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1.
Medicine (Baltimore) ; 100(35): e27132, 2021 Sep 03.
Artigo em Inglês | MEDLINE | ID: mdl-34477160

RESUMO

RATIONALE: Primary pleural angiosarcoma (PPA) is an extremely rare malignancy for which there is no consensus on treatment. The clinical course of PPA is usually quickly fatal, regardless of the treatment used. PATIENT CONCERNS: We describe the rare case of a 52-year-old man who presented initially with hemoptysis and received emergency surgery for the primary. DIAGNOSES: He received a confirmed diagnosis of primary pleural angiosarcoma (PPA) by postoperative pathology and was subsequently treated with radiotherapy and chemotherapy, but had failed and was intolerant to chemotherapy. INTERVENTIONS: The patient had 5% tumor PD-L1 positivity with 22C3 pharmDx and received pembrolizumab (200 mg every 21 days) for 13 cycles. OUTCOMES: The disease remained well controlled according to the RECIST 1.1. criteria. He is currently under observation and waiting to start the next cycle of immunotherapy. LESSON: Our case report suggests that the use of anti-PD-1 therapy does show efficacy in the treatment of PPA and may provide a viable treatment option for patients.


Assuntos
Inibidores de Checkpoint Imunológico/uso terapêutico , Neoplasias Pleurais/tratamento farmacológico , Sarcoma/tratamento farmacológico , Humanos , Masculino , Pessoa de Meia-Idade , Pleura/patologia , Neoplasias Pleurais/diagnóstico por imagem , Neoplasias Pleurais/patologia , Sarcoma/diagnóstico por imagem , Sarcoma/patologia , Tomografia Computadorizada por Raios X
3.
Int J Mol Sci ; 22(14)2021 Jul 13.
Artigo em Inglês | MEDLINE | ID: mdl-34299136

RESUMO

Soft tissue sarcomas (STSs) are a family of rare malignant tumors encompassing more than 80 histologies. Current therapies for metastatic STS, a condition that affects roughly half of patients, have limited efficacy, making innovative therapeutic strategies urgently needed. From a molecular point of view, STSs can be classified as translocation-related and those with a heavily rearranged genotype. Although only the latter display an increased mutational burden, molecular profiles suggestive of an "immune hot" tumor microenvironment are observed across STS histologies, and response to immunotherapy has been reported in both translocation-related and genetic complex STSs. These data reinforce the notion that immunity in STSs is multifaceted and influenced by both genetic and epigenetic determinants. Cumulative evidence indicates that a fine characterization of STSs at different levels is required to identify biomarkers predictive of immunotherapy response and to discover targetable pathways to switch on the immune sensitivity of "immune cold" tumors. In this review, we will summarize recent findings on the interplay between genetic landscape, molecular profiling and immunity in STSs. Immunological and molecular features will be discussed for their prognostic value in selected STS histologies. Finally, the local and systemic immunomodulatory effects of the targeted drugs imatinib and sunitinib will be discussed.


Assuntos
Biomarcadores Tumorais/antagonistas & inibidores , Regulação Neoplásica da Expressão Gênica , Imunoterapia/métodos , Terapia de Alvo Molecular , Sarcoma/imunologia , Microambiente Tumoral/imunologia , Animais , Biomarcadores Tumorais/imunologia , Humanos , Sarcoma/tratamento farmacológico , Sarcoma/patologia
4.
Medicine (Baltimore) ; 100(28): e26632, 2021 Jul 16.
Artigo em Inglês | MEDLINE | ID: mdl-34260555

