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1.
Am J Clin Oncol ; 43(9): 667-669, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32889838

RESUMO

OBJECTIVE: Surgery followed by postoperative radiation therapy (RT) is the standard of care for soft tissue sarcomas (STS) of the head and neck that are high grade or have close or positive margins. METHODS: The authors retrospectively reviewed adult patients with head and neck STS treated with RT at a single institution between 1981 and 2017. All patients who were 19 years and older with STS of the head and neck-excluding rhabdomyosarcoma, angiosarcoma, and Ewing tumors-were included in this study. Toxicity was graded using Common Terminology Criteria for Adverse Events (CTCAE), version 4. RESULTS: Among 34 patients with head and neck STS treated with postoperative RT (33) or primary RT (1) who met the inclusion criteria, the median age at diagnosis was 45 years (range, 20 to 83). Overall, 37% had T1 tumors, 50% had high-grade histology (grade 3), and 26% had microscopically positive margins. The median RT dose was 65 Gy to the primary site; 29% received elective nodal irradiation. The median follow-up for living patients was 16.6 years (range, 0.6 to 30). At 5 and 10 years, the local control rates were 88% and 80%, the regional control rates were 97% and 97%, the freedom from distant metastases rates were 100% and 100%, the cause-specific survival rates were 88% and 81%, and the overall survival rates were 85% and 69%. Two patients (6%) developed late grade 3+ complications. CONCLUSION: Our study demonstrates that surgery and radiotherapy for STS of the head and neck have excellent disease outcomes.


Assuntos
Neoplasias de Cabeça e Pescoço/radioterapia , Recidiva Local de Neoplasia , Sarcoma/radioterapia , Neoplasias de Tecidos Moles/radioterapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Fracionamento da Dose de Radiação , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Neoplasia Residual , Radioterapia Adjuvante/efeitos adversos , Estudos Retrospectivos , Sarcoma/patologia , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia , Taxa de Sobrevida , Adulto Jovem
2.
Lancet Oncol ; 21(10): 1366-1377, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32941794

RESUMO

BACKGROUND: Unlike for extremity sarcomas, the efficacy of radiotherapy for retroperitoneal sarcoma is not established. The aim of this study was to evaluate the impact of preoperative radiotherapy plus surgery versus surgery alone on abdominal recurrence-free survival. METHODS: EORTC-62092 is an open-label, randomised, phase 3 study done in 31 research institutions, hospitals, and cancer centres in 13 countries in Europe and North America. Adults (aged ≥18 years) with histologically documented, localised, primary retroperitoneal sarcoma that was operable and suitable for radiotherapy, who had not been previously treated and had a WHO performance status and American Society of Anesthesiologists score of 2 or lower, were centrally randomly assigned (1:1), using an interactive web response system and a minimisation algorithm, to receive either surgery alone or preoperative radiotherapy followed by surgery. Randomisation was stratified by hospital and performance status. Radiotherapy was delivered as 50·4 Gy (in 28 daily fractions of 1·8 Gy) in either 3D conformal radiotherapy or intensity modulated radiotherapy, and the objective of surgery was a macroscopically complete resection of the tumour mass with en-bloc organ resection as necessary. The primary endpoint was abdominal recurrence-free survival, as assessed by the investigator, and was analysed in the intention-to-treat population. Safety was analysed in all patients who started their allocated treatment. This trial is registered with ClinicalTrials.gov, NCT01344018. FINDINGS: Between Jan 18, 2012 and April 10, 2017, 266 patients were enrolled, of whom 133 were randomly assigned to each group. The median follow-up was 43·1 months (IQR 28·8-59·2). 128 (96%) patients from the surgery alone group had surgery, and 119 (89%) patients in the radiotherapy and surgery group had both radiotherapy and surgery. Median abdominal recurrence-free survival was 4·5 years (95% CI 3·9 to not estimable) in the radiotherapy plus surgery group and 5·0 years (3·4 to not estimable) in the surgery only group (hazard ratio 1·01, 95% CI 0·71-1·44; log rank p=0·95). The most common grade 3-4 adverse events were lymphopenia (98 [77%] of 127 patients in the radiotherapy plus surgery group vs one [1%] of 128 patients in the surgery alone group), anaemia (15 [12%] vs ten [8%]), and hypoalbuminaemia (15 [12%] vs five [4%]). Serious adverse events were reported in 30 (24%) of 127 patients in the radiotherapy plus surgery group, and in 13 (10%) of 128 patients in the surgery alone group. One (1%) of 127 patients in the radiotherapy plus surgery group died due to treatment-related serious adverse events (gastropleural fistula), and no patients in the surgery alone group died due to treatment-related serious adverse events. INTERPRETATION: Preoperative radiotherapy should not be considered as standard of care treatment for retroperitoneal sarcoma. FUNDING: European Organisation for Research and Treatment of Cancer, and European Clinical Trials in Rare Sarcomas.


