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1.
J Pak Med Assoc ; 71(Suppl 5)(8): S75-S78, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34634021

RESUMO

OBJECTIVE: To study the frequency of the thigh, hip and groin soft tissue sarcomas and retrospectively analyse the management, treatment results, and outcomes of these uncommon malignant tumours, in a tertiary care hospital of the city of Karachi. Methodology: Data of soft tissue tumours registered from 2017-2018 was retrieved during January 2019 to March 2019 from Aga Khan University Hospital, Karachi bone and soft tissue tumour registry. A retrospective review was performed and all soft tissue tumour cases treated with surgical intervention (with adjuvant /neoadjuvant therapy) or palliative intention were included. RESULTS: Total 119 cases of soft tissue tumours (STS) were identified out of which 85 were malignant cases (sarcomas) while 30 were benign. On presentation 84 (70.6%) were primary cases. On topographical distribution, there were 25 patients who had hip, groin and thigh sarcoma. Of these, 15 were males and 10 were females. As treatment, neo-adjuvant radiation was done in 4 (16%) patients and adjuvant chemo/radio therapy was given to 13 (52%) patients. Wide margin excision was performed in 19 (76%) patients and 4 (16%) had amputation. Reconstruction was offered to 3 (12%) patients. In post-surgical complications, 1 (4%) patient had wound infection. On final surgical histopathology, majority of the sarcomas were liposarcomas, myxofibrosarcoma, synovial sarcoma and Leiomyosarcoma. Post-surgery recurrence occurred in 7 (28%) patients. Overall survival was 76%. CONCLUSIONS: In treatment of soft tissue sarcoma, limb salvage is an achievable option and survival results are also good.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Feminino , Virilha , Humanos , Masculino , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/terapia , Estudos Retrospectivos , Sarcoma/epidemiologia , Sarcoma/terapia , Neoplasias de Tecidos Moles/epidemiologia , Neoplasias de Tecidos Moles/terapia , Centros de Atenção Terciária , Coxa da Perna , Resultado do Tratamento
2.
BMJ Open ; 11(9): e047175, 2021 09 02.
Artigo em Inglês | MEDLINE | ID: mdl-34475158

RESUMO

OBJECTIVES: The COVID-19 pandemic is unprecedented as a global crisis over the last century. How do specialist surgeons make decisions about patient care in these unprecedent times? DESIGN: Between April and May 2020, we conducted an international qualitative study. Sarcoma surgeons from diverse global settings participated in 60 min interviews exploring surgical decision making during COVID-19. Interview data were analysed using an inductive thematic analysis approach. SETTING: Participants represented public and private hospitals in 14 countries, in different phases of the first wave of the pandemic: Australia, Argentina, Canada, India, Italy, Japan, Nigeria, Singapore, Spain, South Africa, Switzerland, Turkey, UK and USA. PARTICIPANTS: From 22 invited sarcoma surgeons, 18 surgeons participated. Participants had an average of 19 years experience as a sarcoma surgeon. RESULTS: 17/18 participants described a decision they had made about patient care since the start of the pandemic that was unique to them, that is, without precedence. Common to 'unique' decisions about patient care was uncertainty about what was going on and what would happen in the future (theme 1: the context of uncertainty), the impact of the pandemic on resources or threat of the pandemic to overwhelm resources (theme 2: limited resources), perceived increased risk to self (theme 3: duty of care) and least-worst decision making, in which none of the options were perceived as ideal and participants settled on the least-worst option at that point in time (theme 4: least-worst decision making). CONCLUSIONS: In the context of rapidly changing standards of justice and beneficence in patient care, traditional decision-making frameworks may no longer apply. Based on the experiences of surgeons in this study, we describe a framework of least-worst decision making. This framework gives rise to actionable strategies that can support decision making in sarcoma and other specialised fields of surgery, both during the current crisis and beyond.


Assuntos
COVID-19 , Sarcoma , Tomada de Decisões , Humanos , Pandemias , SARS-CoV-2 , Sarcoma/epidemiologia , Sarcoma/cirurgia
3.
J Pediatr Hematol Oncol ; 43(6): e832-e840, 2021 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-34397617

RESUMO

BACKGROUND: We sought to compare survival outcomes of sarcomas in the pediatric and adolescent/young adult populations with universal care access in the Military Health System (MHS) to those from the United States general population. METHODS: We compared data from the Department of Defense's (DoD) Automated Central Tumor Registry (ACTUR) and the National Cancer Institute's (NCI) Surveillance, Epidemiology, and End Results (SEER) program on the overall survival of patients 24 years or younger with histologically or microscopically confirmed sarcoma between diagnosed between January 1, 1987, and December 31, 2013. The Kaplan-Meier survival curves were used to compare survival between the 2 patient populations. Cox proportional hazard models were used to estimate hazard ratios (HRs) and 95% confidence intervals (CIs) comparing ACTUR relative to SEER. RESULTS: The final analysis included 309 and 1236 bone sarcoma cases and 465 and 1860 soft tissue sarcoma cases from ACTUR and SEER, respectively. Cox proportional hazards analysis showed soft tissue sarcoma patients in ACTUR had significantly better overall (HR=0.73, 95% CI=0.55-0.98) and 5-year overall (HR=0.63, 95% CI=0.46-0.86) survival compared with SEER patients, but no significant difference in overall or 5-year overall survival between ACTUR and SEER patients with bone sarcoma. CONCLUSION: Survival data from the ACTUR database demonstrated significantly improved overall survival for soft tissue sarcomas and equivalent survival in bone sarcomas compared with that reported by SEER.


