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1.
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
2.
Hum Genet ; 140(8): 1241-1252, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34059954

RESUMO

Malignant peripheral nerve sheath tumors (MPNST) are aggressive soft tissue sarcomas with poor prognosis, developing either sporadically or in persons with neurofibromatosis type 1 (NF1). Loss of CDKN2A/B is an important early event in MPNST progression. However, many reported MPNSTs exhibit partial or no inactivation of CDKN2A/B, raising the question of whether there is more than one molecular path for MPNST initiation. We present here a comprehensive genomic analysis of MPNST cell lines and tumors to explore in depth the status of CDKN2A. After accounting for CDKN2A deletions and point mutations, we uncovered a previously unnoticed high frequency of chromosomal translocations involving CDKN2A in both MPNST cell lines and primary tumors. Most identified translocation breakpoints were validated by PCR amplification and Sanger sequencing. Many breakpoints clustered in an intronic 500 bp hotspot region adjacent to CDKN2A exon 2. We demonstrate the bi-allelic inactivation of CDKN2A in all tumors (n = 15) and cell lines (n = 8) analyzed, supporting a single molecular path for MPNST initiation in both sporadic and NF1-related MPNSTs. This general CDKN2A inactivation in MPNSTs has implications for MPNST diagnostics and treatment. Our findings might be relevant for other tumor types with high frequencies of CDKN2A inactivation.


Assuntos
Carcinogênese/genética , Inibidor p16 de Quinase Dependente de Ciclina/genética , Neurofibromatose 1/genética , Neurofibrossarcoma/genética , Polimorfismo de Nucleotídeo Único , Sarcoma/genética , Translocação Genética , Sequência de Bases , Carcinogênese/metabolismo , Carcinogênese/patologia , Linhagem Celular Tumoral , Cromossomos Humanos Par 9 , Inibidor p16 de Quinase Dependente de Ciclina/deficiência , Éxons , Genoma Humano , Humanos , Neurofibromatose 1/complicações , Neurofibromatose 1/metabolismo , Neurofibromatose 1/patologia , Neurofibrossarcoma/etiologia , Neurofibrossarcoma/metabolismo , Neurofibrossarcoma/patologia , Sarcoma/etiologia , Sarcoma/metabolismo , Sarcoma/patologia , Células de Schwann/metabolismo , Células de Schwann/patologia , Sequenciamento Completo do Genoma
3.
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
4.
JAMA Netw Open ; 3(10): e2013929, 2020 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-33006617

RESUMO

Importance: The association of radiation and chemotherapy with the development of secondary sarcoma is known, but the contemporary risk has not been well characterized for patients with cancers of the abdomen and pelvis. Objective: To compare the risk of secondary sarcoma among patients treated with combinations of surgery, radiation, or chemotherapy with patients treated with surgery alone and the general population. Design, Setting, and Participants: This population-based cohort study included 173 580 patients in Ontario, Canada, with nonmetastatic cancer of the prostate, bladder, colon, rectum or anus, cervix, uterus, or testis. Patients were enrolled from January 1, 2002, to January 31, 2017. Data analysis was conducted from March 1, 2019, to January 31, 2020. Exposures: Treatment combinations of radiation, chemotherapy, and surgery. Main Outcome and Measures: Diagnosis of sarcoma based on histologic codes from the Ontario Cancer Registry. Time to sarcoma was compared using a cause-specific proportional hazard model. Results: Of 173 580 patients, most were men (125 080 [72.1%]), and the largest group was aged between 60 and 69 years (58 346 [33.6%]). Most patients had genitourinary cancer (86 235 [51.4%]) or colorectal cancer (69 241 [39.9%]). Overall, 64 301 (37.1%) received surgery alone, 51 220 (29.5%) received radiation alone, 15 624 (9.0%) were treated with radiation and chemotherapy, 15 252 (8.8%) received radiation with surgery, and 11 822 (6.8%) received all 3 treatments. A total of 332 patients (0.2%) had sarcomas develop during a median (interquartile range) follow-up of 5.7 (2.2-8.9) years. The incidence of sarcoma was 0.3% among those who underwent radiation alone (138 of 51 220) and radiation with chemotherapy (40 of 15 624), 0.2% among those who received radiation and surgery (36 of 15 252) and all 3 modalities (25 of 11 822), and 0.1% among those who received surgery with chemotherapy (13 of 14 861) and surgery alone (80 of 64 801). Compared with a reference group of patients who had surgery alone, the greatest risk of sarcoma was found among patients who underwent a combination of radiation and chemotherapy (cause-specific relative hazard [csRH], 4.07; 95% CI, 2.75-6.01; P < .001), followed by patients who had radiation alone (csRH, 2.35; 95% CI, 1.77-3.12; P < .001), radiation with surgery (csRH, 2.33; 95% CI, 1.57-3.46; P < .001), and all 3 modalities (csRH, 2.27; 95% CI, 1.44-3.58; P < .001). In the general population, 7987 events occurred during 46 554 803 person-years (17.2 events per 100 000 person-years). The standardized incidence ratio for sarcoma among patients treated with radiation compared with the general population was 2.41 (95% CI, 1.57-3.69; 41.3 events per 100 000 person-years). The annual number of cases of sarcoma increased from 2009 (15 per 100 000 persons) to 2016 (32 per 100 000 persons), but the annual rate did not change during the study period. Conclusions and Relevance: In this cohort study, patients treated with radiation or chemotherapy for abdominopelvic cancers had an increased rate of sarcoma. Although the absolute rate is low, patients and physicians should be aware of this increased risk of developing sarcoma.