Assuntos
Terapia Neoadjuvante , Neoplasias Retroperitoneais/radioterapia , Sarcoma/radioterapia , Idoso , Intervalo Livre de Doença , Europa (Continente) , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Terapia Neoadjuvante/efeitos adversos , América do Norte , Radioterapia Adjuvante/efeitos adversos , Radioterapia Conformacional/efeitos adversos , Neoplasias Retroperitoneais/patologia , Neoplasias Retroperitoneais/cirurgia , Sarcoma/patologia , Sarcoma/cirurgia , Resultado do Tratamento
3.
DNA Cell Biol ; 39(9): 1558-1572, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32845706

RESUMO

Sarcomas are a broad family of cancers that arise from cells of mesenchymal origin in virtually every tissue of the body. Some transcription factors (TFs) have been reported to be involved in the pathogenesis and metastasis of sarcomas. The expression of certain long noncoding RNAs (lncRNAs) has been correlated with the degree of cancer prognosis. There is an urgent need to effectively integrate TFs and lncRNA/microRNA/mRNA regulatory axis and further identify more key regulators that play crucial roles in sarcomas. We performed a network-based computational analysis to investigate the lncRNA-TF cross talks via integrating lncRNA-TF ceRNA interactions and TF-TF protein-protein interactions. Multiple topology analyses were performed to the sarcomas-related global lncRNA-TF network. Several lncRNAs or TFs with central topology structures were identified as key regulators and used to locate a hub-associated lncRNA-TF subnetwork. Three functional modules were identified from the sarcomas-related global lncRNA-TF network, which have shown significant pathway enrichment and prognosis capability. The lncRNAs and TFs of these modules were shown to participate in sarcoma-related biological phenomena through involving in mitogen-activated protein kinases (MAPK), Jak-STAT, and transforming growth factor (TGF-beta) signaling pathways. More importantly, a subset of core lncRNA-TF cross talks that might form positive feedback loops to control biological processes of sarcomas was identified. These core lncRNA-TF positive feedback loops showed more TF binding affinity than other lncRNAs. All the results can help us uncover the molecular mechanism of sarcomas and provide a novel way for diagnosis biomarker and therapeutic target identification.


Assuntos
Regulação Neoplásica da Expressão Gênica , Redes Reguladoras de Genes , RNA Longo não Codificante/genética , Sarcoma/genética , Fatores de Transcrição/genética , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Retroalimentação Fisiológica , Humanos , Sistema de Sinalização das MAP Quinases , RNA Longo não Codificante/metabolismo , Sarcoma/patologia , Fatores de Transcrição/metabolismo
4.
Zhonghua Bing Li Xue Za Zhi ; 49(8): 816-820, 2020 Aug 08.
Artigo em Chinês | MEDLINE | ID: mdl-32746549

RESUMO

Objective: To investigate the clinicopathological characteristics, genetic features, diagnosis and differential diagnosis of pulmonary artery intimal sarcoma (PAIS). Methods: Three cases of PAIS were collected from Jiangsu Province People's Hospital (from February 2016 to November 2019). The clinical data, imaging examination, morphology, immunostaining, and molecular changes were retrospectively analyzed. Results: There were 1 male and 2 females (age: 32, 50, 60 years), who had symptoms of cough, asthma or chest tightness. Imaging findings indicated low density filling defects which were suspected as thrombus, embolism or myxoma. Grossly, the main tumor was located in the elastic arteries and their lobar branches, also extended into the atrium and ventricle, with lung parenchymal infiltration focally. Microscopically, tumor cells were predominantly composed of abundant spindle cells with obvious atypia and myxoid background, resembling fibroblastic or myofibroblastic differentiation. Active mitotic figures and necrosis could be seen in some areas. Immunohistochemical staining of vimentin was strongly positive, while pan-cytokeratin, S-100, desmin, Fli-1, CD31, SMA and ERG etc were variably positive only in focal areas. FISH detection showed amplification of MDM2 gene in three cases and EGFR gene in two cases. Metastatic lesions were found in one case by 18, 32 and 42 months after surgery respectively. There was no recurrence or metastasis in the other two cases. Conclusions: PAIS is one of exceptionally poor differentiated mesenchymal tumor that arises from the arterial intima of elastic pulmonary arteries. There was no definite differention in morphology. Gene detection shows amplification of MDM2 and EGFR gene. This tumor often has poor prognosis with aggressive behavior. Complete resection is the only effective therapeutic option. There is disagreement as to whether chemotherapy and radiotherapy can improve survival.


