnus Hallin, Robin L Jones, Cyril Fisher, Khin Thway
{"title":"Diagnostic Differences in Expert Second-Opinion Consultation Cases at a Tertiary Sarcoma Center.","authors":"Asha Rupani,&nbsp;Magnus Hallin,&nbsp;Robin L Jones,&nbsp;Cyril Fisher,&nbsp;Khin Thway","doi":"10.1155/2020/9810170","DOIUrl":"https://doi.org/10.1155/2020/9810170","url":null,"abstract":"<p><p>Soft tissue tumors are diagnostically challenging, and it is recommended that these are reported or reviewed by specialist soft tissue pathologists. We present our experience with second-opinion (consultation) cases in a specialist tertiary sarcoma center. The aim of this study was to determine areas of diagnostic difficulty in soft tissue pathology. We assessed 581 second-opinion cases which were reviewed by two experienced pathologists in a period of one year. There was 62% concordance between the original and the second-opinion diagnosis, with diagnostic discrepancy in 38%. The largest group of soft tissue neoplasms received for second opinion was fibroblastic/myofibroblastic tumors, and most major diagnostic problems were encountered in adipocytic and so-called \"fibrohistiocytic\" tumors. Major diagnostic errors impacting management were found in 148 cases (25%). Morphologic assessment of tumors, judicious use of molecular techniques, newer immunostains and their interpretation, along with importance of knowledge of rarer entities were found to be most useful in avoiding errors.</p>","PeriodicalId":21431,"journal":{"name":"Sarcoma","volume":"2020 ","pages":"9810170"},"PeriodicalIF":0.0,"publicationDate":"2020-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/9810170","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38589111","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 15
Time Trends and Prognostic Factors for Overall Survival in Myxoid Liposarcomas: A Population-Based Study. 肌样脂肪肉瘤总生存期的时间趋势和预后因素:基于人群的研究
Sarcoma Pub Date : 2020-09-22 eCollection Date: 2020-01-01 DOI: 10.1155/2020/2437850
Jules Lansu, Winan J Van Houdt, Michael Schaapveld, Iris Walraven, Michiel A J Van de Sande, Vincent K Y Ho, Rick L Haas
{"title":"Time Trends and Prognostic Factors for Overall Survival in Myxoid Liposarcomas: A Population-Based Study.","authors":"Jules Lansu, Winan J Van Houdt, Michael Schaapveld, Iris Walraven, Michiel A J Van de Sande, Vincent K Y Ho, Rick L Haas","doi":"10.1155/2020/2437850","DOIUrl":"10.1155/2020/2437850","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>A population-based study was performed of patients with primary localized (<i>n</i> = 851) and metastatic (<i>n</i> = 50) MLS diagnosed in The Netherlands between 1989 and 2016, based on data from the National Cancer Registry.</p><p><strong>Results: </strong>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, <i>p</i>=0.00), a tumor size >5 cm (HR 2.18; <i>p</i>=0.00), and tumor location (trunk HR 1.29; <i>p</i>=0.09, upper limb HR 0.83; <i>p</i>=0.55, and \"other\" locations HR 2.73; <i>p</i>=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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":21431,"journal":{"name":"Sarcoma","volume":"2020 ","pages":"2437850"},"PeriodicalIF":0.0,"publicationDate":"2020-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7528038/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38464948","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Pharmacologic Inhibition of Ezrin-Radixin-Moesin Phosphorylation is a Novel Therapeutic Strategy in Rhabdomyosarcoma. 抑制Ezrin-Radixin-Moesin磷酸化是横纹肌肉瘤的一种新的治疗策略。
Sarcoma Pub Date : 2020-09-09 eCollection Date: 2020-01-01 DOI: 10.1155/2020/9010496
Austin Proudfit, Nabanita Bhunia, Debasis Pore, Yvonne Parker, Daniel Lindner, Neetu Gupta
{"title":"Pharmacologic Inhibition of Ezrin-Radixin-Moesin Phosphorylation is a Novel Therapeutic Strategy in Rhabdomyosarcoma.","authors":"Austin Proudfit,&nbsp;Nabanita Bhunia,&nbsp;Debasis Pore,&nbsp;Yvonne Parker,&nbsp;Daniel Lindner,&nbsp;Neetu Gupta","doi":"10.1155/2020/9010496","DOIUrl":"https://doi.org/10.1155/2020/9010496","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":21431,"journal":{"name":"Sarcoma","volume":"2020 ","pages":"9010496"},"PeriodicalIF":0.0,"publicationDate":"2020-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/9010496","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38448569","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 7
Epidemiology of Soft Tissue Sarcoma and Bone Sarcoma inItaly: Analysis of Data from 15 Population-Based Cancer Registries 意大利软组织肉瘤和骨肉瘤的流行病学:来自15个基于人群的癌症登记处的数据分析
Sarcoma Pub Date : 2020-09-01 DOI: 10.1155/2020/6142613
Sabrina Fabiano, P. Contiero, Giulio Barigelletti, Anna D’Agostino, A. Tittarelli, L. Mangone, I. Bisceglia, Salvatore Bongiorno, Lucia Elena De Lorenzis, G. Mazzoleni, M. Castaing, T. Intrieri, A. Puppo, G. Carrozzi, G. Sini, llaria Fontanili, L. Boschetti, R. Tumino, F. Bella, A. Fanetti, Maurizio Castelli, A. Ardizzone, F. Vittadello, S. Sciacca, G. Manneschi, C. Casella, C. Cirilli, F. Pala, M. Michiara, S. Migliazza, F. Ferrari, E. Spata, Y. Dinaro, I. Cometti, A. Maghini, G. Tagliabue