RESUMO

ABSTRACT: BCL7B plays a potential role in the progression of various cancers, while its role in sarcomas is unknown. We aimed to evaluate BCL7B's diagnostic and prognostic value, and potential BCL7B-related mechanisms in sarcomas based on The Cancer Genome Atlas (TCGA) database. We collected patients with sarcoma from TCGA. Wilcoxon rank sum test was used to compare the expression of BCL7B in sarcoma samples with different clinical-pathologic features. Univariate Cox regression analysis and multivariate Cox regression analysis were used to evaluate prognosis factors for sarcoma. Gene set enrichment analysis (GSEA) was conducted to elucidate the significant functions and pathways associated with BCL7B. BCL7B was a potential biomarker for distinguishing normal and tumor tissues with the analysis of ROC curve (AUC = 0.588). Low BCL7B expression was significantly correlated with tumor multifocal (OR = 0.39 for yes vs no), larger residual tumor (OR = 0.40 for R1,R2 vs RO), male gender (OR = 0.48 for male vs female) and White race (OR = 0.29 for White vs Asian, Black or African American). High BCL7B expression was correlated with leiomyosarcoma histological type (OR = 6.08 for leiomyosarcoma vs dedifferentiated liposarcoma, pleomorphic sarcoma). Univariate and multivariate Cox regression analysis showed that low BCL7B expression was independently associated with poor overall survival (P = .008). GSEA showed that GPCR (G protein-coupled receptors) ligand binding, secreted factors, class A1 rhodopsin-like receptors, extracellular matrix organization, core matrisome, Fc epsilon receptor I mediated NF-κB activation, and WNT signaling pathway were differentially enriched in BCL7B low expression phenotype (|NES| > 1, adjusted P value <.05, and FDR value <0.25). BCL7B may play an important role in sarcoma progression and may be a potential biomarker for prognosis and diagnosis in sarcomas.


Assuntos
Proteínas/genética , Sarcoma/genética , Sarcoma/patologia , Idoso , Biomarcadores Tumorais , Biologia Computacional , Grupos de Populações Continentais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Análise de Regressão , Sarcoma/diagnóstico , Fatores Sexuais
5.
Aging (Albany NY) ; 13(11): 15126-15138, 2021 06 03.
Artigo em Inglês | MEDLINE | ID: mdl-34081621

RESUMO

Sarcomatoid hepatocellular carcinoma (sHCC) is a rare type of liver malignancy. Currently, the tumor immune features of sHCC are poorly understood. We recruited 31 patients with resected sHCC for whom tissue samples and complete clinicopathologic and follow-up data were available. To understand the immune infiltration of sHCC, immunohistochemical staining was performed on the resected sHCC samples to compare the expressions of programmed death-1 (PD-1), programmed death-ligand 1 (PD-L1), B7-H3, indoleamine 2,3-dioxygenase (IDO), lymphocyte-activation gene 3 (LAG-3), CD8, FOXP3, and CD68 in tumor and peritumoral tissues. Kaplan-Meier and Cox regression analyses were used to assess the predictive value of immune markers. Sarcomatoid components were characterized with significantly higher expression of PD-L1 and B7-H3 in tumor cells than in conventional HCC components, as well as in peritumoral tissue. Additionally, sarcomatoid components had a higher density of FOXP3+ and LAG-3+ cells and a lower density of CD8+ cells than conventional HCC components or peritumoral tissue. Higher expression of PD-L1 in tumor cells significantly correlated with higher densities of CD8+, PD-1+, and LAG-3+ cells. Increased tumor PD-L1 expression and decreased CD8+ T-cell density were associated with poor overall survival (OS) and disease-free survival (DFS) in patients of sHCC. These findings suggest further characterization on relative mechanism of sHCC immune infiltration may identify therapeutic targets for immunotherapy.


Assuntos
Carcinoma Hepatocelular/imunologia , Neoplasias Hepáticas/imunologia , Linfócitos do Interstício Tumoral/imunologia , Sarcoma/imunologia , Biomarcadores Tumorais/metabolismo , Carcinoma Hepatocelular/patologia , Contagem de Células , Intervalo Livre de Doença , Humanos , Proteínas de Checkpoint Imunológico/metabolismo , Estimativa de Kaplan-Meier , Neoplasias Hepáticas/patologia , Análise Multivariada , Proteínas de Neoplasias/metabolismo , Recidiva Local de Neoplasia/patologia , Prognóstico , Sarcoma/patologia
6.
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
7.
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
8.
Bone Joint J ; 103-B(6): 1142-1149, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-34058874