Assuntos
Artéria Pulmonar , Sarcoma , Neoplasias Vasculares/patologia , Adulto , Feminino , Humanos , Hibridização in Situ Fluorescente , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sarcoma/patologia , Vimentina
5.
Bone Joint J ; 102-B(8): 1088-1094, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32731827

RESUMO

AIMS: The existing clinical guidelines do not describe a clear indication for adjuvant radiotherapy (RT) in the treatment of superficial soft tissue sarcomas (STSs). We aimed to determine the efficacy of adjuvant RT for superficial STSs. METHODS: We retrospectively studied 304 patients with superficial STS of the limbs and trunk who underwent surgical resection at a tertiary sarcoma centre. The efficacy of RT was investigated according to the tumour size and grade: group 1, ≤ 5 cm, low grade; group 2, ≤ 5cm, high grade; group 3, > 5 cm, low grade; group 4, > 5 cm, high grade. RESULTS: The five- and ten-year local recurrence-free survival (LRFS) for all patients was 88% and 81%, respectively. While the efficacy of adjuvant RT was not proven in local control of all patients (five-year LRFS; RT+, 90% versus RT-, 83%; p = 0.074), the LRFS was significantly improved by adjuvant RT in group 2 (five-year LRFS; RT+, 96% versus RT-, 82%; p = 0.019), and group 4 (five-year LRFS; RT+, 87% versus RT-, 73%; p = 0.027). In groups 2 and 4, adjuvant RT significantly reduced the LR risk if the resection margin was clear but less than 5 mm; the LR rate was 7% with adjuvant RT compared with 26% with surgery alone (p = 0.003). There was no statistical relationship with the use of adjuvant RT and survival in every group. CONCLUSION: Adjuvant RT reduces the risk of local recurrence in patients with superficial high-grade STS regardless of tumour size, especially when resection margin is less than 5 mm. Cite this article: Bone Joint J 2020;102-B(8):1088-1094.


Assuntos
Recidiva Local de Neoplasia/epidemiologia , Sarcoma/radioterapia , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/radioterapia , Neoplasias de Tecidos Moles/cirurgia , Adulto , Institutos de Câncer , Estudos de Coortes , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Invasividade Neoplásica/patologia , Estadiamento de Neoplasias , Prognóstico , Radioterapia Adjuvante , Estudos Retrospectivos , Medição de Risco , Papel (figurativo) , Sarcoma/mortalidade , Sarcoma/patologia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Análise de Sobrevida , Adulto Jovem
6.
Vasc Endovascular Surg ; 54(7): 579-585, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32744174

RESUMO

Objective: To describe the types of intervention and determine patency and survival after arterial and venous reconstruction after surgical excision of sarcomas. Methods: Between November 2001 and July 2015, 42 patients with sarcomas and vascular involvement underwent surgical oncologic resection followed by arterial or venous reconstruction or preservation of the native vascular bundle. Univariate, multivariate, and Kaplan-Meier survival analyses were performed on abstracted data, which included demographics, risk factors, oncologic and vascular treatment modalities, postoperative complications, graft patency, and survival outcomes. Results: A total of 42 sarcomas required vascular assistance for oncologic removal. The majority of sarcomas were malignant fibrous histiocytoma (23.8%), and the most common anatomic location was the retroperitoneum (48%). A total of 12 revascularizations procedures were performed, including 5 arterial, 3 venous, and 2 concomitant arterial and venous. In 32 cases, a vascular surgeon was needed for vessel ligation, repair, or mobilization. The overall 2- and 5-year survival was 77.7% and 26.2%, respectively, with no significant survival difference between patients who underwent revascularization compared to those without revascularization. There was a 100% patency rate in all cases at last follow-up, regardless of the type of vascular reconstruction (median 18 months, range 1-29 months). On multivariate analysis, chronic obstructive pulmonary disease (COPD; P = .002) and positive surgical margins (P = .003) were associated with decreased survival. Most cases were performed in the last 5 years of the study (n = 27, 64.3%). Conclusions: Vascular reconstruction is feasible after surgical oncologic resection of sarcomas with good mid-term patency and limb preservation. Factors independently associated with mortality included COPD and positive surgical margins.


Assuntos
Artérias/cirurgia , Sarcoma/cirurgia , Procedimentos Cirúrgicos Vasculares , Veias/cirurgia , Adulto , Idoso , Artérias/patologia , Chicago , Feminino , Humanos , Salvamento de Membro , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Invasividade Neoplásica , Complicações Pós-Operatórias/mortalidade , Doença Pulmonar Obstrutiva Crônica/mortalidade , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Sarcoma/mortalidade , Sarcoma/patologia , Fatores de Tempo , Resultado do Tratamento , Grau de Desobstrução Vascular , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Procedimentos Cirúrgicos Vasculares/mortalidade , Veias/patologia
7.
Medicine (Baltimore) ; 99(29): e21118, 2020 Jul 17.
Artigo em Inglês | MEDLINE | ID: mdl-32702862

