RESUMO
The first Lancet Oncology Commission on Global Cancer Surgery was published in 2015 and serves as a landmark paper in the field of cancer surgery. The Commission highlighted the burden of cancer and the importance of cancer surgery, while documenting the many inadequacies in the ability to deliver safe, timely, and affordable cancer surgical care. This Commission builds on the first Commission by focusing on solutions and actions to improve access to cancer surgery globally, developed by drawing upon the expertise from cancer surgery leaders across the world. We present solution frameworks in nine domains that can improve access to cancer surgery. These nine domains were refined to identify solutions specific to the six WHO regions. On the basis of these solutions, we developed eight actions to propel essential improvements in the global capacity for cancer surgery. Our initiatives are broad in scope, pragmatic, affordable, and contextually applicable, and aimed at cancer surgeons as well as leaders, administrators, elected officials, and health policy advocates. We envision that the solutions and actions contained within the Commission will address inequities and promote safe, timely, and affordable cancer surgery for every patient, regardless of their socioeconomic status or geographic location.
Assuntos
Neoplasias , Cirurgiões , Humanos , Neoplasias/cirurgia , Saúde Global , Política de SaúdeRESUMO
BACKGROUND: The COVID-19 outbreak has resulted in collision between patients infected with SARS-CoV-2 and those with cancer on different fronts. Patients with cancer have been impacted by deferral, modification, and even cessation of therapy. Adaptive measures to minimize hospital exposure, following the precautionary principle, have been proposed for cancer care during COVID-19 era. We present here a consensus on prioritizing recommendations across the continuum of sarcoma patient care. MATERIAL AND METHODS: A total of 125 recommendations were proposed in soft-tissue, bone, and visceral sarcoma care. Recommendations were assigned as higher or lower priority if they cannot or can be postponed at least 2-3 months, respectively. The consensus level for each recommendation was classified as "strongly recommended" (SR) if more than 90% of experts agreed, "recommended" (R) if 75%-90% of experts agreed and "no consensus" (NC) if fewer than 75% agreed. Sarcoma experts from 11 countries within the Sarcoma European-Latin American Network (SELNET) consortium participated, including countries in the Americas and Europe. The European Society for Medical Oncology-Magnitude of clinical benefit scale was applied to systemic-treatment recommendations to support prioritization. RESULTS: There were 80 SRs, 35 Rs, and 10 NCs among the 125 recommendations issued and completed by 31 multidisciplinary sarcoma experts. The consensus was higher among the 75 higher-priority recommendations (85%, 12%, and 3% for SR, R, and NC, respectively) than in the 50 lower-priority recommendations (32%, 52%, and 16% for SR, R, and NC, respectively). CONCLUSION: The consensus on 115 of 125 recommendations indicates a high-level of convergence among experts. The SELNET consensus provides a tool for sarcoma multidisciplinary treatment committees during the COVID-19 outbreak. IMPLICATIONS FOR PRACTICE: The Sarcoma European-Latin American Network (SELNET) consensus on sarcoma prioritization care during the COVID-19 era issued 125 pragmatical recommendations distributed as higher or lower priority to protect critical decisions on sarcoma care during the COVID-19 pandemic. A multidisciplinary team from 11 countries reached consensus on 115 recommendations. The consensus was lower among lower-priority recommendations, which shows reticence to postpone actions even in indolent tumors. The European Society for Medical Oncology-Magnitude of Clinical Benefit scale was applied as support for prioritizing systemic treatment. Consensus on 115 of 125 recommendations indicates a high level of convergence among experts. The SELNET consensus provides a practice tool for guidance in the decisions of sarcoma multidisciplinary treatment committees during the COVID-19 outbreak.