RESUMO

AIMS: Time to treatment initiation (TTI) is generally defined as the time from the histological diagnosis of malignancy to the initiation of first definitive treatment. There is no consensus on the impact of TTI on the overall survival in patients with a soft-tissue sarcoma. The purpose of this study was to determine if an increased TTI is associated with overall survival in patients with a soft-tissue sarcoma, and to identify the factors associated with a prolonged TTI. METHODS: We identified 23,786 patients from the National Cancer Database who had undergone definitive surgery between 2004 and 2015 for a localized high-grade soft-tissue sarcoma of the limbs or trunk. A Cox proportional hazards model was used to examine the relationship between a number of factors and overall survival. We calculated the incidence rate ratio (IRR) using negative binomial regression models to identify the factors that affected TTI. RESULTS: Patients in whom the time to treatment initiation was prolonged had poorer overall survival than those with a TTI of 0 to 30 days. These were: 31 to 60 days (hazard ratio (HR) 1.08, p = 0.011); 61 to 90 days (HR 1.11, p = 0.044); and 91 days (HR 1.22; p = 0.003). The restricted cubic spline showed that the hazard ratio increased substantially with a TTI longer than 50 days. Non-academic centres (vs academic centres; IRR ranging from 0.64 to 0.86; p < 0.001) had a shorter TTI. Those insured by Medicaid (vs private insurance; IRR 1.34), were uninsured (vs private insurance; IRR 1.17), or underwent a transition in care (IRR 1.62) had a longer TTI. CONCLUSION: A time to treatment initiation of more than 30 days after diagnosis was independently associated with poorer survival. The hazard ratio showed linear increase, especially if the TTI was more than 50 days. We recommend starting treatment within 30 days of diagnosis to achieve the highest likelihood of cure for localized high-grade soft-tissue sarcomas in the limbs and trunk, even when a patient needs to be referred to a specialist centre. Cite this article: Bone Joint J 2021;103-B(6):1142-1149.


Assuntos
Sarcoma/mortalidade , Sarcoma/cirurgia , Tempo para o Tratamento , Idoso , Extremidades , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Sistema de Registros , Estudos Retrospectivos , Sarcoma/patologia , Taxa de Sobrevida , Tórax , Estados Unidos/epidemiologia
9.
J Surg Oncol ; 124(4): 679-686, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34120344

RESUMO

BACKGROUND: The aim of the study is to evaluate functional and oncological outcomes of patients undergoing abdominal wall soft tissue tumors (AWSTT) surgery. METHODS: All consecutive patients that underwent surgery for malignant and intermediate AWSTT from 1999 to 2019 were retrospectively analyzed. RESULTS: Ninety-two patients were identified, 20 (22%) operated on for a desmoid tumor and 72 (78%) for a soft tissue sarcoma (STS). Fifty-two patients (57%) had in toto resection of the abdominal wall (from the skin to the peritoneum) and 9 (10%) required simultaneous visceral resection. The closure was direct in 28 patients (30%) and requiring a mesh, a flap or a combination of the two in respectively 42, 16, and 6 patients (47%, 17%, 6%). The postoperative complications rate was 26%. Thirteen patients (14%) developed an incisional hernia after a median delay of 27 months. After a median follow-up of 40 months, out of the 72 patients operated on for STS, 7 (10%) developed local recurrence and 11 (15%) distant recurrence. The median recurrence-free and overall survivals were 61 and 116, months respectively. CONCLUSIONS: Management of AWSTT requires extensive surgery but allows good local control with an acceptable rate of incisional hernia.