Assuntos
Neoplasias Abdominais/tratamento farmacológico , Neoplasias Abdominais/radioterapia , Neoplasias Abdominais/cirurgia , Segunda Neoplasia Primária/etiologia , Neoplasias Pélvicas/tratamento farmacológico , Neoplasias Pélvicas/radioterapia , Neoplasias Pélvicas/cirurgia , Sarcoma/etiologia , Neoplasias Abdominais/complicações , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Ontário/epidemiologia , Neoplasias Pélvicas/complicações , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
5.
JBJS Case Connect ; 10(3): e1900577, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32910603

RESUMO

CASE: A 78-year-old man was followed for an incidentally found, asymptomatic lesion in his right proximal femur that was unchanged radiographically for 11 years. He developed pain and was believed to have experienced a stress fracture through the lesion. The lesion was biopsied, showing a high-grade pleomorphic sarcoma with an underlying senescent intraosseous lipoma. He was ultimately treated with wide excision and reconstruction of the proximal femur. CONCLUSION: This case highlights the importance of obtaining a tissue diagnosis for lesions that become symptomatic.


Assuntos
Neoplasias Femorais/patologia , Fêmur/patologia , Lipoma/complicações , Sarcoma/etiologia , Idoso , Neoplasias Femorais/diagnóstico por imagem , Fêmur/diagnóstico por imagem , Humanos , Lipoma/diagnóstico por imagem , Lipoma/patologia , Imageamento por Ressonância Magnética , Masculino , Sarcoma/diagnóstico por imagem , Sarcoma/patologia
6.
Breast Cancer Res Treat ; 183(3): 669-675, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32696318

RESUMO

PURPOSE: Sarcomas of the breast account for about 1% of all breast malignancies. The aim of this national survey was to explore etiologic and prognostic factors. METHODS: Utilizing national Swedish registers, all patients registered with mesenchymal tumors in the breast during the period 1993-2013 (n = 344) were identified and compared to up to ten age and gender matched controls. Cancer history was retrieved for cases and controls. Conditional Poisson regression models were used for calculation of odds ratios. RESULTS: Previous breast cancer was overrepresented among patients with angiosarcoma. The highest risk occurred ≥ 5 years after treatment for breast cancer (OR 73.9, 95% confidence interval, CI, 25.4-215; P < 0.001). An increase in incidence of angiosarcoma was observed during the study period (1.10, 95% CI 1.05-1.16; P < 0.001). The overall incidence of breast sarcoma increased from 1.52 to 2.04 cases per million per year. Angiosarcoma of the breast was associated with a significant excess mortality compared to age-matched controls (HR 4.65, 95% CI 3.01-7.19; P < 0.001). CONCLUSIONS: Angiosarcoma increased in incidence and displayed a more severe clinical course, with significantly shorter survival. The strong association between a history of breast cancer 5 years or more prior to the diagnosis of angiosarcoma points to radiotherapy as a contributing factor.