Assuntos
Neoplasias Ósseas/epidemiologia , Sarcoma/epidemiologia , Neoplasias de Tecidos Moles/epidemiologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Serviços de Saúde Militar , Programa de SEER , Análise de Sobrevida , Estados Unidos/epidemiologia , Adulto Jovem
4.
Syst Rev ; 10(1): 231, 2021 08 13.
Artigo em Inglês | MEDLINE | ID: mdl-34389054

RESUMO

BACKGROUND: Sarcomas are a rare and heterogeneous group of tumors originating from mesenchymal or connective tissue. They represent less than 1% of all adult cancers. The etiology and epidemiology of sarcomas remain understudied and poorly understood. The main objective of our study was to systematically assess the association between various occupational exposures and risk of sarcomas. METHODS: We performed a systematic literature search using the PubMed, Scopus, EMBASE and Cochrane databases to identify relevant cohort and case-control studies. A meta-analysis method was applied on the incidence and mortality outcomes where the estimate with 95% confidence interval (CI) was obtained. RESULTS: We included a total of 50 publications in our systematic review and 35 in meta-analysis. For exposures to phenoxy herbicides and chlorophenols, the pooled odds ratio (OR) for sarcoma was 1.85 (95% CI: 1.22, 2.82), based on 16 studies with 2254 participants, while the pooled standardized mortality ratio was 40.93 (95% CI 2.19, 765.90), based on 4 cohort studies with 59,289 participants. For exposure to vinyl chloride monomers the pooled risk ratios for angiosarcoma of the liver and other STS were 19.23 (95% CI 2.03, 182.46) and 2.23 (95 CI 1.55, 3.22) respectively based on 3 cohort studies with 12,816 participants. Exposure to dioxins was associated with an increased STS mortality; the pooled standardized mortality ratio was 2.56 (95% CI 1.60, 4.10) based on 4 cohort studies with 30,797 participants. Finally, woodworking occupation was associated with an increased risk of STS with the pooled OR of 2.16 (95% CI 1.39, 3.36). CONCLUSIONS: Our findings suggest a positive association between higher exposure to dioxins and increased mortality from STS, between cumulative exposure to vinyl chloride monomers and increased mortality from angiosarcoma of the liver and STS, and between woodworking occupation and STS incidence. These findings were all statistically significant.


Assuntos
Doenças Profissionais , Exposição Ocupacional , Sarcoma , Adulto , Estudos de Coortes , Humanos , Incidência , Doenças Profissionais/epidemiologia , Exposição Ocupacional/efeitos adversos , Sarcoma/epidemiologia , Sarcoma/etiologia
5.
J Surg Oncol ; 124(4): 635-645, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34091907

RESUMO

BACKGROUND: Primary sarcomas originating from the mobile spine portends a particularly sinister outcome. Rarity of the disease process has resulted in inconsistent data due to small sample size and heterogeneity in patient selection and analytics. METHODS: Surveillance, Epidemiology and End Result (SEER) database from 1975 to 2017 was queried to report incidence and survival data in 712 patients in the United States. Kaplan-Meier and Cox Regression were used to determine the prognostic factors affecting survival. RESULTS: Incidence of spinal sarcoma was 0.019 per 100,000 persons in 2017 and has not significantly changed since 2000 (p > 0.05). Disease-specific 5-year survival for the entire cohort was 57%. Osteosarcoma has the worst 5-year survival (39%) and chondrosarcoma has the best 5-year survival (69%). Independent predictors of survival for the entire cohort included age, grade, and stage. Stage was an independent predictor of survival for every histologic subtype. Additional predictors of survival for spinal osteosarcoma, Ewing sarcoma, and chondrosarcoma included age, size, and grade, respectively. CONCLUSIONS: The current study is an analysis of a population-based registry reporting incidence survival data for patients with sarcoma of mobile vertebral column. Survival and prognostic factors vary by histologic subtypes. There is lack of improvement in survival over the last three decades.


Assuntos
Programa de SEER/estatística & dados numéricos , Sarcoma/epidemiologia , Sarcoma/mortalidade , Coluna Vertebral/patologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Prognóstico , Sarcoma/classificação , Sarcoma/patologia , Taxa de Sobrevida , Estados Unidos/epidemiologia , Adulto Jovem
6.
JCO Glob Oncol ; 7: 1067-1073, 2021 06.
Artigo em Inglês | MEDLINE | ID: mdl-34191537

RESUMO

PURPOSE: In Scotland, approximately 350 sarcoma cases are diagnosed per year and treated in one of the five specialist centers. Many patients are required to travel long distances to access specialist care. The COVID-19 pandemic brought a number of rapid changes into the care for patients with cancer, with increasing utilization of telemedicine. We aimed to evaluate how the utilization of telemedicine affects professionals and patients across Scotland and care delivery, at the Beatson West of Scotland Cancer Centre Sarcoma Unit. METHODS: Between June 8 and August 25, 2020, we invited patients and professional sarcoma multidisciplinary team members to participate in separate online anonymous survey questionnaires, to assess their attitudes toward telemedicine. Data were extracted, and descriptive statistics were performed. RESULTS: Patient satisfaction (n = 64) with telemedicine was high (mean = 9.4/10) and comparable with traditional face-to-face appointments (mean = 9.5/10). Patients were receptive to the use of telemedicine in certain situations, with patients strongly opposed to being told bad news via telemedicine (88%). Providers recommended the use of telemedicine in certain patient populations and reported largely equivalent workloads when compared with traditional consultations. Providers reported that telemedicine should be integrated into regular practice (66%), with patients echoing this indicating a preference for a majority of telemedicine appointments (57%). CONCLUSION: Telemedicine in sarcoma care is favorable from both clinician and patient perspectives. Utilization of telemedicine for patients with rare cancers such as sarcomas is an innovative approach to the delivery of care, especially considering the time and financial pressures on patients who often live a distance away from specialist centers. Patients and providers are keen to move toward a more flexible, mixed system of care.