RESUMO

INTRODUCTION: Lipomas are rarely found in primary mesenchymal tumors of the hypopharynx and larynx. When they do appear, they often macroscopically resemble a retention cyst or laryngeal nodule. The laryngeal spindle cell lipoma, a variant, is extremely rare. PATIENT CONCERNS: A 65-year-old woman presented with a 3-month history of pharyngeal paraesthesia. Laryngoscopy revealed the presence of a well-encapsulated, smooth-surfaced, yellowish, pedicled mass on the left epiglottis. Magnetic resonance imaging confirmed the epiglottic mass. DIAGNOSIS: Following excision of the mass, the diagnosis of an spindle cell lipoma was established and confirmed by immunohistochemistry. INTERVENTIONS: Surgical excision of the lesion using a controlled-temperature plasma technique (coblation). OUTCOMES: At the last (4-year) follow-up evaluation, the patient was asymptomatic and without recurrence. CONCLUSION: SLC involvement of the epiglottis is rare. Coblation is an effective means to remove it.


Assuntos
Sarcoma/diagnóstico , Idoso , Feminino , Humanos , Laringoscopia/métodos , Laringe/patologia , Imagem por Ressonância Magnética/métodos , Faringe/anormalidades , Faringe/fisiopatologia , Sarcoma/patologia , Sarcoma/cirurgia
9.
Cancer Treat Rev ; 88: 102058, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32619864

RESUMO

Soft tissue sarcomas constitute 1% of adult malignant tumors. They are a heterogeneous group of more than 50 different histologic types. Isolated limb perfusion is an established treatment strategy for locally advanced sarcomas. Since its adoption for sarcomas in 1992, after the addition of TNFα, few modifications have been done and although indications for the procedure are essentially the same across centers, technical details vary widely. The procedures mainly involves a 60 min perfusion with melphalan and TNFα under mild hyperthermia, achieving a limb preservation rate of 72-96%; with an overall response rates from 72 to 82.5% and an acceptable toxicity according to the Wieberdink scale. The local failure rate is 27% after a median follow up of 14-31 months compared to 40% of distant recurrences after a follow up of 12-22 months. Currently there is no consensus regarding the benefit of ILP per histotype, and the value of addition of radiotherapy or systemic treatment. Further developments towards individualized treatments will provide a better understanding of the population that can derive maximum benefit of ILP with the least morbidity.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Quimioterapia do Câncer por Perfusão Regional/métodos , Sarcoma/tratamento farmacológico , Neoplasias de Tecidos Moles/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Quimioterapia do Câncer por Perfusão Regional/efeitos adversos , Quimioterapia do Câncer por Perfusão Regional/tendências , Ensaios Clínicos Fase II como Assunto , Extremidades/irrigação sanguínea , Extremidades/patologia , Humanos , Hipertermia Induzida/métodos , Melfalan/administração & dosagem , Melfalan/efeitos adversos , Ensaios Clínicos Controlados Aleatórios como Assunto , Sarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Fator de Necrose Tumoral alfa/administração & dosagem , Fator de Necrose Tumoral alfa/efeitos adversos
10.
Medicine (Baltimore) ; 99(27): e20880, 2020 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-32629676

RESUMO

To determine the value of 3T magnetic resonance imaging (MRI) texture analysis in differentiating high- from low-grade soft-tissue sarcoma.Forty-two patients with soft-tissue sarcomas who underwent 3T MRI were analyzed. Qualitative and texture analysis were performed on T1-, T2- and fat-suppressed contrast-enhanced (CE) T1-weighted images. Various features of qualitative and texture analysis were compared between high- and low-grade sarcoma. Areas under the receiver operating characteristic curves (AUC) were calculated for texture features. Multivariate logistic regression analysis was used to analyze the value of qualitative and texture analysis.There were 11 low- and 31 high-grade sarcomas. Among qualitative features, signal intensity on T1-weighted images, tumor margin on T2-weighted images, tumor margin on fat-suppressed CE T1-weighted images and peritumoral enhancement were significantly different between high- and low-grade sarcomas. Among texture features, T2 mean, T1 SD, CE T1 skewness, CE T1 mean, CE T1 difference variance and CE T1 contrast were significantly different between high- and low-grade sarcomas. The AUCs of the above texture features were > 0.7: T2 mean, .710 (95% confidence interval [CI] .543-.876); CE T1 mean, .768 (.590-.947); T1 SD, .730 (.554-.906); CE T1 skewness, .751 (.586-.916); CE T1 difference variance, .721 (.536-.907); and CE T1 contrast, .727 (.530-.924). The multivariate logistic regression model of both qualitative and texture features had numerically higher AUC than those of only qualitative or texture features.Texture analysis at 3T MRI may provide additional diagnostic value to the qualitative MRI imaging features for the differentiation of high- and low-grade sarcomas.