Assuntos
Neoplasias da Mama , Neoplasias Induzidas por Radiação , Sarcoma , Neoplasias da Mama/epidemiologia , Neoplasias da Mama/etiologia , Estudos de Casos e Controles , Feminino , Humanos , Prognóstico , Sarcoma/epidemiologia , Sarcoma/etiologia , Suécia/epidemiologia
7.
Rev Med Interne ; 41(8): 559-561, 2020 Aug.
Artigo em Francês | MEDLINE | ID: mdl-32712043

RESUMO

INTRODUCTION: Extramedullary hematopoiesis is a complication of myeloproliferative neoplasms or of chronic hemolysis. The more frequent localizations are splenic, ganglionic or paraspinal. Rarely, extramedullary hematopoiesis is associated with solid cancer. CASE REPORT: We report an original case of sarcoma located in an extramedullary hematopoiesis mass in a 72-year-old woman suffering from hereditary spherocytosis. An asymptomatic right paravertebral mass was found in 2004; the biopsy confirmed extramedullary hematopoiesis. In 2016, the patient was hospitalized due to paravertebral pain. Computed tomography showed the extension of the right paraspinal mass to pleura and mediastinum as well as vertebral bone lysis. Positron emission tomography showed an intense hypermetabolism. The biopsy showed undifferentiated sarcoma. CONCLUSION: This case report illustrates the risk of neoplastic transformation of extramedullary hematopoiesis, and the need for a biopsy when confronted to atypical aspect.


Assuntos
Hematopoese Extramedular/fisiologia , Sarcoma/diagnóstico , Esferocitose Hereditária/complicações , Neoplasias Torácicas/diagnóstico , Idoso , Evolução Fatal , Feminino , Humanos , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/etiologia , Sarcoma/etiologia , Esferocitose Hereditária/diagnóstico , Neoplasias Torácicas/etiologia
8.
Intern Med ; 59(17): 2149-2153, 2020 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-32493854

RESUMO

We herein report a case of pulmonary artery sarcoma (PAS) in a 64-year-old woman. She was admitted to our hospital because of massive genital bleeding from endometrial cancer. Contrast-enhanced computed tomography (CT) revealed a left pulmonary artery mass and deep vein thrombosis. She underwent anticoagulant therapy for one year. However, the mass lesion gradually expanded. 18F-Fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT showed a positive uptake of FDG by the mass. An endovascular catheter biopsy was performed for the differentiation of endometrial cancer metastasis or primary sarcoma. The biopsy specimen tissue comprised spindle-shaped cells. Thus, the patient was diagnosed with PAS.


Assuntos
Cateterismo/métodos , Neoplasias do Endométrio/complicações , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/cirurgia , Artéria Pulmonar/fisiopatologia , Sarcoma/diagnóstico , Sarcoma/cirurgia , Biópsia/métodos , Procedimentos Endovasculares , Feminino , Humanos , Neoplasias Pulmonares/etiologia , Neoplasias Pulmonares/fisiopatologia , Pessoa de Meia-Idade , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Sarcoma/etiologia , Sarcoma/fisiopatologia , Resultado do Tratamento
9.
Ann Thorac Surg ; 110(6): e469-e471, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-32505704

RESUMO

Keloids are considered as benign fibroproliferative skin tumors, and rare cases of malignancies have been reported. We present a case of low-grade myofibroblastic sarcoma arising from a recurrent painful keloid scar on the right chest wall after video-assisted thoracic surgery for pneumothorax in a 77-year-old man. Wide composite excision of the keloid, surrounding ribs, and partial diaphragm were performed. The chest wall pleural defect was reconstructed with Teflon (Chemours, Wilmington, DE), and soft tissue was reconstructed with a transverse rectus abdominis myocutaneous flap. This case highlights that refractory keloids may be considered a harbinger of malignancy.


Assuntos
Queloide/complicações , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/patologia , Sarcoma/etiologia , Sarcoma/patologia , Neoplasias Cutâneas/etiologia , Neoplasias Cutâneas/patologia , Cirurgia Torácica Vídeoassistida , Idoso , Humanos , Masculino , Gradação de Tumores , Parede Torácica
10.
Blood ; 136(17): 1980-1983, 2020 10 22.
Artigo em Inglês | MEDLINE | ID: mdl-32518951
11.
Future Oncol ; 16(17): 1211-1223, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32396026

RESUMO

Sarcomas are rare and heterogeneous malignant tumors of mesenchymal origin. A total of 25-50% of patients treated with initial curative intent will develop as recurrent and metastatic disease. In the recurrent and metastatic setting, effect of chemotherapy is limited; therefore, more effective therapies are urgently desired. As a brake for activation of T cell, PD-1/PD-L1 plays a crucial role in the progression of tumor by altering status of immune surveillance. Some success has been acquired recently in the use of PD-1/PD-L1 inhibitors for the treatment of several solid tumors, for examples, non-small-cell lung cancer and melanoma. Immunotherapeutic strategies based on PD-1/PD-L1 for sarcomas have also been explored these years. As in other cancers, major challenges are identification of biomarkers to predict response for immunotherapy, optimization of patient's benefit and minimization of side effects. Therefore, we focused on potential biomarkers of immunotherapy for treatment of sarcomas in this review.