Assuntos
Neoplasias Abdominais/cirurgia , Hospitais com Alto Volume de Atendimentos/estatística & dados numéricos , Recidiva Local de Neoplasia/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/mortalidade , Sarcoma/cirurgia , Neoplasias Abdominais/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Prognóstico , Estudos Retrospectivos , Sarcoma/patologia , Taxa de Sobrevida , Adulto Jovem
10.
Bull Cancer ; 108(6): 654-667, 2021 Jun.
Artigo em Francês | MEDLINE | ID: mdl-33985762

RESUMO

Sarcoma consists in a group of rare malignant tumours of mesenchymal origin characterized by their vast clinical, pathological and biological heterogeneity. The pathological diagnosis of sarcoma relies classically of the differentiation features of tumour cells, with dozens of different tumour subtypes described in the last international classifications. Over the last decades, the advances in the development of new techniques of molecular biology have led to a major complexification of sarcoma classification, with the identification of multiple and specific molecular alterations that have led to significant changes for patients diagnostic, prognostic and therapeutic management. This review aims at giving an overview on the current knowledge of the molecular biology of soft tissue sarcoma, and emphasizes on their consequences for the daily management of patients.


Assuntos
Sarcoma/genética , Feminino , Amplificação de Genes , Fusão Gênica , Humanos , Mutação , Prognóstico , Rabdomiossarcoma/genética , Sarcoma/patologia , Sarcoma de Ewing/genética , Translocação Genética , Microambiente Tumoral/genética , Neoplasias Uterinas/genética
11.
World Neurosurg ; 152: 1-2, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-34058364

RESUMO

Retroperitoneal sarcomas may grow extremely large before becoming clinically symptomatic, and curative resection often has high associated morbidity. An 83-year-old man presented with insidious-onset abdominal pain and weight loss owing to a 16.3 × 13.1 × 25.8-cm retroperitoneal high-grade myxoid spindle cell sarcoma. The patient was ultimately deemed to be unfit for surgery before rapidly succumbing to his disease. This case illustrates both the indolent growth seen in these lesions and the importance of proper patient selection. Older patients with large, high-grade lesions and multiple associated comorbidities are often poor surgical candidates, as the associated surgical morbidity outweighs the potential survival benefit. While the ultimate treatment plan relies on shared decision making by the patient and the healthcare provider, the decision to pursue surgical intervention should take into consideration the patient's broader clinical condition.


Assuntos
Neoplasias Retroperitoneais/patologia , Sarcoma/patologia , Idoso de 80 Anos ou mais , Braquiterapia , Humanos , Masculino , Segunda Neoplasia Primária/patologia , Neoplasias da Próstata/patologia , Neoplasias da Próstata/radioterapia , Neoplasias Retroperitoneais/complicações , Sarcoma/complicações , Perda de Peso
12.
Br J Radiol ; 94(1123): 20210088, 2021 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-33989031

RESUMO

The lungs are the commonest site of metastasis for primary high-grade bone and soft tissue sarcoma, but current guidelines on the management of pulmonary nodules do not specifically cater for this group of patients. The current article reviews the literature from the past 20 years that has reported the CT features of pulmonary metastases in the setting of known primary bone and soft tissue sarcoma, with emphasis on osteosarcoma, chondrosarcoma, and trunk and extremity soft tissue sarcoma, the aim being to aid radiologists who report chest CT of musculoskeletal sarcoma patients in deciding which lesions should be considered metastatic, which lesions are indeterminate and require follow-up, and which lesions are of no concern.


Assuntos
Neoplasias Ósseas/patologia , Condrossarcoma/patologia , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/secundário , Osteossarcoma/patologia , Sarcoma/patologia , Tomografia Computadorizada por Raios X , Humanos , Gradação de Tumores , Radiografia Torácica
13.
Anticancer Res ; 41(5): 2719-2726, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33952503