Assuntos
Imagem por Ressonância Magnética/métodos , Sarcoma/diagnóstico por imagem , Sarcoma/patologia , Neoplasias de Tecidos Moles/diagnóstico por imagem , Neoplasias de Tecidos Moles/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Processamento de Imagem Assistida por Computador/métodos , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Reconhecimento Automatizado de Padrão , Adulto Jovem
11.
Asian Cardiovasc Thorac Ann ; 28(5): 282-285, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32483975

RESUMO

Primary pulmonary intimal sarcoma is rare. Differentiating it from pulmonary thromboembolism is difficult because of similarities in clinical symptoms and imaging findings. Positron-emission tomography-computed tomography has been useful for diagnosing primary pulmonary intimal sarcoma. We describe a rare case of primary pulmonary intimal sarcoma that showed no abnormal 18F-fluorodeoxyglucose uptake on positron-emission tomography. We resected the mass and performed right ventricular outflow tract reconstruction. Proper diagnosis is necessary to determine appropriate therapy, Clinicians must consider the possibility of primary pulmonary intimal sarcoma even if imaging findings are inconsistent with the disease.


Assuntos
Fluordesoxiglucose F18/administração & dosagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Artéria Pulmonar/diagnóstico por imagem , Compostos Radiofarmacêuticos/administração & dosagem , Sarcoma/diagnóstico por imagem , Túnica Íntima/diagnóstico por imagem , Neoplasias Vasculares/diagnóstico por imagem , Idoso , Feminino , Humanos , Valor Preditivo dos Testes , Artéria Pulmonar/patologia , Artéria Pulmonar/cirurgia , Sarcoma/patologia , Sarcoma/cirurgia , Resultado do Tratamento , Túnica Íntima/patologia , Túnica Íntima/cirurgia , Neoplasias Vasculares/patologia , Neoplasias Vasculares/cirurgia
12.
J Cancer Res Ther ; 16(2): 397-400, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32474530

RESUMO

Retroperitoneal leiomyosarcoma is relatively uncommon. Leiomyosarcoma has accounted for about 5%-10% of soft-tissue sarcoma, and 1/2-2/3 of the primary lesions were retroperitoneal, with a cumulative 5-year survival rate of only 35%.Leiomyosarcoma is one kind of soft-tissue sarcoma with the lowest survival rates due to the invasive growth, difficult treatment, and poor prognosis.The present study reported a case of a 78-year-old male diagnosed as left retroperitoneal leiomyosarcoma, who had received three operations. Computed tomography (CT) demonstrated a mass of approximately 12.9 cm × 6.9 cm × 6.6 cm in his retroperitoneal region. The Eastern cooperative oncology group and numerical rating scale scores of pain were 1 and 5, respectively. Multiple treatment strategies were administered, including the application of drainage and125I seed implantation. A total of 90125I seeds were implanted into the tumor through repetitious operations, with 30 seeds each time. Treatment planning system was involved to calculate the source distribution.125I seeds with the activity of 0.5 mCi were implanted under the guidance of CT, and dosimetric verification was performed after the operation. D90 (90% minimum prescription dose received by target volume) was 40 Gy. Follow-up was performed after 6 months, and complete response was achieved in the local lesions. However, there was no evidence-based treatment currently and the majority of our knowledge was based on results from case reports, thus further studies would be required.


Assuntos
Braquiterapia/métodos , Radioisótopos do Iodo/uso terapêutico , Leiomiossarcoma/radioterapia , Inoculação de Neoplasia , Neoplasias Retroperitoneais/radioterapia , Sarcoma/radioterapia , Idoso , Humanos , Leiomiossarcoma/patologia , Masculino , Dosagem Radioterapêutica , Neoplasias Retroperitoneais/patologia , Sarcoma/patologia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
13.
Cancer Sci ; 111(9): 3303-3312, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32579783

RESUMO

Alveolar soft part sarcoma (ASPS), epithelioid sarcoma (ES), and clear cell sarcoma (CCS) are known to be chemoresistant tumors. The aim of this study was to investigate the effect of pazopanib on these chemoresistant tumors. This study is designed as a single-arm, multicenter, investigator-initiated phase II trial. Patient enrollment was undertaken between July 2016 and August 2018 at 10 hospitals participating in the Japanese Musculoskeletal Oncology Group. The primary end-point is the CBR (CBR, including complete or partial response and stable disease) at 12 weeks after treatment with pazopanib according to RECIST. Eight patients were enrolled within the period. The histological subtypes were 5 ASPS, 2 ES, and 1 CCS. The median follow-up period was 22.2 (range, 4.9-24.9) months. All patients initially received pazopanib 800 mg once daily. The CBRs were 87.5% (7 of 8) and 75.0% (6 of 8) according to RECIST and Choi criteria at 12 weeks after pazopanib treatment, respectively. The CBRs at 12 weeks according to RECIST were 80.0%, 100.0%, and 100.0% in ASPS, ES, and CCS, respectively. Partial response was observed in 1 ASPS according to RECIST and 3 ASPS and 1 ES according to Choi criteria at 12 weeks after pazopanib treatment. This study documented antitumor activity of pazopanib, especially in ASPS. These results support the frontline use of pazopanib for ASPS. Prospective data collection is desired using both RECIST and Choi criteria for these rare chemoresistant tumors.