Assuntos
COVID-19/epidemiologia , Oncologia/organização & administração , Oncologia/normas , Sarcoma/terapia , COVID-19/prevenção & controle , Consenso , Europa (Continente)/epidemiologia , Humanos , América Latina/epidemiologia , Assistência ao Paciente/normas , Guias de Prática Clínica como Assunto , SARS-CoV-2 , Sarcoma/diagnósticoRESUMO
INTRODUCTION:: Acral lentiginous melanoma (ALM) is an aggressive variant of melanoma; the incidence, prevalence, and prognosis differ among populations. We analyzed clinicopathological features and survival in Hispanics, a population with high ALM prevalence. MATERIAL AND METHODS:: From 1144 patients with melanoma, we analyzed 715 ALMs and 429 non-ALMs from the National Referral Cancer Centre and compared survival. RESULTS:: Of the ALM group, 62.8% were female; the median age was 58 years. The mean Breslow thickness was 3.56 ± 7.16 mm. ALM patients showed an estimated 5-year disease-specific survival (DSS) of 53.3%, 52.7%, and 40.8% for stage I, II, and III, respectively. For non-ALM patients, the DSS rates were 66%, 60.8%, and 48.4% for stage I, II, and III disease, respectively. Overall, the 1-, 3-, and 5-year DSS rates for patients with ALM were 85.1%, 59.4%, and 46.3%, respectively; for non-ALM patients, they were 81.3%, 64.8%, and 55.7%, respectively ( P = .168). In the multivariate analysis, factors associated with decreased DSS were high Breslow thickness, recurrence, ulceration, male sex, and advanced stage. CONCLUSIONS:: The 1-, 3-, and 5-year DSS rates of patients with ALM were not statistically different from those of non-ALM patients. In addition to known adverse prognostic factors, male sex was also associated with worse survival.
Assuntos
Melanoma/mortalidade , Idoso , Feminino , Humanos , Masculino , Melanoma/diagnóstico , Melanoma/epidemiologia , México/epidemiologia , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Análise de SobrevidaRESUMO
BACKGROUND: The aim of this study was to analyze the clinicopathologic features of melanoma in México as the demographics of melanoma are not well known in Mexican and Latin American people. MATERIALS AND METHODS: A total of 1219 patients with cutaneous melanoma were analyzed through a retrospective database collected from a cancer referral institute, and the results were compared with developed countries. RESULTS: Median age was 57 years, and 713 (58.5 %) were females. The most common melanoma subtype was acral lentiginous melanoma (ALM), which was observed in 538 (44.1 %) patients. Median Breslow thickness was 5.2 mm. Among 837 patients with complete data, the 5-year disease-specific survival (DSS) was 52.3 %. Factors associated with worse DSS on univariate analysis were Breslow thickness (p < 0.001), recurrence (p < 0.001), ulceration (p < 0.001), positive margin (p < 0.001), ALM (p = 0.001), and male sex (p = 0.001). Multivariate analysis demonstrated Breslow thickness [hazard ratio (HR) 1.45, 95 % confidence interval (CI) 1.27-1.65; p < 0.001], positive margin (HR 1.25, 95 % CI 1.03-1.57; p = 0.018), recurrence (HR 9.56, 95 % CI 6.89-9.87; p = 0.002), ALM (HR 8.07, 95 % CI 6.77-8.95; p = 0.004), and male sex (HR 1.33, 95 % CI 1.06-1.68; p = 0.013) as independent risk factors for DSS. CONCLUSION: Our patients had worse prognosis compared with data from the US Surveillance, Epidemiology, and End Results database. We found male sex and ALM as independent risk factors for worse survival, in addition to known risk factors.