RESUMO

BACKGROUND/AIM: We present a case of uterine dedifferentiated mesonephric-like adenocarcinoma (MLA). CASE REPORT: A 54-year-old woman underwent total hysterectomy for a uterine mass under the impression of a uterine sarcoma. Histologically, MLA exhibited various growth patterns including tubular and glandular architecture. Undifferentiated carcinoma (UC) displayed discohesive tumor cells without any obvious architecture. Immunohistochemically, UC was positive for epithelial markers in very few scattered tumor cells. MLA exhibited the wild-type p53 expression pattern, whereas UC showed a uniform and strong p53 immunoreactivity. Targeted sequencing analysis revealed an identical Kirsten rat sarcoma viral oncogene homolog (KRAS) mutation in both components. A pathogenic missense tumor protein 53 (TP53) mutation was detected in UC, but not in MLA. CONCLUSION: The mutant p53 expression pattern exclusively detected in UC was concordant with the presence of missense TP53 mutation. Our observations suggested that TP53 mutation is associated with the possible transformation from MLA to UC.


Assuntos
Adenocarcinoma/diagnóstico , Carcinoma/diagnóstico , Sarcoma/diagnóstico , Doenças Uterinas/diagnóstico , Adenocarcinoma/genética , Adenocarcinoma/patologia , Adenocarcinoma/cirurgia , Carcinoma/genética , Carcinoma/patologia , Carcinoma/cirurgia , Desdiferenciação Celular/genética , Feminino , Humanos , Histerectomia , Pessoa de Meia-Idade , Sarcoma/genética , Sarcoma/patologia , Sarcoma/cirurgia , Doenças Uterinas/patologia , Doenças Uterinas/cirurgia , Ductos Mesonéfricos/diagnóstico por imagem , Ductos Mesonéfricos/patologia , Ductos Mesonéfricos/cirurgia
14.
Jpn J Clin Oncol ; 51(7): 1088-1093, 2021 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-33822987

RESUMO

OBJECTIVE: The aim of this systematic review was to evaluate the efficacy of metastasectomy for patients with advanced soft tissue sarcoma and to develop a recommendation outlining clinical guidelines for soft tissue sarcoma. METHODS: We searched the pertinent literature from January 1985 to December 2017. Two reviewers evaluated and screened the literature independently for eligibility and extracted data. We evaluated the quality of body of evidence and made a recommendation according to the Grading of Recommendations Development and Evaluation methodology. RESULTS: Among 244 identified studies, only 10 were finally included in this review and no randomized controlled trial reports were present. The median survival period after metastasectomy ranged from 9.6 to 39.6 months, and the 5-year survival rate ranged from 8 to 52%. The complication rate ranged from 7.3 to 25%, and the perioperative mortality rate was 0-1%. The guidelines committee proposed 'Metastasectomy can be offered for malignant soft tissue tumours with distant metastases'. This recommendation gained 100% consensus among the members of the guidelines group. CONCLUSIONS: Although the level of evidence is very low, many retrospective studies support a clinical advantage for metastasectomy, and surgical indications should be carefully considered for patients with metastasis from soft tissue sarcoma. Metastasectomy is an option for patients with metastasis and should be done only if it can be performed safely and if potential advantages outweigh disadvantages.


Assuntos
Metastasectomia , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/cirurgia , Humanos , Sarcoma/patologia , Neoplasias de Tecidos Moles/patologia
15.
Acta Orthop Traumatol Turc ; 55(2): 154-158, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33847578

RESUMO

OBJECTIVE: This study aimed to investigate amputation-related factors after limb-salvage surgery (LSS) in patients with extremity-located bone and soft-tissue sarcomas and determine the relationship between these factors and patient survival. METHODS: In this retrospective study at our institution, patients in whom LSS was first performed because of an extremity-located musculoskeletal sarcoma, and subsequently amputation was carried out for various indications were included. Patient and tumor characteristics, details of surgical procedures, indications of amputation, number of operations, presence of metastasis before amputation, and post-amputation patient survival rates were analyzed. RESULTS: A total of 25 patients (10 men, 15 women; mean age=41.96±21.88 years), in whom amputation was performed after LSS as initial resection of an extremity sarcoma or re-resection(s) of a local recurrence, were included in the study. The leading oncological indication for amputation was local recurrence that occurred in 18 (72%) patients. Non-oncological indications included prosthetic infection in 5 (20%), mechanical failure in 1 (4%), and skin necrosis in 1 (4%) patient. The patients underwent a median of 2 (range, 1-4) limb-salvage procedures before amputation. Distant organ metastasis was detected in 22 (88%) patients during follow-up; in 13 (52%) of these patients, metastasis was present before amputation. A total of 11 (44%) patients were alive at the time of study with no evidence of the disease (n=3) or with disease (n=8), and 14 (56%) patients died of disease. The mean overall and post-amputation survival were 47±20.519 (range, 11-204) months and 22±4.303 (range, 2-78) months, respectively. The median follow-up was 27 (range, 6-125) months. CONCLUSION: The most common causes of amputation after LSS were local recurrence and prosthetic infection. Patients who underwent amputation after LSS developed a high rate of distant organ metastasis during follow-up and had reduced survival. LEVEL OF EVIDENCE: Level IV, Therapeutic Study.