Assuntos
Inibidores da Angiogênese/uso terapêutico , Resistencia a Medicamentos Antineoplásicos , Pirimidinas/uso terapêutico , Sarcoma/tratamento farmacológico , Sarcoma/patologia , Sulfonamidas/uso terapêutico , Adolescente , Adulto , Idoso , Inibidores da Angiogênese/administração & dosagem , Inibidores da Angiogênese/efeitos adversos , Feminino , Humanos , Japão , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Metástase Neoplásica , Estadiamento de Neoplasias , Pirimidinas/administração & dosagem , Pirimidinas/efeitos adversos , Sarcoma/terapia , Sulfonamidas/administração & dosagem , Sulfonamidas/efeitos adversos , Resultado do Tratamento , Adulto Jovem
14.
Br J Radiol ; 93(1114): 20190856, 2020 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-32559116

RESUMO

OBJECTIVES: Sarcoma patients often undergo surveillance chest CT for detection of pulmonary metastases. No data exist on the optimal surveillance interval for chest CT. The aim of this study was to estimate pulmonary metastasis growth rate in sarcoma patients. METHODS: This was a retrospective review of 95 patients with pulmonary metastases (43 patients with histologically confirmed metastases and 52 with clinically diagnosed metastases) from sarcoma treated at an academic tertiary-care center between 01 January 2000 and 01 June 2019. Age, sex, primary tumor size, grade, subtype, size and volume of the pulmonary metastasis over successive chest CT scans were recorded. Two metastases per patient were chosen if possible. Multivariate linear mixed-effects models with random effects for each pulmonary metastasis and each patient were used to estimate pulmonary metastasis growth rate, evaluating the impact of patient age, tumor size, tumor grade, chemotherapy and tumor subtype. We estimated the pulmonary metastasis volume doubling time using these analyses. RESULTS: Maximal primary tumor size at diagnosis (LRT statistic = 2.58, df = 2, p = 0.275), tumor grade (LRT statistic = 1.13, df = 2, p = 0.567), tumor type (LRT statistic = 7.59, df = 6, p = 0.269), and patient age at diagnosis (LRT statistic = 0.735, df = 2, p = 0.736) were not statistically significant predictors of pulmonary nodule growth from baseline values. Chemotherapy decreased the rate of pulmonary nodule growth from baseline (LRT statistic = 7.96, df = 2, p = 0.0187). 95% of untreated pulmonary metastases are expected to grow less than 6 mm in 6.4 months. There was significant intrapatient and interpatient variation in pulmonary metastasis growth rate. Pulmonary metastasis volume growth rate was best fit with an exponential model in time. The volume doubling time for pulmonary metastases assuming an exponential model in time was 143 days (95% CI (104, 231) days). CONCLUSIONS: Assuming a 2 mm nodule is the smallest reliably detectable nodule by CT, the data suggest that an untreated pulmonary metastasis is expected to grow to 8 mm in 8.4 months (95% CI (4.9, 10.2) months). Tumor size, grade and sarcoma subtype did not significantly alter pulmonary metastasis growth rate. However, chemotherapy slowed the pulmonary metastasis growth rate. ADVANCES IN KNOWLEDGE: CT surveillance intervals for pulmonary metastases can be estimated based on metastasis growth rate. There was significant variation in the pulmonary metastasis growth rate between metastases within patient and between patients. Pulmonary nodule volume growth followed an exponential model, linear in time.


Assuntos
Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/secundário , Nódulos Pulmonares Múltiplos/diagnóstico por imagem , Nódulos Pulmonares Múltiplos/secundário , Sarcoma/patologia , Tomografia Computadorizada por Raios X , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
15.
Medicine (Baltimore) ; 99(21): e20165, 2020 May 22.
Artigo em Inglês | MEDLINE | ID: mdl-32481285

RESUMO

Soft tissue sarcoma (STS) of the extremities are a rare tumor. Metastases develop in about 40%-50% of patients, most of whom die from their disease. We sought to identify potential risk factors associated with metastatic diseases upon presentation for patients with STS and established a reliable nomogram model to predict distant metastasis of STS at presentation. The current study retrospectively analyzed 3884 STS of the extremities or trunk patients from the Surveillance, Epidemiology, and End Results (SEER) database between 2010 and 2015. Based on patient registration, all patients were randomly allocated to training sets and validation sets (2:1). Then, univariate and binary logistic regression analysis was used to determine the significantly correlated predictors of metastasis. Finally, the nomogram model was established, using these predictors and validated it. 311 (8.21%) of the cases experienced distant metastatic disease was present at the time of presentation. The nomogram was developed from age, histology subtype, primary site, tumor size, grade and depth. Encouragingly, the nomogram showed favorable calibration with C-index 0.790 in the training set and 0.801 in validation set. The DCA showed that the novel model was clinically useful. This nomogram model had a high precision to predict the metastasis of soft tissue sarcoma of the extremities. We expect this model could be used in different clinical consultation and established risk assessment.