Assuntos
Sarda Melanótica de Hutchinson/patologia , Recidiva Local de Neoplasia , Neoplasias Cutâneas/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Extremidades , Feminino , Humanos , Sarda Melanótica de Hutchinson/epidemiologia , Sarda Melanótica de Hutchinson/mortalidade , Incidência , Masculino , México/epidemiologia , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/mortalidade , Estadiamento de Neoplasias , Neoplasia Residual , Estudos Retrospectivos , Fatores de Risco , Fatores Sexuais , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/mortalidade , Taxa de Sobrevida , Carga Tumoral , Adulto JovemRESUMO
BACKGROUND: Isolated limb perfusion (ILP) is a well-established surgical procedure for the administration of high dose chemotherapy to a limb for the treatment of advanced extremity malignancy. Although the technique of ILP was first described over 60 years ago, ILP is utilised in relatively few specialist centres, co-located with tertiary or quaternary cancer centres. The combination of high dose cytotoxic chemotherapy and the cytokine tumour necrosis factor alpha (TNFα), mandates leakage monitoring to prevent potentially serious systemic toxicity. Since the procedure is performed at relatively few specialist centres, an ILP working group was formed with the aim of producing technical consensus guidelines for the procedure to streamline practice and to provide guidance for new centres commencing the technique. METHODS: Between October 2021 and October 2023 a series of face to face online and hybrid meetings were held in which a modified Delphi process was used to develop a unified consensus document. After each meeting the document was modified and recirculated and then rediscussed at subsequent meeting until a greater than 90% consensus was achieved in all recommendations. RESULTS: The completed consensus document comprised 23 topics in which greater than 90% consensus was achieved, with 83% of recommendations having 100% consensus across all members of the working group. The consensus recommendations covered all areas of the surgical procedure including pre-operative assessment, drug dosing and administration, perfusion parameters, hyperthermia, leakage monitoring and theatre logistics, practical surgical strategies and also post-operative care, response evaluation and staff training. CONCLUSION: We present the first joint expert-based consensus statement with respect to the technical aspects of ILP that can serve as a reference point for both existing and new centres in providing ILP.
Assuntos
Quimioterapia do Câncer por Perfusão Regional , Extremidades , Humanos , Quimioterapia do Câncer por Perfusão Regional/métodos , Consenso , Técnica Delphi , Extremidades/irrigação sanguínea , Neoplasias , Fator de Necrose Tumoral alfaRESUMO
Acral melanoma, which is not ultraviolet (UV)-associated, is the most common type of melanoma in several low- and middle-income countries including Mexico. Latin American samples are significantly underrepresented in global cancer genomics studies, which directly affects patients in these regions as it is known that cancer risk and incidence may be influenced by ancestry and environmental exposures. To address this, here we characterise the genome and transcriptome of 128 acral melanoma tumours from 96 Mexican patients, a population notable because of its genetic admixture. Compared with other studies of melanoma, we found fewer frequent mutations in classical driver genes such as BRAF, NRAS or NF1. While most patients had predominantly Amerindian genetic ancestry, those with higher European ancestry had increased frequency of BRAF mutations and a lower number of structural variants. These BRAF-mutated tumours have a transcriptional profile similar to cutaneous non-volar melanocytes, suggesting that acral melanomas in these patients may arise from a distinct cell of origin compared to other tumours arising in these locations. KIT mutations were found in a subset of these tumours, and transcriptional profiling defined three expression clusters; these characteristics were associated with overall survival. We highlight novel low-frequency drivers, such as SPHKAP, which correlate with a distinct genomic profile and clinical characteristics. Our study enhances knowledge of this understudied disease and underscores the importance of including samples from diverse ancestries in cancer genomics studies.
RESUMO
The use of geriatric assessment (GA) by oncology specialists in Mexico is low. We aimed to explore factors associated with the evaluation of individual GA domains by Mexican oncology specialists. We performed an exploratory analysis of a sequential explanatory mixed-methods study consisting of an online cross-sectional survey of Mexican oncology specialists and follow-up interviews on the use of GA in cancer care. For each GA domain, we performed multivariable logistic regression analyses with the frequency of evaluation of the domains as the dependent variable (dichotomised as never/rarely/sometimes versus most of the time/always). A p-value <0.05 was considered significant. Qualitative data from the interviews were analysed inductively. Of 196 respondents, 62% were male, 50% were surgical oncologists, 51% took care of >10 patients per day and 61.7% had access to a geriatrician. Self-perceived confidence in managing common geriatric conditions was associated with the evaluation of specific GA domains. For instance, self-perceived confidence in managing dementia (OR 2.72; 95% CI 1.42-5.51, p = 0.008) was associated with cognition evaluation, while for evaluation of falls, self-perceived confidence in evaluation of falls (OR 6.31; 95% CI 3.19-12.46, p < 0.001) was significantly associated. Follow-up interviews showed quality and appropriateness of evaluations may not be ideal: in many cases, physicians do not use guideline-recommended tools. For example, evaluation of cognition is commonly performed through non-validated methods which may miss the detection of patients with an impairment in this domain, partly due to limitations in knowledge and time to use recommended tools. In conclusion, self-perceived confidence in evaluating and managing common situations in older adults was associated with the evaluation of GA domains as part of everyday practice in a sample of oncology specialists in Mexico. This analysis supports the use of educational interventions to boost knowledge and confidence regarding the proper use of validated GA tools among oncology specialists.