{"title":"Epidemiology of Soft Tissue Sarcoma and Bone Sarcoma inItaly: Analysis of Data from 15 Population-Based Cancer Registries","authors":"Sabrina Fabiano, P. Contiero, Giulio Barigelletti, Anna D’Agostino, A. Tittarelli, L. Mangone, I. Bisceglia, Salvatore Bongiorno, Lucia Elena De Lorenzis, G. Mazzoleni, M. Castaing, T. Intrieri, A. Puppo, G. Carrozzi, G. Sini, llaria Fontanili, L. Boschetti, R. Tumino, F. Bella, A. Fanetti, Maurizio Castelli, A. Ardizzone, F. Vittadello, S. Sciacca, G. Manneschi, C. Casella, C. Cirilli, F. Pala, M. Michiara, S. Migliazza, F. Ferrari, E. Spata, Y. Dinaro, I. Cometti, A. Maghini, G. Tagliabue","doi":"10.1155/2020/6142613","DOIUrl":"https://doi.org/10.1155/2020/6142613","url":null,"abstract":"Sarcomas are a heterogeneous group of rare cancers of mesenchymal origin. In this study, we provide updated, world age-standardised incidence rate (ASR) and European age-standardised incidence rate for malignant soft tissue sarcoma (ICD-O-3 topographic code C47–C49) and bone sarcoma (C40, C41) in Italy, by area (north, centre, and south) and by cancer registry. We also assess morphology in relation to site and area and assess metastases at diagnosis. We analysed 1,112 cases, with incidence 2009–2012, provided by 15 cancer registries (CRs) affiliated to the Association of Italian Cancer Registries (AIRTUM). Overall, ASR was 1.7/100,000/year for soft tissue sarcoma and 0.7 for bone sarcoma. Central Italy had the highest (2.4) ASR and south Italy had the lowest (1.6) ASR for soft tissue sarcoma. Central Italy had the highest (1.1) ASR and north Italy had the lowest (0.7) ASR for bone sarcoma. By CR, ASRs ranged from 1.1 to 2.6 for soft tissue sarcoma and from 0 to 1.4 for bone sarcoma. The most frequent soft tissue sarcomas were sarcoma not otherwise specified (NOS) (29.4%) and liposarcoma (22.2%); the most common bone sarcoma was chondrosarcoma (37.6%). Soft tissue sarcomas occurred most frequently (35.6%) in lower limb connective tissue; bone sarcomas arose mainly (68.8%) in long bones. The frequencies of morphologies arising at different sites varied considerably by Italian area; for example, 20% of hemangiosarcomas occurred in the head and neck in south Italy with 17% at this site in the centre and 6% in the north. For soft tissue sarcoma, the highest ASRs of 2.6 and 2.4 contrast with the lowest ASRs 1.1 and 1.3, suggesting high-risk hot spots that deserve further investigation. The marked variations in morphology distribution with site and geography suggest geographic variation in risk factors that may also repay further investigation particularly since sarcoma etiology is poorly understood.","PeriodicalId":21431,"journal":{"name":"Sarcoma","volume":"2020 1","pages":"1-10"},"PeriodicalIF":0.0,"publicationDate":"2020-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/6142613","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45848532","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 12
Angiosarcoma of the Scalp and Face: A Dosimetric Comparison of HDR Surface Applicator Brachytherapy and VMAT. 头皮和面部血管肉瘤:HDR表面涂抹器近距离放疗和VMAT的剂量学比较。
Sarcoma Pub Date : 2020-08-25 eCollection Date: 2020-01-01 DOI: 10.1155/2020/7615248
Devarati Mitra, Yaguang Pei, Ivan Buzurovic, Phillip M Devlin, Katherine Thornton, Chandrajit P Raut, Elizabeth H Baldini, Miranda B Lam
{"title":"Angiosarcoma of the Scalp and Face: A Dosimetric Comparison of HDR Surface Applicator Brachytherapy and VMAT.","authors":"Devarati Mitra,&nbsp;Yaguang Pei,&nbsp;Ivan Buzurovic,&nbsp;Phillip M Devlin,&nbsp;Katherine Thornton,&nbsp;Chandrajit P Raut,&nbsp;Elizabeth H Baldini,&nbsp;Miranda B Lam","doi":"10.1155/2020/7615248","DOIUrl":"https://doi.org/10.1155/2020/7615248","url":null,"abstract":"<p><strong>Purpose: </strong>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).</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":21431,"journal":{"name":"Sarcoma","volume":"2020 ","pages":"7615248"},"PeriodicalIF":0.0,"publicationDate":"2020-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/7615248","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38364068","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 4
Predicting Pathologic Bone Lesions Using Scout Computed Tomography (CT) Imaging. 使用Scout计算机断层扫描(CT)成像预测病理性骨病变。
Sarcoma Pub Date : 2020-08-06 eCollection Date: 2020-01-01 DOI: 10.1155/2020/5105196
Michael J Colello, Erin R Pichiotino, Stephanie L Tanner, Scott E Porter, Richard W Gurich
{"title":"Predicting Pathologic Bone Lesions Using Scout Computed Tomography (CT) Imaging.","authors":"Michael J Colello,&nbsp;Erin R Pichiotino,&nbsp;Stephanie L Tanner,&nbsp;Scott E Porter,&nbsp;Richard W Gurich","doi":"10.1155/2020/5105196","DOIUrl":"https://doi.org/10.1155/2020/5105196","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":21431,"journal":{"name":"Sarcoma","volume":"2020 ","pages":"5105196"},"PeriodicalIF":0.0,"publicationDate":"2020-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2020/5105196","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38312298","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
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