Assuntos
Amputação , Neoplasias Ósseas , Extremidades , Salvamento de Membro , Recidiva Local de Neoplasia , Sarcoma , Adulto , Amputação/métodos , Amputação/estatística & dados numéricos , Neoplasias Ósseas/patologia , Neoplasias Ósseas/cirurgia , Extremidades/patologia , Extremidades/cirurgia , Feminino , Humanos , Salvamento de Membro/efeitos adversos , Salvamento de Membro/métodos , Masculino , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/cirurgia , Avaliação de Processos e Resultados em Cuidados de Saúde , Estudos Retrospectivos , Sarcoma/patologia , Sarcoma/cirurgia , Taxa de Sobrevida , Fatores de Tempo
16.
Anticancer Res ; 41(4): 1927-1935, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33813398

RESUMO

BACKGROUND/AIM: To identify prognostic imaging biomarkers from staging chest computed tomography (CT) in patients with sarcomas. PATIENTS AND METHODS: CT scans for baseline staging, and surveillance 1-year CT scans in patients newly diagnosed with sarcoma were evaluated. Pectoralis muscle area (PMA), pectoralis muscle index (PMI) and pectoralis CT attenuation density (PMT) were measured. Cox proportional-hazard models were used to determine the association with progression-free survival (PFS) and overall survival (OS). RESULTS: There were 147 patients (53.1% male) who were followed for a median 1,414 days (range=219-4851 days). Approximately 47.6% (70/147) of patients progressed and 29.9% (44/147) died. Multivariable Cox-proportional hazards models adjusting for gender, tumor grade and chemotherapy treatment showed that a higher baseline PMT and baseline PMI were associated with increased OS. CONCLUSION: Higher baseline PMI and PMT are associated with increased overall survival in patients with sarcoma.


Assuntos
Neoplasias Ósseas/diagnóstico , Músculos Peitorais/diagnóstico por imagem , Sarcoma/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Anatomia Transversal/métodos , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/patologia , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculos Peitorais/patologia , Prognóstico , Radiografia Torácica/métodos , Estudos Retrospectivos , Sarcoma/tratamento farmacológico , Sarcoma/mortalidade , Sarcoma/patologia , Neoplasias de Tecidos Moles/tratamento farmacológico , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Análise de Sobrevida
17.
BMC Cancer ; 21(1): 398, 2021 Apr 13.
Artigo em Inglês | MEDLINE | ID: mdl-33849475