RESUMO
PURPOSE: There is limited information regarding the use of the geriatric assessment (GA) for older adults with cancer in developing countries. We aimed to describe geriatric oncology practice among Mexican oncology professionals and to identify barriers and facilitators for the implementation of GA into the routine care of older adults with cancer in Mexico. METHODS: We conducted an explanatory sequential mixed-methods study. We administered an online survey to cancer specialists in Mexico about the routine use of GA and barriers for its use. We then conducted online semistructured interviews with survey respondents selected by their use of GA, expanding on barriers and facilitators for performing GA. Descriptive statistical analyses were performed for quantitative data; qualitative data were analyzed inductively through thematic analysis. We developed joint displays to integrate quantitative/qualitative results. RESULTS: We obtained 196 survey responses: 37 physicians (18.9%) reported routinely performing a GA. Medical oncologists (P = .002) and physicians seeing ≤ 10 patients/day (P = .010) were more likely to use GA. The most frequent barriers for GA use were lack of qualified personnel (49%), limited knowledge (43.9%), and insufficient time (37.2%). In the interviews (n = 22), the limited availability of geriatricians was commonly mentioned. Respondents highlighted the lack of geriatric oncology knowledge among cancer specialists and geriatricians. Saturation of oncology services and a lack of effective referral pathways for GA were also common issues. Facilitators included availability of geriatricians, system/administrative facilitators, presence of a multidisciplinary team, and availability of geriatric oncology education. CONCLUSION: The routine use of geriatric oncology principles in Mexico is limited by the availability of qualified personnel and by insufficient knowledge. An educational intervention could improve the implementation of GA in cancer care.
Assuntos
Oncologia , Neoplasias , Idoso , Avaliação Geriátrica/métodos , Geriatras , Humanos , México , Neoplasias/terapiaRESUMO
OBJECTIVE: To identify the scientific literature on cutaneous melanoma in Latin America and compile all available epidemiologic data to demonstrate the need for reliable regional and country-specific data on incidence and mortality estimates. METHODS: Literature searches were conducted in PubMed, Embase, LILACS, and Google Scholar databases for epidemiologic studies from 1 January 2000 to 31 October 2010 related to melanoma in Argentina, Brazil, Colombia, Mexico, Puerto Rico, and Venezuela. A final search on melanoma cases was carried out using country-specific population-based cancer registries. No statistical analyses were conducted. RESULTS: For all six countries, most epidemiological research on cutaneous melanoma consists of hospital-based or case-control studies. Very few studies report incidence and mortality rates. Attempts to estimate disease rates have relied on national incidence and mortality data and information extracted from cancer registries. While predominance of European ancestry is a known risk factor for developing melanoma, the association of melanoma and ethnicity is not well-documented in some of the populations reviewed. Latin Americans are frequently exposed to ultraviolet (UV) radiation due to the tropical weather, high altitude, and thinning ozone layer in some regions. Tanned skin is viewed as healthy and beautiful. While melanoma public health campaigns have been under way in Latin America for decades, increasing melanoma awareness remains imperative. CONCLUSIONS: There is an urgent need to collect accurate epidemiologic melanoma data in Latin America. Future research in the region should include more comprehensive, country-specific, population-based studies to allow for comparative evaluation of incidence and mortality rates.