Assuntos
COVID-19 , Sarcoma , Telemedicina , Humanos , Pandemias , Satisfação do Paciente , SARS-CoV-2 , Sarcoma/epidemiologia , Sarcoma/terapia , Escócia/epidemiologia
7.
J Surg Oncol ; 124(3): 390-399, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-33866561

RESUMO

BACKGROUND: Patients undergoing an orthopedic surgery for bone or soft tissue sarcoma are at increased venous thromboembolism (VTE) risk. Unfortunately, there is a lack of thromboprophylaxis guidelines in this population. The purpose of this systematic review was to determine the soft tissue and bone sarcoma VTE rate and to explore the thromboprophylaxis regimens used. METHODS: The databases MEDLINE, EMBASE, and CENTRAL were queried using keywords related to VTE and long bone malignancy requiring surgical intervention to 2020. Included studied reported VTE rate in patients with surgically managed extremity sarcoma. Descriptive statistics and weighted mean totals were calculated. RESULTS: A total of 2082 studies were screened and 23 studies were included. The overall VTE rate was 2.9%, with a rate of 3.7% and 1.4% in patients with bone and soft tissue sarcomas, respectively. Low-molecular-weight heparin was the most commonly used chemoprophylaxis. CONCLUSIONS: There is a high VTE rate following sarcoma surgery. The VTE rate is higher in bone sarcoma surgery, which may be attributed to differences in surgery and postoperative recovery. There was no consensus on the duration or type of thromboprophylaxis used. Future research is needed to determine the most effective thromboprophylaxis regimen in patients with sarcoma and whether individualized thromboprophylaxis is required.


Assuntos
Neoplasias Ósseas/cirurgia , Procedimentos Ortopédicos/estatística & dados numéricos , Osteossarcoma/cirurgia , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/cirurgia , Tromboembolia Venosa/epidemiologia , Neoplasias Ósseas/epidemiologia , Neoplasias Ósseas/patologia , Estudos de Casos e Controles , Extremidades/patologia , Extremidades/cirurgia , Humanos , Estudos Observacionais como Assunto , Procedimentos Ortopédicos/efeitos adversos , Osteossarcoma/epidemiologia , Osteossarcoma/patologia , Ensaios Clínicos Controlados Aleatórios como Assunto , Sarcoma/epidemiologia , Neoplasias de Tecidos Moles/epidemiologia , Neoplasias de Tecidos Moles/patologia , Tromboembolia Venosa/etiologia
8.
Jpn J Clin Oncol ; 51(7): 1080-1087, 2021 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-33791777

RESUMO

OBJECTIVE: The relationship between the adolescent and young adult age groups and poor overall survival in soft tissue sarcoma and the risk factors for poor outcomes in adolescent and young adult patients with soft tissue sarcoma were analyzed. METHODS: The medical records of 7759 Japanese patients diagnosed with soft tissue sarcoma from 2006-13 were accessed from the Bone and Soft Tissue Tumor registry. The epidemiological features of adolescent and young adult patients were compared with those of other age groups. The cancer survival rates were calculated using the Kaplan-Meier method. The prognostic factors for cancer survival were analyzed with the Cox proportional hazards models. The primary endpoint for prognosis was tumor-related death. RESULTS: There were 210 children, 1467 adolescent and young adults, 2771 adults and 3311 elderly among the 7759 patients identified with soft tissue sarcoma. Compared with other age groups, the proportions of myxoid/round cell liposarcoma, synovial sarcoma, malignant peripheral nerve sheath tumor, primitive neuroectodermal tumor and rhabdomyosarcoma in adolescent and young adult patients were the highest, but none was significantly more prevalent in adolescent and young adult patients. On multivariate analysis, age was not a prognostic factor for poor cancer survival among adolescent and young adult patients with soft tissue sarcoma. The cancer survival rates of adolescent and young adult patients with malignant peripheral nerve sheath tumor were poorer than those of the other age groups; however, adolescent and young adult age was not a prognostic factor on multivariate analysis in malignant peripheral nerve sheath tumor patients. CONCLUSIONS: Our study is the first to investigate soft tissue sarcoma in adolescent and young adult patients using the nationwide Bone and Soft Tissue Tumor registry. Adolescent and young adult age is not a prognostic factor for poor cancer survival among those with soft tissue sarcoma in Japan.