RESUMO

BACKGROUND: Soft-tissue sarcomas (STS) are rare tumors of the soft tissue. Recent diagnostic studies on STS mainly dealt with only few cases of STS and did not investigate the post-therapeutic performance of MRI in a routine clinical setting. Therefore, we assessed the long-term diagnostic accuracy of MRI for detecting recurrent STS at a multidisciplinary sarcoma center. METHODS: In all, 1055 postoperative follow-up MRIs of 204 patients were included in the study. MRI follow-up scans were systematically reviewed for diagnostic values (true-positive/-negative and false-positive/-negative results) in detecting recurrences. Pathological reports and follow-up MRIs were set as baseline references. RESULTS: The median age of the patients was 55.3 ± 18.2 years. Of the patients, 34.8% presented with recurrences. Here, 65 follow-up scans were true positive, 23 false positive, 6 false negative, and 961 true negative. The overall sensitivity and specificity of MRI for detecting recurrences were 92 and 98%, respectively, with an accuracy of 97%. For intramuscular lesions and after surgery alone the sensitivity was higher (95 and 97%, respectively) than for subcutaneous lesions and surgery with additional radiation therapy (83 and 86%, respectively), at similarly high specificities (96-98%). The 6 false-negative results were found in streaky (n = 2) and small ovoid/nodular (n = 4) recurring lesions. The false-positive lesions imitated streaky (n = 14), ovoid/nodular (n = 8), and polycyclic/multilobulated recurring tumors (n = 1). All false-positive results were found in patients in whom the primary tumors were polycyclic/multilobulated in appearance. CONCLUSION: MRI shows a high diagnostic accuracy for detecting recurrent STS, with a high sensitivity and specificity. The diagnostic accuracy decreases in subcutaneous lesions and after surgery with radiation therapy, compared to intramuscular lesions and surgery alone. Radiologists should pay particular attention to streaky and small ovoid/nodular recurring lesions and patients with polycyclic/multilobulated primary tumors.


Assuntos
Imageamento por Ressonância Magnética , Sarcoma/diagnóstico por imagem , Sarcoma/patologia , Neoplasias de Tecidos Moles/diagnóstico por imagem , Neoplasias de Tecidos Moles/patologia , Adulto , Idoso , Feminino , Seguimentos , Humanos , Imageamento por Ressonância Magnética/métodos , Imageamento por Ressonância Magnética/normas , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Recidiva Local de Neoplasia , Estadiamento de Neoplasias , Período Pós-Operatório , Prognóstico , Sarcoma/cirurgia , Sensibilidade e Especificidade , Neoplasias de Tecidos Moles/cirurgia , Resultado do Tratamento
18.
J Cancer Res Clin Oncol ; 147(7): 2003-2011, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33811537

RESUMO

BACKGROUND: Undifferentiated pleomorphic sarcoma (UPS) is a sarcoma with a poor prognosis. A clinical trial, SARC028, revealed that treatment with anti-PD-1 drugs was effective against UPS. Studies have reported that UPS expresses PD-L1, sometimes strongly (≥ 50%). However, the mechanism of PD-L1 expression in UPS has remained unclear. CKLF-like MARVEL transmembrane domain containing 6 (CMTM6) was identified as a novel regulator of PD-L1 expression. The positive relationship between PD-L1 and CMTM6 has been reported in several studies. The aim of this study was thus to examine CMTM6 expression in UPS and evaluate the relationship between PD-L1 and CMTM6 in this disease. MATERIALS AND METHODS: Fifty-one primary UPS samples were subjected to CMTM6 and PD-L1 immunostaining. CMTM6 expression was assessed using proportion and intensity scores. CMTM6 gene copy number was also evaluated using a real-time PCR-based copy number assay. We also analyzed the mRNA expression and copy number variation of PD-L1 and CMTM6 in The Cancer Genome Atlas (TCGA) data. RESULTS: TCGA data indicated that the mRNAs encoded by genes located around 3p22 were coexpressed with CMTM6 mRNA in UPS. Both proportion and intensity scores of CMTM6 positively correlated with strong PD-L1 expression (≥ 50%) (both p = 0.023). CMTM6 copy number gain increased CMTM6 expression. Patients with UPS with a high CMTM6 intensity score had a worse prognosis for overall survival. CONCLUSIONS: UPS showed variation in CMTM6 copy number and CMTM6 expression. CMTM6 expression was significantly correlated with PD-L1 expression, especially with strong PD-L1 expression.