Assuntos
Melanoma/epidemiologia , Neoplasias Cutâneas/epidemiologia , Estudos de Casos e Controles , Estudos de Coortes , Projetos de Pesquisa Epidemiológica , Educação em Saúde , Necessidades e Demandas de Serviços de Saúde , Humanos , Incidência , América Latina/epidemiologia , Neoplasias Induzidas por Radiação/epidemiologia , Fatores de Risco , Luz Solar , Raios Ultravioleta/efeitos adversosRESUMO
Acral melanoma (AM) is a malignant cutaneous melanocytic tumour specifically located on the palms, soles, and nail apparatus, which are areas of glabrous (hairless) skin. Acral lentiginous melanoma, a subtype of AM, represents a histopathological subtype diagnosis of cutaneous melanoma with unique morphological and structural features. Despite clear definitions, the misuse of these terms and the inconsistency in reporting the histopathological features of AM cases have become a major obstacle to the study of the disease. In this review, we discuss the epidemiology, histopathological features, prognosis, and genetic profile of AM, highlighting the differences observed when histopathological subtypes are considered. The increasing global effort to characterise AM cases from ethnically diverse populations would benefit greatly from a more consistent classification of the disease.
Assuntos
Melanoma , Neoplasias Cutâneas , Animais , Biomarcadores Tumorais/genética , Pé/patologia , Mãos/patologia , Humanos , Melanoma/epidemiologia , Melanoma/genética , Melanoma/patologia , Unhas/patologia , Prognóstico , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/patologiaRESUMO
Although breast conserving surgery is the standard of care for patients with localized breast cancer in high-income countries, little is known about its use in developing countries, where disparities in access to treatment may lead to an increased use of mastectomy. We examined the use of breast conserving surgery at a Mexican cancer center after the implementation of a public insurance program aimed at providing coverage for previously uninsured patients. Between 2006 and 2016, 4519 women received surgical treatment for breast cancer, of which 39% had early-stage disease. The proportion of patients treated with breast conserving surgery increased from 10% in the 2006-2009 period to 33% in the 2013-2016 period, with most of this increase occurring among women with early-stage disease (17-52%). Improving access to care and reducing the financial burden of breast cancer in developing countries may lead to an increased use of breast conserving surgery.
Assuntos
Neoplasias da Mama/cirurgia , Seguro Saúde/estatística & dados numéricos , Mastectomia Segmentar/tendências , Adulto , Idoso , Neoplasias da Mama/tratamento farmacológico , Neoplasias da Mama/patologia , Feminino , Humanos , México , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Estadiamento de Neoplasias , Estudos RetrospectivosRESUMO
INTRODUCTION: Lymph node metastasis (LNM) in soft tissue sarcomas (STS) are uncommon, occurring in only 3% - 5% of all sarcomas, and are classified as Stage IV, along with distant metastasis (DM). This paper compares the prognosis of patients with lymphatic and DM, in extremity STS (eSTS). METHODS: A retrospective study was carried out in a high-volume sarcoma center; 853 patients with eSTS sarcomas were identified and classified from January 1, 1997 to December 31, 2017. Cases with pathological confirmation of LNM were included. Five-year survival rates were analyzed using the Kaplan-Meier method and the Cox proportional hazards model. RESULTS: LNM was present in 46 of the cases (5.4%), with an overall survival of 21 months (95% confidence interval [CI], 16.7 - 25.2), compared to 18 months (95% confidence interval [CI], 14.2 - 21.7) in those with only DM. Median recurrence-free survival was 21 months (95% confidence interval [CI], 19.7 - 22.4), vs. 20 months (95% confidence interval [CI], 16.2- 23.7), respectively. LNM only and DM only had also a similar OS of 21 months (95% CI 16.7-25.2) vs 18 months (95% CI 14.2-21.7. N1M1 cases had the worse median OS with 15 months (95% confidence interval [CI], 10.9-19.7) CONCLUSIONS: Overall survival and recurrence free survival in patients with lymph node disease and metastatic disease are similar. However prognosis is worse in N1M1. Use of systemic treatment in patients with LNM is not as common as in metastatic cases, this difference in treatment and the fact that prognosis is similar suggests that both biological behavior and effect of treatment have been underestimated. A subclassification of clinical stage IV might be the next step.