Assuntos
Sarcoma/epidemiologia , Neoplasias de Tecidos Moles/epidemiologia , Adolescente , Idoso , Feminino , Humanos , Japão/epidemiologia , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Prognóstico , Sistema de Registros , Estudos Retrospectivos , Adulto Jovem
9.
Pediatr Hematol Oncol ; 38(3): 251-264, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33616477

RESUMO

While sarcoma immunology has advanced with regard to basic, and even some applied topics, this disease has not been subject to more recent immunogenomics approaches. Thus, we assessed the immune receptor recombinations available from the cancer genome atlas (TCGA) sarcoma database via tumor sample exome and RNASeq files. Results indicated that recovery of T-cell receptor-alpha recombination reads (TRA) correlated with a better survival rate, with the expression of T-cell biomarkers, and with tumor sample apoptosis signatures consistent with the longer patient survival times. Furthermore, samples representing TRA complementarity determining region-3 (CDR3) net charge per residue (NCPR) based complementarity with the corresponding sarcoma mutanome had a better survival rate, and more granzyme expression, than samples lacking such complementarity. By specifically using RNASeq-recovered TRA CDR3s and related NCPR assessments, three genes, TP53, ATRX, and RB1, were identified as being key components of the mutanome-based complementarity. Thus, these genes may represent key immune system targets for soft tissue sarcomas. Also, several key results from above were reproduced with a pediatric osteosarcoma dataset, work that led to identification of MUC6 mutations as potentially linked to a strong immune response. In sum, TRA CDR3s are likely to be important prognostic indicators, and possibly a beginning tool for immunotherapy development strategies, for adult and pediatric sarcomas.


Assuntos
Regiões Determinantes de Complementaridade/genética , Receptores de Antígenos de Linfócitos T alfa-beta/genética , Sarcoma/genética , Aminoácidos/genética , Criança , Regiões Determinantes de Complementaridade/química , Exoma , Humanos , Estimativa de Kaplan-Meier , Mutação , Receptores de Antígenos de Linfócitos T alfa-beta/química , Sarcoma/epidemiologia , Eletricidade Estática , Taxa de Sobrevida
10.
Ann Surg Oncol ; 28(11): 6852-6860, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33538930

RESUMO

Soft tissue sarcomas arising in visceral organs are rare and lack validated tumor-staging protocols. Clinicopathologic features and clinical outcomes of 2698 visceral sarcomas identified in the Surveillance, Epidemiology, and End Results Program (SEER) database were compared with sarcomas arising in the extremities/trunk (n = 10,237) or retroperitoneum (n = 1067) using standard statistical techniques. Important prognostic criteria for visceral sarcomas, as in other anatomic sites, included tumor size, histologic grade, and presence of metastatic disease. After adjustment for pertinent confounding factors, visceral sarcomas showed cancer-specific survival rates similar to those arising in the retroperitoneum but had worse outcomes than sarcomas in the extremities/trunk. Therefore, the prognostic performance of two different staging algorithms for retroperitoneal sarcomas was evaluated for their use in staging sarcomas of visceral organs. The current AJCC 8th edition and the recently derived Vanderbilt system for staging retroperitoneal sarcoma both showed adequate discrimination, as assessed by multiple clinical concordance indices, and no evidence of miscalibration. Therefore, the authors concluded that previously validated staging systems for retroperitoneal sarcomas based on conventional prognostic factors (histologic grade, tumor size, and presence of metastatic disease) are applicable to visceral sarcomas and should be incorporated into the next edition of the AJCC Cancer Staging Manual.


Assuntos
Neoplasias Retroperitoneais , Sarcoma , Neoplasias de Tecidos Moles , Humanos , Estadiamento de Neoplasias , Prognóstico , Neoplasias Retroperitoneais/epidemiologia , Neoplasias Retroperitoneais/patologia , Medição de Risco , Programa de SEER , Sarcoma/epidemiologia , Sarcoma/patologia , Neoplasias de Tecidos Moles/patologia
11.
JAMA Netw Open ; 4(2): e210112, 2021 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-33630087

RESUMO

Importance: Genetic disorders are historically defined through phenotype-first approaches. However, risk estimates derived from phenotype-linked ascertainment may overestimate severity and penetrance. Pathogenic variants in DICER1 are associated with increased risks of rare and common neoplasms and thyroid disease in adults and children. This study explored how effectively a genome-first approach could characterize the clinical traits associated with germline DICER1 putative loss-of-function (pLOF) variants in an unselected clinical cohort. Objective: To examine the prevalence, penetrance, and phenotypic characteristics of carriers of germline DICER1 pLOF variants via genome-first ascertainment. Design, Setting, and Participants: This cohort study classifies DICER1 variants in germline exome sequence data from 92 296 participants of the Geisinger MyCode Community Health Initiative. Data for each MyCode participant were used from the start of the Geisinger electronic health record to February 1, 2018. Main Outcomes and Measures: Prevalence of germline DICER1 variation; penetrance of malignant tumors and thyroid disease in carriers of germline DICER1 variation; structured, manual review of electronic health records; and DICER1 sequencing of available tumors from an associated cancer registry. Results: A total of 92 296 adults (mean [SD] age, 59 [18] years; 98% white; 60% female) participated in the study. Germline DICER1 pLOF variants were observed in 1 in 3700 to 1 in 4600 participants, more than double the expected prevalence. Malignant tumors (primarily thyroid carcinoma) were observed in 4 of 25 participants (16%) with DICER1 pLOF variants, which is comparable (by 50 years of age) to the frequency of neoplasms in the largest registry- and clinic-based (phenotype-first) DICER1 studies published to date. DICER1 pLOF variants were significantly associated with risks of thyroidectomy (odds ratio [OR], 6.0; 95% CI, 2.2-16.3; P = .007) and thyroid cancer (OR, 9.2; 95% CI, 2.1-34.7; P = .02) compared with controls, but there was not a significant increase in the risk of goiter (OR, 1.8; 95% CI, 0.7-4.9). A female patient in her 80s who was a carrier of a germline DICER1 hotspot variant was apparently healthy on electronic health record review. The term DICER1 did not appear in any of the medical records of the 25 participants with a pLOF DICER1 variant, even in those affected with a known DICER1-associated tumor or thyroid phenotype. Conclusions and Relevance: This cohort study was able to ascertain individuals with germline DICER1 variants based on a genome-first approach rather than through a previously established DICER1-related phenotype. Use of the genome-first approach may complement more traditional approaches to syndrome delineation and may be an efficient approach for risk estimation.