Assuntos
Antineoplásicos Imunológicos/uso terapêutico , Biomarcadores Tumorais , Imunomodulação/efeitos dos fármacos , Sarcoma/tratamento farmacológico , Sarcoma/etiologia , Animais , Antineoplásicos Imunológicos/administração & dosagem , Antineoplásicos Imunológicos/efeitos adversos , Reparo do DNA , Suscetibilidade a Doenças , Humanos , Repetições de Microssatélites , Terapia de Alvo Molecular , Mutação , Sarcoma/diagnóstico , Microambiente Tumoral/imunologia
12.
Medicina (Kaunas) ; 56(4)2020 Mar 31.
Artigo em Inglês | MEDLINE | ID: mdl-32244416

RESUMO

Radiation-induced sarcoma (RIS) has been reported as a late secondary malignancy following radiotherapy for various types of cancer with a median latency of 10 years. We describe an early RIS that developed in an adolescent within three years of treatment (including PD-L1 check-point inhibitor Nivolumab) of a relapsed classic Hodgkin lymphoma (HL) and was diagnosed post-mortem. The patient died of the progressive RIS that was misleadingly assumed to be a resistant HL based on the positive PET/CT scan. Repetitive tumor biopsies are warranted in cases of aggressive and multi-drug resistant HL to validate imaging findings, ensure correct diagnosis and avoid overtreatment.


Assuntos
Doença de Hodgkin/radioterapia , Neoplasias Induzidas por Radiação/etiologia , Nivolumabe/uso terapêutico , Sarcoma/etiologia , Adolescente , Antineoplásicos Imunológicos/uso terapêutico , Feminino , Doença de Hodgkin/tratamento farmacológico , Humanos , Tomografia por Emissão de Pósitrons/métodos
13.
Int J Mol Sci ; 21(8)2020 Apr 24.
Artigo em Inglês | MEDLINE | ID: mdl-32344731

RESUMO

Sarcomas represent one of the most challenging tumor types to treat due to their diverse nature and our incomplete understanding of their underlying biology. Recent work suggests cyclin-dependent kinase (CDK) pathway activation is a powerful driver of sarcomagenesis. CDK proteins participate in numerous cellular processes required for normal cell function, but their dysregulation is a hallmark of many pathologies including cancer. The contributions and significance of aberrant CDK activity to sarcoma development, however, is only partly understood. Here, we describe what is known about CDK-related alterations in the most common subtypes of sarcoma and highlight areas that warrant further investigation. As disruptions in CDK pathways appear in most, if not all, subtypes of sarcoma, we discuss the history and value of pharmacologically targeting CDKs to combat these tumors. The goals of this review are to (1) assess the prevalence and importance of CDK pathway alterations in sarcomas, (2) highlight the gap in knowledge for certain CDKs in these tumors, and (3) provide insight into studies focused on CDK inhibition for sarcoma treatment. Overall, growing evidence demonstrates a crucial role for activated CDKs in sarcoma development and as important targets for sarcoma therapy.


Assuntos
Quinases Ciclina-Dependentes/metabolismo , Sarcoma/etiologia , Sarcoma/metabolismo , Fatores Etários , Animais , Antineoplásicos/farmacologia , Biomarcadores , Ciclo Celular/efeitos dos fármacos , Quinases Ciclina-Dependentes/antagonistas & inibidores , Suscetibilidade a Doenças , Regulação Neoplásica da Expressão Gênica , Humanos , Modelos Biológicos , Terapia de Alvo Molecular , Inibidores de Proteínas Quinases/farmacologia , Sarcoma/tratamento farmacológico , Sarcoma/patologia , Transdução de Sinais , Transcrição Genética
14.
Head Neck ; 42(9): 2660-2668, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32343457