Assuntos
Linfonodos/patologia , Metástase Linfática/diagnóstico , Recidiva Local de Neoplasia/epidemiologia , Sarcoma/diagnóstico , Adulto , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Sarcoma/mortalidade , Sarcoma/secundário , Sarcoma/terapia , Taxa de SobrevidaRESUMO
Acral lentiginous melanoma is a histological subtype of cutaneous melanoma that occurs in the glabrous skin of the palms, soles and the nail unit. Although in some countries, particularly in Latin America, Africa and Asia, it represents the most frequently diagnosed subtype of the disease, it only represents a small proportion of melanoma cases in European-descent populations, which is partially why it has not been studied to the same extent as other forms of melanoma. As a result, its unique genomic drivers remain comparatively poorly explored, as well as its causes, with current evidence supporting a UV-independent path to tumorigenesis. In this review, we discuss current knowledge of the aetiology and diagnostic criteria of acral lentiginous melanoma, as well as its epidemiological and histopathological characteristics. We also describe what is known about the genomic landscape of this disease and review the available biological models to explore potential therapeutic targets.
Assuntos
Doenças do Pé/patologia , Melanócitos/patologia , Melanoma/patologia , Doenças da Unha/patologia , Neoplasias Cutâneas/patologia , HumanosRESUMO
BACKGROUND: Soft tissue sarcomas (STS) are a heterogeneous group of rare tumours that represent less than 1% of all malignant, solid tumours in adults. There is limited epidemiological information regarding STS in Latin America. Therefore, the objective of this study is to present an epidemiological profile of these tumours observed at a single reference centre. METHODS: A retrospective study was carried out based on hospital records obtained from a registry of 879 patients with STS of the extremities who were treated at the National Cancer Institute of Mexico from January 1, 1994 to December 31, 2017. Epidemiological variables and relevant clinical data were collected. Five-year survival rates were analysed using Kaplan-Meier estimates, and a multivariate Cox proportional-hazards model measured associations. RESULTS: A total of 879 records were collected. The median age was 45 years (15-95 years), and the ratio of men to women was 1:1, with 433 men (49.3%), and 446 women (50.7%). The median tumour size was 11.4 cm (2-49 cm). The most prevalent histological variants were liposarcomas and synovial sarcomas. The lower limb was the most frequently affected extremity, with the thigh being the most common site followed by the leg. A majority of the patients were diagnosed at clinical stages IIIA-IV. CONCLUSIONS: The data collected from the present cohort provides an overview of the epidemiological profile of STS at a single reference centre in Latin America, and allow comparison with global data.
RESUMO
To date, the impact, timeline and duration of COVID-19 pandemic remains unknown and more than ever it is necessary to provide safe pathways for cancer patients. Multiple triage systems for nonemergent surgical procedures have been published, but potentially curative cancer procedures are essential surgery rather than elective surgery. In the present and future scenario of our country, thoracic oncology teams may have the difficult decision of weighing the utility of surgical intervention against the risk for inadvertent COVID-19 exposure for patients and medical staff. In consequence, traditional pathways of surgical care must be adjusted to reduce the risk of infection and the use of resources. It is recommended that all thoracic cancer patients should be offered treatment according to the accepted standard of care until shortage of services require a progressive reduction in surgical cases. Here, we present a consensus of recommendations discussed by a multidisciplinary panel of experts on thoracic oncology and based on the best available evidence, and hope it will provide a modifiable framework of guidance for local strategy planners in thoracic cancer care services in Mexico. KEY POINTS: SIGNIFICANT FINDINGS OF THE STUDY: This article provides recommendations to guarantee the continuity of surgical care for thoracic oncology cases during COVID-19 pandemic, whilst maintaining the safety of patients and medical staff. WHAT THIS STUDY ADDS: This guideline is the result of an expert consensus on thoracic surgical oncology with recommendations adapted to medical, economic and social realities of Mexico.