Assuntos
Metástase Neoplásica/patologia , Nomogramas , Sarcoma/patologia , Neoplasias de Tecidos Moles/patologia , Adulto , Estudos de Casos e Controles , Extremidades/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Programa de SEER , Análise de Sobrevida
16.
Medicine (Baltimore) ; 99(26): e20719, 2020 Jun 26.
Artigo em Inglês | MEDLINE | ID: mdl-32590747

RESUMO

BACKGROUND: Regorafenib, a multitargeted tyrosine kinase inhibitor, proved to be active in patients with soft tissue sarcomas (STS). METHODS: We conducted an open-label, non-randomized, single-center phase II study in advanced pretreated STS patients. Patients received regorafenib 160 mg daily on days 1 enrule 21 of a 28-day cycle. The primary endpoint was the progression-free survival (PFS) at 8 weeks. Toxicity was registered. RESULTS: Between April 2015 and November 2016, 21 patients were enrolled in the trial. A total of 13 out of 21 evaluable patients (61.9%) were progression-free at 8 weeks. Median PFS was 3.8 months (95% CI: 2.1-9.4). Median overall survival was 14.8 months (95% CI: 7.7-27.8). In the intention-to-treat population, we reported a PFS of 66.7% at 3 months (95% CI: 40.4-83.4) and 16.7% at 12 months (95% CI: 4.1-36.5). As per the RECIST criteria, the response rate was 4.7% (1 partial response out of 21 evaluable patients) with a clinical benefit rate of 61.9%; no complete response was observed. Treatment was well tolerated. CONCLUSION: Regorafenib shows signs of clinical activity in patients with advanced STS. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov NCT02307500.


Assuntos
Neoplasias Ovarianas , Neoplasias Pancreáticas , Compostos de Fenilureia , Piridinas , Sarcoma , Timoma , Antineoplásicos/administração & dosagem , Antineoplásicos/efeitos adversos , Monitoramento de Medicamentos/métodos , Inibidores Enzimáticos/administração & dosagem , Inibidores Enzimáticos/efeitos adversos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Ovarianas/tratamento farmacológico , Neoplasias Ovarianas/patologia , Neoplasias Pancreáticas/tratamento farmacológico , Neoplasias Pancreáticas/patologia , Compostos de Fenilureia/administração & dosagem , Compostos de Fenilureia/efeitos adversos , Intervalo Livre de Progressão , Piridinas/administração & dosagem , Piridinas/efeitos adversos , Critérios de Avaliação de Resposta em Tumores Sólidos , Sarcoma/tratamento farmacológico , Sarcoma/patologia , Timoma/tratamento farmacológico , Timoma/patologia , Resultado do Tratamento
17.
J Cardiothorac Surg ; 15(1): 71, 2020 May 04.
Artigo em Inglês | MEDLINE | ID: mdl-32366268

RESUMO

BACKGROUND: Primary undifferentiated spindle cell sarcoma in the right ventricle is an extremely rare tumor. Radical surgical excision is the optimal treatment for long-term survival due to poor response to chemotherapy or radiotherapy at an advanced stage. CASE PRESENTATION: A 42-year-old man with no previous medical history presented with mild dyspnea on exertion and abdominal distension that lasted a week. Computed tomography (CT) revealed a huge homogeneous mass completely obstructing the right ventricle and extending into the pulmonary trunk. However, he suddenly collapsed the next day while on his way to an echocardiography. An extracorporeal membrane oxygenation (ECMO) device was inserted percutaneously and ECMO support was urgently initiated. Based on consideration of right ventricular outflow tract (RVOT) obstruction in the initial CT scan, we decided to remove the mass from the right ventricle immediately. The main mass was resected to relieve the RVOT obstruction, and after the operation, the ECMO was removed from the operation room. However, the patient failed to regain consciousness and electroencephalography (EEG) and subsequent magnetic resonance imaging (MRI) indicated severe hypoxic brain damage. We assume CPR was unsuccessful because the mass completely blocked the RVOT. Pathology revealed the mass was an undifferentiated spindle cell sarcoma. CONCLUSIONS: We present the case of a 42-year-old male with cardiac arrest due to right ventricular outflow tract obstruction by a tumor of the right ventricle. Surgical resection was performed and in histopathology it was proved to be an undifferentiated spindle cell sarcoma.