Assuntos
RNA Helicases DEAD-box/genética , Penetrância , Fenótipo , Ribonuclease III/genética , Doenças da Glândula Tireoide/genética , Neoplasias da Glândula Tireoide/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Genoma , Mutação em Linhagem Germinativa , Bócio Nodular/epidemiologia , Bócio Nodular/genética , Doença de Graves/epidemiologia , Doença de Graves/genética , Heterozigoto , Humanos , Hipotireoidismo/epidemiologia , Hipotireoidismo/genética , Neoplasias Renais/epidemiologia , Neoplasias Renais/genética , Mutação com Perda de Função , Masculino , Pessoa de Meia-Idade , Neoplasias/epidemiologia , Neoplasias/genética , Neoplasias Ovarianas/epidemiologia , Neoplasias Ovarianas/genética , Prevalência , Blastoma Pulmonar/epidemiologia , Blastoma Pulmonar/genética , Sarcoma/epidemiologia , Sarcoma/genética , Tumor de Células de Sertoli-Leydig/epidemiologia , Tumor de Células de Sertoli-Leydig/genética , Tumores do Estroma Gonadal e dos Cordões Sexuais/epidemiologia , Tumores do Estroma Gonadal e dos Cordões Sexuais/genética , Neoplasias Testiculares/epidemiologia , Neoplasias Testiculares/genética , Doenças da Glândula Tireoide/epidemiologia , Neoplasias da Glândula Tireoide/epidemiologia , Nódulo da Glândula Tireoide/epidemiologia , Nódulo da Glândula Tireoide/genética , Tireoidectomia/estatística & dados numéricos , Tireotoxicose/epidemiologia , Tireotoxicose/genética , Tumor de Wilms/epidemiologia , Tumor de Wilms/genética , Adulto Jovem
12.
PLoS One ; 16(2): e0246958, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33630918

RESUMO

BACKGROUND: Since 2010, nationwide networks of reference centers for sarcomas (RREPS/NETSARC/RESOS) collected and prospectively reviewed all cases of sarcomas and connective tumors of intermediate malignancy (TIM) in France. METHODS: The nationwide incidence of sarcoma or TIM (2013-2016) was measured using the 2013 WHO classification and confirmed by a second independent review by expert pathologists. Simple clinical characteristics, yearly variations and correlation of incidence with published clinical trials are presented and analyzed. RESULTS: Over 150 different histological subtypes are reported from the 25172 patients with sarcomas (n = 18712, 74,3%) or TIM (n = 6460, 25.7%), with n = 5838, n = 6153, n = 6654, and n = 6527 yearly cases from 2013 to 2016. Over these 4 years, the yearly incidence of sarcomas and TIM was therefore 70.7 and 24.4 respectively, with a combined incidence of 95.1/106/year, higher than previously reported. GIST, liposarcoma, leiomyosarcomas, undifferentiated sarcomas represented 13%, 13%, 11% and 11% of tumors. Only GIST, as a single entity had a yearly incidence above 10/106/year. There were respectively 30, 64 and 66 different histological subtypes of sarcomas or TIM with an incidence ranging from 10 to 1/106, 1-0.1/106, or < 0.1/106/year respectively. The 2 latter incidence groups represented 21% of the patients with 130 histotypes. Published phase III and phase II clinical trials (p<10-6) are significantly higher with sarcomas subtypes with an incidence above 1/106 per. CONCLUSIONS: This nationwide registry of sarcoma patients, with exhaustive histology review by sarcoma experts, shows that the incidence of sarcoma and TIM is higher than reported, and that tumors with a very low incidence (1<106/year) are less likely to be included in clinical trials.


Assuntos
Sarcoma/epidemiologia , Sarcoma/patologia , Adolescente , Adulto , Idoso , Feminino , França/epidemiologia , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Estudos Prospectivos , Sarcoma/classificação , Sarcoma/diagnóstico , Organização Mundial da Saúde , Adulto Jovem
13.
Cancer Radiother ; 25(1): 21-25, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33384223