RESUMO

BACKGROUND: The aim of this study was to integrate the available data published on radiation-induced sarcoma of the oral cavity into an analysis of its clinical features, treatment modalities and prognostic factors. METHODS: An electronic search was undertaken in September 2019. The eligibility criteria included publications that had enough clinical and histological information to confirm the diagnosis. RESULTS: Forty-two publications with 122 radiation-induced sarcoma of the oral cavities (RISOCs) were included. The mean latency period was 114 months and mean radiation total dose was 62.5 Gy. The tumors were more prevalent in males between 50 and 60 years old and the mandible was the most affected site. Osteosarcoma was the most prevalent histological type and patients were mostly treated with radical surgery. CONCLUSIONS: RISOC showed a poor survival rate of 15.1% in 5-year follow-up. Gender and histological type were independently associated with survival.


Assuntos
Neoplasias Ósseas , Neoplasias Induzidas por Radiação , Osteossarcoma , Sarcoma , Neoplasias de Tecidos Moles , Humanos , Masculino , Mandíbula , Pessoa de Meia-Idade , Neoplasias Induzidas por Radiação/epidemiologia , Neoplasias Induzidas por Radiação/etiologia , Sarcoma/epidemiologia , Sarcoma/etiologia , Sarcoma/terapia
16.
Taiwan J Obstet Gynecol ; 59(2): 275-281, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32127150

RESUMO

OBJECTIVE: To evaluate the risk of encountering unexpected uterine smooth muscle tumors of uncertain malignant potential (STUMPs) or sarcomas during surgical treatment of mesenchymal tumors of the uterus using morcellation. MATERIAL AND METHODS: Data were collected retrospectively from subjects who were pathologically diagnosed with uterine leiomyoma or its variants, STUMP or other premalignant mesenchymal tumors of uterus, or sarcoma during surgical treatment between July 2014 and June 2017. RESULTS: A total of 3785 women were investigated; 2824 laparoscopic procedures (74.6%) were performed, and an electronic power morcellator was used in 1636 patients (43.2%). Sixteen women (0.42%) were diagnosed with STUMP and 14 (0.37%) were diagnosed with uterine sarcoma. The incidence rate of unexpected STUMP or uterine sarcoma was 0.61% (23 of 3785 women); unexpected STUMP in 13 (0.34%), and unexpected sarcoma was in 10 (0.26%). Moreover, the unexpected leiomyosarcoma rate was 0.08% (3 in 3785). The rate of unintended morcellation of STUMPs was relatively high at 0.26% (10 in 3785), however, that for uterine sarcomas was 0.05% (2 in 3785). CONCLUSION: The risks of unintended morcellation were very low for sarcomas and STUMPs, although the risk of the latter was approximately 5-fold that of the former. To reduce the unintended dissemination of tumors, patients suspected of having malignancies should be provided adequate information regarding their treatment options as well as their associated risks. Meanwhile, improved preoperative screening methods for STUMP and sarcoma should be established.


Assuntos
Mesenquimoma/cirurgia , Morcelação/efeitos adversos , Complicações Pós-Operatórias/etiologia , Sarcoma/etiologia , Tumor de Músculo Liso/etiologia , Neoplasias Uterinas/cirurgia , Adulto , Feminino , Humanos , Incidência , Leiomiossarcoma/epidemiologia , Leiomiossarcoma/etiologia , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , República da Coreia/epidemiologia , Estudos Retrospectivos , Sarcoma/epidemiologia , Tumor de Músculo Liso/epidemiologia , Neoplasias Uterinas/epidemiologia , Neoplasias Uterinas/etiologia
17.
Semin Oncol ; 47(1): 73-84, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-32201016

RESUMO

Soft-tissue sarcomas (STS) are a group of rare mesenchymal tumors that constitutes ∼1% of all solid tumors. It remains a rare tumor which lacks effective treatment options. Precision oncology may be of interest in this regard by identifying potential targets for emerging novel therapies. Neurotrophic receptor tyrosine kinase (NTRK) fusions are rare oncogenic driver mutations found in a broad range of common and rare tumor subtypes including STS. The recent approvals of NTRK inhibitors (larotrectinib and entrectinib) represent new therapeutic options in the drug armamentarium especially valuable in advanced STS given the paucity of treatment options and the generally poor prognosis of these tumors. We review the methods used to detect NTRK fusions in STS with focus on incidence, diagnosis and management of these rare and intriguing oncogenic targets.