Assuntos
Neoplasias Cardíacas/diagnóstico por imagem , Sarcoma/diagnóstico por imagem , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem , Adulto , Reanimação Cardiopulmonar , Dispneia/etiologia , Ecocardiografia , Oxigenação por Membrana Extracorpórea , Parada Cardíaca/etiologia , Parada Cardíaca/terapia , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/cirurgia , Ventrículos do Coração , Humanos , Hipóxia Encefálica/etiologia , Imagem por Ressonância Magnética , Masculino , Artéria Pulmonar , Sarcoma/complicações , Sarcoma/patologia , Sarcoma/cirurgia , Tomografia Computadorizada por Raios X , Obstrução do Fluxo Ventricular Externo/etiologia , Obstrução do Fluxo Ventricular Externo/patologia , Obstrução do Fluxo Ventricular Externo/cirurgia
18.
J Surg Oncol ; 122(3): 506-514, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32410271

RESUMO

BACKGROUND AND OBJECTIVES: Primary pulmonary sarcoma (PPS) accounts for less than 1.1% of all pulmonary tumors. Few outcome data are reported. We evaluated outcome and prognostic factors in our series. METHODS: We retrospectively reviewed all patients who underwent resection for PPS in our center from 2002 to 2018. Survival was calculated from the date of surgery until last follow-up. Impact on survival of gender, type of lung resection, completeness of resection, grade, size, and TNM staging for lung cancer and soft tissue sarcoma (STS) was assessed. RESULTS: Thirteen patients were included. Eight (61.5%) patients received neoadjuvant treatment. Median tumor size at diagnosis was 11.5 cm (1-30 cm). Type of lung resection was wedge (n = 2, 15%), lobectomy (n = 4, 31%), intrapericardial (n = 3, 23%), and extrapleural pneumonectomies (n = 4, 31%). In-hospital mortality was 8%. Overall 5-year survival was 60%. Median disease-free survival was 17 months. Tumor size was a predictor for survival (P = .02) and recurrence (P = .05). Gender (P = .04) and type of lung resection (P = .04) were predictors of survival. T stage for STS of trunk and extremity, and TNM stage for lung cancer were predictors for recurrence (P = .03 and P = .04, respectively). CONCLUSION: Surgical resection within a multimodality therapy concept in highly selected patients can offer good long-term outcome.


Assuntos
Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/cirurgia , Sarcoma/patologia , Sarcoma/cirurgia , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Quimiorradioterapia Adjuvante , Quimioterapia Adjuvante , Intervalo Livre de Doença , Feminino , Humanos , Neoplasias Pulmonares/terapia , Masculino , Pessoa de Meia-Idade , Terapia Neoadjuvante , Estadiamento de Neoplasias , Pneumonectomia/métodos , Estudos Retrospectivos , Sarcoma/terapia , Taxa de Sobrevida , Adulto Jovem
19.
Biomed Khim ; 66(2): 151-155, 2020 Feb.
Artigo em Russo | MEDLINE | ID: mdl-32420896

RESUMO

The aim of the study was to determine the level of sex steroid hormones in white matter of the brain of rats with tumors combined with chronic neurogenic pain (CNP), which was modeled by bilateral sciatic nerve ligation. The study included albino male rats (n=74). In the main group, M1 sarcoma was transplanted subcutaneously (n=11) or into the subclavian vein (n=11) 45 days after CNP modeling. Two comparison groups (n=13 each) included sham operated animals (without CNP) with M1 sarcoma transplanted subcutaneously and intravenously. Control groups included animals with CNP and sham operated animals. Rats were euthanized on day 21 of the carcinogenesis. Levels of total and free testosterone (T), estrone (E1), estradiol (E2), estriol (E3) and progesterone (P4) in the brain white matter were measured using ELISA kits ("Cusabio", China). CNP caused a decrease in the total and free T by 1.5 times (p<0.05), E2 and P4 by 1.9 and 3 times, respectively, E3 by 1.6 times (p<0.05), as well as an increase in E1 by 1.4 times (p<0.05) as compared to the corresponding levels in the brain white matter of rats without CNP. CNP stimulated M1 sarcoma growth in both subcutaneous and intravenous transplantation. Regardless of the tumor site, the dynamics of total T, E2 and E3 in the brain had similar features, but the dynamics of free T, P4 and E1 differed. Thus, changes in the level of neurosteroids in the white matter of rat brain with CNP and tumor growth alone or associated with CNP are a reaction to stress.


Assuntos
Química Encefálica , Neoplasias Experimentais/patologia , Neuroesteroides/análise , Dor/patologia , Sarcoma/patologia , Animais , Estradiol , Estrona , Masculino , Transplante de Neoplasias , Progesterona , Ratos
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