Assuntos
Antígeno B7-H1/genética , Biomarcadores Tumorais/genética , Variações do Número de Cópias de DNA , Regulação Neoplásica da Expressão Gênica , Proteínas com Domínio MARVEL/genética , Proteínas da Mielina/genética , Sarcoma/genética , Sarcoma/patologia , Idoso , Feminino , Seguimentos , Humanos , Masculino , Prognóstico , Taxa de Sobrevida
19.
Int J Surg Oncol ; 2021: 8871557, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33833875

RESUMO

Soft-tissue sarcomas are malignant tumors that require good management within specialized centers. Our study aims to assess the benefit of handling these kinds of tumors using the Multidisciplinary Meeting (MDM) approach. The current paper details this approach through a prospective study that has lasted for 42 months in the HASSAN II University Hospital Center, Fez, Morocco. During this research work, 116 cases were selected with an average age of 53 years. In 95.7% of the cases, it was found that the lower limb was the most frequent tumor type (78.4%). Also, ninety-two (92) patients (79.3%) have had a prior biopsy. Ninety-nine (99) patients (85.3%) have received a magnetic resonance imaging scan (MRI) before surgery. Sixty-three (63) patients were operated on, including R0 resection used for 37 patients, R1 used for 21 patients, and R2 used for five patients. As a result, liposarcomas were the most frequent type (30.1%), followed by synovial sarcomas (14.6%), leiomyosarcomas (9.5%), ewing sarcoma (8.6), and undifferentiated pleomorphic sarcomas (7.7%). In addition, neoadjuvant chemotherapy was used for 36 patients. The other 22 patients received adjuvant chemotherapy and/or radiotherapy. The overall survival rate was 60.56 months, which proves a significant improvement, thanks to the multidisciplinary meeting approach. Conclusion. The conducted investigation has shown that using MDM for managing soft-tissue sarcomas of extremities improves the patients' survival rate. Moreover, results have proven MDM might allow optimal treatment regarding less local recurrence and metastasis.


Assuntos
Comunicação Interdisciplinar , Equipe de Assistência ao Paciente , Sarcoma/diagnóstico , Sarcoma/terapia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/terapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Extremidades/patologia , Extremidades/cirurgia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos , Sarcoma/mortalidade , Sarcoma/patologia , Neoplasias de Tecidos Moles/mortalidade , Neoplasias de Tecidos Moles/patologia , Adulto Jovem
20.
Vet Clin Pathol ; 50(2): 236-239, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33797110

RESUMO

Currently, canine soft tissue sarcoma (STS) grading is based on histopathology. In humans, several studies have demonstrated concordance between cytologic grading systems for STS and histologic grade. The aim of this study was to correlate several cytologic parameters (smear cellularity, anisokaryosis, nucleolar malignancy score, multinucleation, and the number of mitotic figures per 200 cells) that form part of a human STS cytologic grading system, with histologic grades of canine cutaneous and subcutaneous STS. Three observers (blinded) reviewed the cytologic preparations independently from cases with confirmed histologic diagnoses of STS. A cytologic grading score was assigned for each parameter. Correlations between cytologic grading scores (averaged between observers) and histologic grades were assessed using Spearman's correlation coefficient, with statistical significance defined as P < .05. Twenty-one cases were included in the study (10 Grade I STS, nine Grade II STS, and two Grade III STS). The number of mitotic figures (≥3) per 200 cells was the only parameter that showed a significant but weak, positive correlation with histologic grade (rs  = .469; P = .032). No Grade I tumors had ≥3 mitotic figures per 200 cells; however, ≥3 mitotic figures per 200 cells were only observed in 33% of Grade II tumors and 50% (one out of two) of the Grade III tumors. This pilot study suggests that an increased number of mitotic figures seen on cytology might correlate with higher grade STS; however, the sensitivity of this parameter for grading STS appears to be low.


Assuntos
Doenças do Cão , Sarcoma , Neoplasias de Tecidos Moles , Animais , Citodiagnóstico/veterinária , Doenças do Cão/patologia , Cães , Projetos Piloto , Sarcoma/patologia , Sarcoma/veterinária , Pele/patologia , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/veterinária
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