Assuntos
Biomarcadores Tumorais , Inibidores de Proteínas Quinases/farmacologia , Receptores Proteína Tirosina Quinases/antagonistas & inibidores , Sarcoma/tratamento farmacológico , Sarcoma/etiologia , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ensaios Clínicos como Assunto , Resistencia a Medicamentos Antineoplásicos/genética , Testes Genéticos , Humanos , Terapia de Alvo Molecular , Proteínas de Fusão Oncogênica/antagonistas & inibidores , Proteínas de Fusão Oncogênica/genética , Proteínas de Fusão Oncogênica/metabolismo , Inibidores de Proteínas Quinases/uso terapêutico , Receptores Proteína Tirosina Quinases/genética , Receptores Proteína Tirosina Quinases/metabolismo , Sarcoma/diagnóstico , Sarcoma/epidemiologia , Resultado do Tratamento
18.
Br J Cancer ; 122(8): 1250-1259, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32099095

RESUMO

BACKGROUND: This nationwide study investigated associations between paternal occupational exposure and childhood bone tumours and soft- tissue sarcomas. METHODS: The UK National Registry of Childhood Tumours provided cases of childhood sarcomas born and diagnosed in Great Britain, 1962-2010. Control births, unaffected by childhood cancer, were matched on sex, birth period and birth registration sub-district. Fathers' occupations were assigned to one or more of 33 exposure groups and coded for occupational social class. RESULTS: We analysed 5,369 childhood sarcoma cases and 5380 controls. Total bone tumours, total soft-tissue sarcomas and the subgroups osteosarcoma, rhabdomyosarcoma and Ewing Sarcoma Family of Tumours (ESFT) were considered separately. Significant positive associations were seen between rhabdomyosarcoma and paternal exposure to EMFs (odds ratio = 1.67, CI = 1.22-2.28) and also for ESFT and textile dust (1.93, 1.01-3.63). There were putative protective effects on total bone tumours of paternal dermal exposure to hydrocarbons, metal, metal working or oil mists. CONCLUSIONS: Despite the large size and freedom from bias of this study, our results should be interpreted with caution. Many significance tests were undertaken, and chance findings are to be expected. Nevertheless, our finding of associations between ESFT and paternal exposure to textile dust may support related suggestions in the literature.


Assuntos
Neoplasias Ósseas/etiologia , Exposição Ocupacional/efeitos adversos , Exposição Paterna/efeitos adversos , Sarcoma/etiologia , Estudos de Casos e Controles , Criança , Feminino , Humanos , Masculino , Osteossarcoma/etiologia , Rabdomiossarcoma/etiologia , Sarcoma de Ewing/etiologia
20.
Semin Cancer Biol ; 61: 56-70, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31722230

RESUMO

Sarcomas are a rare group of mesenchymal cancers comprising over 70 different histological subtypes. For the majority of these diseases, the molecular understanding of the basis of their initiation and progression remains unclear. As such, limited clinical progress in prognosis or therapeutic regimens have been made over the past few decades. Proteomics techniques are being increasingly utilised in the field of sarcoma research. Proteomic research efforts have thus far focused on histological subtype characterisation for the improvement of biological understanding, as well as for the identification of candidate diagnostic, predictive, and prognostic biomarkers for use in clinic. However, the field itself is in its infancy, and none of these proteomic research findings have been translated into the clinic. In this review, we provide a brief overview of the proteomic strategies that have been employed in sarcoma research. We evaluate key proteomic studies concerning several rare and ultra-rare sarcoma subtypes including, gastrointestinal stromal tumours, osteosarcoma, liposarcoma, leiomyosarcoma, malignant rhabdoid tumours, Ewing sarcoma, myxofibrosarcoma, and alveolar soft part sarcoma. Consequently, we illustrate how routine implementation of proteomics within sarcoma research, integration of proteomics with other molecular profiling data, and incorporation of proteomics into clinical trial studies has the potential to propel the biological and clinical understanding of this group of complex rare cancers moving forward.


Assuntos
Proteoma , Proteômica , Sarcoma/metabolismo , Animais , Antineoplásicos/farmacologia , Antineoplásicos/uso terapêutico , Biomarcadores , Descoberta de Drogas , Humanos , Terapia de Alvo Molecular , Proteômica/métodos , Sarcoma/diagnóstico , Sarcoma/tratamento farmacológico , Sarcoma/etiologia
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