RESUMO

PURPOSE: The aim of this study was to better understand the incidence and the clinical characteristics of cardiac radiation-induced sarcomas (RIS). MATERIAL AND METHODS: We used the surveillance, epidemiology, and end results (SEER) program cancer registry data, the largest cancer database in the United States in order to identify all cardiac RIS between 1973 and 2015. We relied on the Memorial Sloan-Kettering Cancer Center (MSKCC)-modified 1948 Cahan criterions for RIS identification. RESULTS: Out of 8,136,951 cancer patients from the SEER database, we identified 448 patients diagnosed with cardiac sarcomas. Of these 448 cardiac sarcoma patients, two were considered to have developed a cardiac RIS: a metastatic rhabdomyosarcoma occurring after one to two years following lung carcinoma irradiation, and a soft tissue sarcoma (of unspecified type) developed six years after radiation therapy for an aggressive left-sided breast carcinoma. Based on this observation, we estimated that cardiac RIS represented about 0.4% (95% CI 0.1%-1.6%) of all cardiac sarcomas. A literature review has been conducted and yielded three additional cases of cardiac RIS. CONCLUSION: Cardiac RIS are extremely rare malignancies, associated with a very pejorative prognosis. The two reported histologies are angiosarcomas and rhabdomyosarcomas, which might be over-represented among cardiac RIS. A metastatic evolution is possible for cardiac radiation-induced rhabdomyosarcomas. Surgical excision, when feasible, is a therapeutic option and is the only specific treatment reported to this date.


Assuntos
Neoplasias Cardíacas/epidemiologia , Neoplasias Induzidas por Radiação/epidemiologia , Programa de SEER/estatística & dados numéricos , Sarcoma/epidemiologia , Feminino , Hemangiossarcoma , Humanos , Incidência , Neoplasias Pulmonares/radioterapia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Rabdomiossarcoma/epidemiologia , Neoplasias Unilaterais da Mama/radioterapia , Estados Unidos/epidemiologia
14.
J Am Vet Med Assoc ; 258(2): 179-185, 2021 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-33405989

RESUMO

OBJECTIVE: To investigate the prevalence of pulmonary nodules suggestive of metastasis at the time of initial presentation in dogs with cutaneous or subcutaneous soft tissue sarcomas (STSs) and no previous related thoracic diagnostic imaging. ANIMALS: 146 client-owned dogs with a cutaneous or subcutaneous STS. PROCEDURES: Medical records were retrospectively searched to identify dogs with STSs that underwent initial thoracic diagnostic imaging when presented for referral examination between September 2014 and March 2018. Data collected included patient and tumor characteristics. Results were evaluated for dogs grouped on the basis of variables of interest (eg, STS grade, duration, or history). RESULTS: Initial thoracic imaging was performed with CT (131/146 [89.7%]) or radiography (15 [10.3%]). Although the presence or absence of pulmonary nodules suggestive of metastasis on thoracic imaging was uncertain in 9 dogs, it was certain in the remaining 137 dogs, with nodules present in 16 (11.7%) dogs (5/77 [6%] with grade 1 STSs, 2/36 [6%] with grade 2 STSs, and 9/24 [38%] with grade 3 STSs). The odds of such pulmonary nodules being present on initial examination were higher (OR, 10.8 and 3.14, respectively) for dogs with grade 3 STSs (vs grade 1 or 2 STSs) and for dogs with an STS duration > 3 months (versus ≤ 3 months). CONCLUSIONS AND CLINICAL RELEVANCE: Results indicated that pulmonary staging was a low-yield diagnostic procedure for dogs with grade 1 or 2 cutaneous or subcutaneous STSs, especially when tumors had been present for ≤ 3 months.


Assuntos
Doenças do Cão , Sarcoma , Neoplasias de Tecidos Moles , Administração Cutânea , Animais , Doenças do Cão/diagnóstico por imagem , Doenças do Cão/epidemiologia , Cães , Prevalência , Estudos Retrospectivos , Sarcoma/epidemiologia , Sarcoma/veterinária , Neoplasias de Tecidos Moles/epidemiologia , Neoplasias de Tecidos Moles/veterinária
15.
Exp Mol Pathol ; 118: 104603, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33422488

RESUMO

The aim of this study was to assess the association between NF1 and PTEN gene polymorphisms and the risk of soft tissue sarcomas (STSs). This case-control study collected peripheral blood from 136 patients with STSs and 124 healthy controls. Six single nucleotide polymorphisms (SNPs) of the NF1 gene and five SNPs of the PTEN gene were investigated and genotyped using the SNaPshot assay. The association between the polymorphisms and the risk of STSs was estimated using unconditional logistic regression analysis. The results showed that individuals with the TC/CC genotype for NF1 rs2905789 displayed a significantly increased risk of STSs compared with individuals with wild-type TT (OR = 1.702, 95% CI = 1.002-2.890, P = 0.049). There were no significant differences in the distribution of the genotype or the allele frequencies of the polymorphisms of the NF1 and PTEN genes between the STSs patients and the controls in a Chinese population. Therefore, this study's results suggest that individuals carrying the TC/CC genotype for NF1 rs2905789 may be susceptible to STSs.


Assuntos
Grupo com Ancestrais do Continente Asiático/genética , Biomarcadores Tumorais/genética , Predisposição Genética para Doença , Neurofibromina 1/genética , PTEN Fosfo-Hidrolase/genética , Polimorfismo de Nucleotídeo Único , Sarcoma/epidemiologia , Adulto , Estudos de Casos e Controles , China/epidemiologia , Feminino , Seguimentos , Frequência do Gene , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Sarcoma/genética , Sarcoma/patologia
16.
Laryngoscope ; 131(5): E1468-E1475, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-32946597