Assuntos
COVID-19/epidemiologia , Neoplasias Pulmonares/epidemiologia , Pandemias , Neoplasias Torácicas/epidemiologia , COVID-19/complicações , COVID-19/virologia , Guias como Assunto , Humanos , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/cirurgia , Neoplasias Pulmonares/virologia , Oncologia/tendências , México/epidemiologia , SARS-CoV-2/patogenicidade , Neoplasias Torácicas/complicações , Neoplasias Torácicas/cirurgia , Neoplasias Torácicas/virologia , TriagemRESUMO
Desmoid tumors represent a rare entity of monoclonal origin characterized by locally aggressive behavior and inability to metastasize. Most cases present in a sporadic pattern and are characterized by a mutation in the CTNNB1 gene; while 5-15% show a hereditary pattern associated with APC gene mutation, both resulting in abnormal ß-catenin accumulation within the cell. The most common sites of presentation are the extremities and the thoracic wall, whereas FAP associated cases present intra-abdominally or in the abdominal wall. Histopathological diagnosis is mandatory, and evaluation is guided with imaging studies ranging from ultrasound, computed tomography or magnetic resonance. Current approaches advocate for an initial active surveillance period due to the stabilization and even regression capacity of desmoid tumors. For progressive, symptomatic, or disabling cases, systemic treatment, radiotherapy or surgery may be used. This is a narrative review of this uncommon disease; we present current knowledge about molecular pathogenesis, diagnosis and treatment.
RESUMO
BACKGROUND: Synovial sarcoma is a rare malignant soft tissue tumor, more common in adolescents and young adults and entails a poor prognosis. Several good prognostic factors have been well established such as age less than 25, size less than 5â¯cm and absence of a poorly differentiated component. Inflammation has a well-established role in tumor proliferation and survival. The aim of this study was to investigate the prognostic significance of the neutrophil/lymphocyte ratio (NLR) in a large cohort of synovial sarcoma patients. METHODS: Retrospective study of 169 consecutive patients. We analyzed the relation of preoperative NLR on disease-free survival (DFS) and overall survival (OS) using Kaplan-Meier curves and Cox proportional models. RESULTS: Of the 169 patients included, there were 90(53.3%) females and 79(46.7%) males. Median age was 32yo (11-73). Median survival was 34.1 and mean disease-free survival was 21.4 months. Mean tumor size was 12.5â¯cm (1.2-77â¯cm). Applying receiver operating curve analysis, we determined a cut-off value of 3.5. In univariate and multivariate analysis, increased NLR was significantly associated with poor OS. A <3.5 NLR was an independent prognostic factor in all stages (pâ¯=â¯0.002). CONCLUSIONS: NLR >3.5 was found to be a reliable prognostic factor in this cohort. Given its widespread availability, we believe it's use in clinical practice and further clinical trials should be considered.
Assuntos
Linfócitos/patologia , Neutrófilos/patologia , Sarcoma Sinovial/mortalidade , Sarcoma Sinovial/patologia , Adolescente , Adulto , Idoso , Criança , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Metástase Neoplásica , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Adulto JovemRESUMO
PURPOSE: The BRAF V600E mutation has been described in melanomas occurring in the Caucasian, European, and Asian populations. However, in the Mexican population, the status and clinical significance of BRAF mutation has not been researched on a large scale. METHODS: Consecutive BRAF-tested Mexican patients with metastatic melanoma (n = 127) were analyzed for mutations in exon 15 of the BRAF gene in genomic DNA by real-time polymerase chain reaction technology for amplification and detection. The results were correlated with the clinical-pathologic features and the prognosis of the patients. RESULTS: The frequency of somatic mutation V600E within the BRAF gene was 54.6% (43 of 127 patients). Nodular melanoma was the most prevalent subtype in our population, with BRAF mutations in 37.2% (16 of 55 patients). In contrast, superficial spread had a frequency of 18.6% BRAF mutation (eight of 24). Other clinicopathologic features were assessed to correlate with the mutation status. CONCLUSION: This study searched for the most prevalent BRAF V600E mutation type in melanoma in a heterogeneous population from Mexico. Nodular melanoma was found to be the most prevalent in metastatic presentation and the presence of BRAF V600E mutation, perhaps related to the mixed ancestry; in the north, ancestry is predominantly European and in the south, it is predominantly Asian. The outcomes of the mutation correlations were similar to those found in other populations.