RESUMO

OBJECTIVES: To evaluate the incidence of histopathologic diagnostic discrepancy for patients referred to our institution, identify pathologies susceptible to diagnostic error, and assess the impact on survival of histopathologic diagnostic discrepancies. METHODS: Three hundred ninety-seven patients with sinonasal cancers were identified, and discordance between the outside pathologic report and MD Anderson Cancer Center pathologic report was assessed. Overall survival and disease-specific survival were analyzed using Kaplan-Meier and log rank methods. RESULTS: Discordance of major histopathologic diagnoses was present in 24% (97 of 397) of reports, with sinonasal undifferentiated carcinoma, sarcoma, neuroendocrine carcinoma, and poorly differentiated carcinoma pathologies having the highest change in diagnosis (P < .01). A further 61% (244 of 397) had minor changes such as histologic grade, subtype, or stage, with sarcoma and neuroendocrine carcinoma pathologies being most susceptible to change (P < .02). Overall, the 5-year overall survival (OS) and disease-specific survival (DSS) was reduced in patients with a major change in histopathologic diagnosis (59.2% vs. 70.2% (P = .02) and 72.9% vs. 81.2% (P = .02), respectively). Furthermore, patients with a major change in diagnosis and prior treatment experienced a significant reduction in 5-year OS (61.9% vs. 70.4%, P = .03 < .01) and DSS (72.4% vs. 81.5%, P = .04). CONCLUSION: Histopathological diagnosis of sinonasal tumors is complex and challenging given the rarity of the disease. Obtaining the correct diagnosis is important for treatment selection and survival. In histologies prone to misdiagnoses, obtaining a second opinion from experienced head and neck pathologists at a high-volume institution may potentially lead to a change in treatment recommendations that could result in improved survival in patients with sinonasal malignancies. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E1468-E1475, 2021.


Assuntos
Carcinoma Neuroendócrino/diagnóstico , Carcinoma/diagnóstico , Erros de Diagnóstico/estatística & dados numéricos , Neoplasias do Seio Maxilar/diagnóstico , Neoplasias dos Seios Paranasais/diagnóstico , Seios Paranasais/patologia , Sarcoma/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma/epidemiologia , Carcinoma/patologia , Carcinoma/terapia , Carcinoma Neuroendócrino/epidemiologia , Carcinoma Neuroendócrino/patologia , Carcinoma Neuroendócrino/terapia , Criança , Pré-Escolar , Erros de Diagnóstico/prevenção & controle , Feminino , Humanos , Incidência , Estimativa de Kaplan-Meier , Masculino , Neoplasias do Seio Maxilar/epidemiologia , Neoplasias do Seio Maxilar/patologia , Neoplasias do Seio Maxilar/terapia , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Neoplasias dos Seios Paranasais/epidemiologia , Neoplasias dos Seios Paranasais/patologia , Neoplasias dos Seios Paranasais/terapia , Encaminhamento e Consulta/estatística & dados numéricos , Estudos Retrospectivos , Sarcoma/epidemiologia , Sarcoma/patologia , Sarcoma/terapia , Adulto Jovem
17.
Pediatr Blood Cancer ; 68(2): e28754, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33034155

RESUMO

INTRODUCTION: The aim of this study is to evaluate demographics, clinical data, and survival rates of children with cancer over 22 years, and to compare the outcomes, before and after a national health reform was performed. MATERIAL AND METHODS: Files of patients, aged 0-19 years, diagnosed with cancer at the Istanbul University Oncology Institute during 1990-2012 were evaluated retrospectively. RESULTS: The mean age at diagnosis of 2413 patients was 7.5 ± 5.1 years (range 3 days to 19 years). Male/female ratio was 1.26. After 2002, the number of patients diagnosed at a localized/low-risk stage compared to advanced stage significantly increased (60.7% vs 65.1%, P = .03). Comparing the period before 2002 to after 2002, a lower percentage of patients were diagnosed with advanced stage non-Hodgkin lymphoma (62.1% vs 45.1%, P = .03), retinoblastoma (9.5% vs 1.4%, P = .005), soft tissue sarcomas (52.1% vs 38.3%, P = .01), neuroblastoma (82.4% vs 56.2%, P = .005), and carcinomas (72.9% vs 65.4%, P = .04) after 2002. The 5-year survival rate of all patients during the entire period was 74.4%. The survival rate significantly increased for non-Hodgkin lymphoma (63.7% vs 91.8%, P < .0001), neuroblastoma (46.8% vs 70.5%, P = .025), and renal tumors (70% vs 92.3%, P = .013) after 2002. CONCLUSIONS: The increase in patients diagnosed at a localized/low-risk stage and the increase in survival of some types of cancer over years is promising. The national health care reform, enabling patients to easily access free health services, increased awareness, improvement in oncological treatment, and supportive care may have contributed to the progress achieved, and may be a model for other developing countries.


Assuntos
Carcinoma/epidemiologia , Linfoma não Hodgkin/epidemiologia , Neuroblastoma/epidemiologia , Retinoblastoma/epidemiologia , Sarcoma/epidemiologia , Adolescente , Carcinoma/mortalidade , Carcinoma/terapia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Linfoma não Hodgkin/mortalidade , Linfoma não Hodgkin/terapia , Masculino , Neuroblastoma/mortalidade , Neuroblastoma/terapia , Retinoblastoma/mortalidade , Retinoblastoma/terapia , Estudos Retrospectivos , Sarcoma/mortalidade , Sarcoma/terapia , Turquia/epidemiologia , Adulto Jovem
18.
Cancer Epidemiol ; 70: 101857, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33249363

RESUMO

BACKGROUND: Previous studies have noted the incidence of radiation-induced sarcomas (RIS) but have not investigated the relative risk (RR) of developing RIS based on primary tumor organ disease site. By examining data from the Surveillance, Epidemiology, and End Results (SEER) database, we hypothesized that breast cancer would have a higher incidence of RIS compared to seventeen other primary cancer sites. METHODS: This was a retrospective cohort study that examined patients from SEER registries between 1973 and 2013. We included patients aged 18 years or older who were diagnosed with cancer and those diagnosed with a cancer who subsequently developed a sarcoma. We excluded patients with missing information on initial radiotherapy treatment or stage. RIS was defined as those who developed a secondary sarcoma near the site of their original malignancy and after a 24-month latency period. RESULTS: Our patients had a mean age of 60 years and follow up time of 9.2 years. Breast cancer comprised the majority with 693,701(36.8%) patients of which 161 (0.02%) had a secondary sarcoma. Of the 359 patients with secondary sarcomas, 242 (67.4%) had RIS. Breast cancer had the highest number of RIS patients at 126 compared to all combined non-breast cancer sites at 116. The RR of RIS in breast cancer versus 19 other primary cancer sites was 1.21 (CI: 1.01-1.45, p < 0.03, adjusted for age at primary diagnosis, gender, and latency). CONCLUSIONS: Our study demonstrated that breast cancer has a higher risk of developing RIS compared to other solid cancers.


Assuntos
Neoplasias Induzidas por Radiação/epidemiologia , Sarcoma/epidemiologia , Estudos de Coortes , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Sistema de Registros , Estudos Retrospectivos , Programa de SEER
19.
Adv Ther ; 38(1): 90-108, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33184778

RESUMO

INTRODUCTION: As a result of the increased use of radiotherapy (RT) and improved long-term overall survival of patients with cancers of the head and neck (HN), the frequency of radiation-induced sarcomas of the head and neck (RISHN) may be increasing. The main objective of this systematic review was to determine the existing evidence on the frequency, treatment, and outcome of RISHN. METHODS: Using PRISMA guidelines we conducted a systematic review of the literature published from 2000 to 2020. RESULTS: Our review includes data of 560 patients from 64 articles. The total frequency of RISHNs among the reviewed series was 0.15%. The most frequent location of the primary tumor treated by RT was the nasopharynx. The mean RT dose used was 62 Gy, mean latency interval between irradiation and occurrence of RISHN was 11.1 years, and the most common RISHN location was the sinonasal region. Osteosarcoma was the principal histology, followed by fibrosarcoma. Surgery was the most frequently applied treatment modality. Of all patients with RISHN, 40.7% died of this disease after a mean interval of 13.9 months. CONCLUSIONS: Notwithstanding the increased use of RT, the number of reported RISHNs has not increased substantially during the past two decades. Surgery with wide margins forms the best therapeutic option for these cases, but the outcome remains poor.


Assuntos
Neoplasias Ósseas , Neoplasias de Cabeça e Pescoço , Osteossarcoma , Sarcoma , Neoplasias de Cabeça e Pescoço/radioterapia , Humanos , Estudos Retrospectivos , Sarcoma/epidemiologia , Sarcoma/etiologia
20.
Jpn J Clin Oncol ; 51(4): 612-621, 2021 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-33283234

RESUMO

BACKGROUND: This study evaluated the safety and efficacy of pazopanib in patients with metastatic soft tissue sarcoma in routine clinical use in Japan. METHODS: It was a multicentre, centrally registered and uncontrolled observational study in patients who received pazopanib for metastatic soft tissue sarcoma, with an observation period of 1 year after the start of drug administration. The study was conducted at 378 investigational sites in Japan from September 2012 to September 2019. Progression-free survival (PFS) and overall survival (OS) were the efficacy endpoints of the study. RESULTS: A total of 1970 patients were enrolled. Of these, 680 with finalized study forms were included in the analysis. Overall, 649 patients were included in the safety analysis set, and 569 were included in the efficacy analysis set. Most of the patients (81.97%) experienced at least one adverse drug reaction (ADR); 22.34% of patients reported serious ADRs and 34.98% of patients experienced grade ≥ 3 ADRs in the safety set. Hypertension (40.37%) and hepatic dysfunction (26.50%) were the two most common ADRs. A total of 262 deaths were reported, of which 12 were due to ADRs. The median PFS was 3.09 months, whereas the median OS was not reached at the end of the 1-year observation period. CONCLUSIONS: The safety and efficacy profiles in this postmarketing observational study were consistent with prior data and registration clinical trials. No new safety signals were observed while treating patients with metastatic soft tissue sarcoma with pazopanib.


Assuntos
Vigilância de Produtos Comercializados , Pirimidinas/uso terapêutico , Sarcoma/tratamento farmacológico , Neoplasias de Tecidos Moles/tratamento farmacológico , Sulfonamidas/uso terapêutico , Adolescente , Adulto , Idoso , Feminino , Humanos , Incidência , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Intervalo Livre de Progressão , Pirimidinas/administração & dosagem , Pirimidinas/efeitos adversos , Pirimidinas/farmacologia , Sarcoma/epidemiologia , Sarcoma/patologia , Neoplasias de Tecidos Moles/epidemiologia , Neoplasias de Tecidos Moles/patologia , Sulfonamidas/administração & dosagem , Sulfonamidas/efeitos adversos , Sulfonamidas/farmacologia , Resultado do Tratamento , Adulto Jovem
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