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BACKGROUND: Merkel cell carcinoma (MCC) comprises a rare malignant primary skin tumor presenting neuroendocrine differentiation. Recently, agents blocking the programmed cell death protein 1 and programmed cell death protein ligand 1 pathway (PD-1/PD-L1) have demonstrated objective and durable tumor regressions in patients presenting advanced MCC. This study aimed to describe the sociodemographic, clinical, and histopathological characteristics of MCC patients, also assessing the prevalence of PD-L1 expression and Merkel cell Polyomavirus (MCPyV), as well as their prognostic roles. METHODS: Data from patients diagnosed with MCC between 1996 and 2019 at a reference cancer center in Rio de Janeiro, southeastern Brazil, were evaluated in a retrospective study. Tumor samples were tested for MCPyV and PD-L1 employing immunohistochemistry. Survival analyses were carried out employing the Kaplan-Meier method and curves were compared using the log-rank test. A multiple semiparametric Cox model was used. Values p < 0.05 were considered significant. RESULTS: A total of 65 patients were included in the study, with a mean age at diagnosis of 72 (standard deviation 13.9). A total of 56.9% (37/65) of the patients were male, 86.2% (56/65) were white, and 56.9% (37/64) were illiterate or with incomplete elementary school. MCPyV immunohistochemistry was positive in 29 cases (44.6%) and PD-L1 positivity was ≥ 1% in 42 cases (64.6%). Significant associations between MCPyV and PD-L1 expression ≥ 1% (p = 0.003) and PD-L1 expression ≥ 5% (p = 0.005) were noted. Concerning the multivariate analysis, only education level and advanced MCC stage indicated statistically significant worse progression-free survival. Regarding overall survival (OS), being male, education level and advanced stage comprised risk factors. The estimated OS at 60 months for stages I to III was of 48.9% and for stage IV, 8.9%. CONCLUSIONS: This is the first large Brazilian cohort to assess the prevalence of MCPyV in MCC tumors, as well as PD-L1 expression and their associations. No correlations were noted between MCPyV infection or PD-L1 expression and survival rates.
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Introducción. El carcinoma de Merkel es un tumor maligno poco frecuente, que afecta principalmente a la población caucásica y cuya etiología guarda relación con el poliomavirus de las células de Merkel. Conlleva mal pronóstico, especialmente en estadios finales. Caso clínico. Se expone el caso de una paciente que presentaba un tumor primario facial de grandes dimensiones, con avanzado grado de extensión, afectación linfática cervical y metástasis parotídea derecha. Fue tratada mediante exéresis de la lesión primaria y cobertura con injerto de piel parcial, linfadenectomía cervical y parotidectomía ipsilateral. Resultados. Se logró mejoría importante en la calidad de vida de la paciente y sobrevida de al menos seis meses. Conclusión. Aunque no está claro el manejo óptimo del carcinoma de Merkel avanzado debido a su mal pronóstico, la cirugía favorece una mejoría en la calidad de vida del paciente y puede tener un papel clave en el manejo del carcinoma de Merkel en los estadios avanzados.
Introduction. Merkel carcinoma is a rare malignant tumor that mainly affects the Caucasian population and whose etiology is related to the Merkel cell polyomavirus. It has a poor prognosis, especially in the final stages. Clinical case. The case of a patient who presented a large primary facial tumor, with an advanced degree of extension, cervical lymphatic involvement and right parotid metastasis is described. She was treated surgically by excision of the primary lesion and coverage with partial skin graft, cervical lymphadenectomy, and ipsilateral parotidectomy. Results. A significant improvement was achieved in the patient's quality of life and survival of at least six months.Conclusion. Although the optimal management of advanced Merkel carcinoma is unclear due to its poor prognosis, surgery improves the patient's quality of life and it can play a key role in the management of Merkel carcinoma in advanced stages.
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Humanos , Carcinoma de Célula de Merkel , Transplante de Pele , Cirurgia Plástica , Carcinoma Neuroendócrino , Neoplasias de Cabeça e PescoçoRESUMO
Prognostic factors in Merkel cell carcinoma (MCC) are scarce. Tumour budding (TB) has been shown to have a prognostic role in different cancers but has not been explored in MCC. We aimed to determine if TB influences survival in MCC. We performed a retrospective evaluation of 45 cases of MCC in a cancer centre. This included a survival analysis involving TB in patients with MCC, and we searched for variables associated with TB. The mean age of the patients was 69 years. Histologically, the average Breslow was 11.36 mm, and the mean mitotic rate was 31.9 mitoses/mm2. The diagnosis was made in clinical stages I and II in 40% of cases, 22.2% in stage III, and 37.8% in stage IV. Tumour budding was low ( 10 buds/0.785 mm2) in 24.4%. There were no clinical or pathological features associated with high TB. Among the prognostic factors for 5-year survival, we found that tumour size and clinical stage were statistically associated with survival (p = 0.031 and 0.021), but TB was not. No clinical or pathological characteristics of MCC are associated with any degree of TB. Tumour budding does not influence overall survival.
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Carcinoma de Célula de Merkel , Neoplasias Cutâneas , Humanos , Idoso , Prognóstico , Estudos RetrospectivosRESUMO
Viruses have been frequently identified in several human neoplasms, but the etiological role of these viruses in some tumors is still a matter of controversy. Polyomaviruses stand out among the main viruses with oncogenic capacity, specifically the Merkel cell polyomavirus (MCPyV). Recent revisions in the taxonomy of polyomaviruses have divided the Polyomaviridae family into six genera, including 117 species, with a total of 14 currently known human-infecting species. Although the oncogenicity of polyomaviruses has been widely reported in the literature since 1950, the first description of a polyomavirus as an etiological agent of a neoplasm in humans was made only in 2008 with the description of MCPyV, present in approximately 80% of cases of Merkel cell carcinoma (MCC), with the integration of its genome to that of the tumor cells and tumor-specific mutations, and it is considered the etiological agent of this neoplasm since then. MCPyV has also been detected in keratinocyte carcinomas, such as basal cell carcinoma and squamous cell carcinoma of the skin in individuals with and without immunosuppression. Data on the occurrence of oncogenic viruses potentially involved in oncogenesis, which cause persistence and tissue injury, related to the Merkel cell polyomavirus are still scarce, and the hypothesis that the Merkel cell polyomavirus may play a relevant role in the genesis of other cutaneous carcinomas in addition to MCC remains debatable. Therefore, the present study proposes to explore the current knowledge about the presence of MCPyV in keratinocyte carcinomas.
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Merkel cell carcinoma (MCC) is a rare aggressive neuroendocrine cutaneous carcinoma with a high mortality rate. The MCC etiology is not fully understood. Merkel cell-associated polyomavirus (MCPyV) was found in MCC patients, indicating a risk factor for the tumor. Caucasian, elderly, and immunocompromised individuals are more likely to develop this tumor. HLA-G consists of a non-classical class I (Ib) HLA molecule with an immunoregulatory function and was associated with tumor escape in different types of tumors, nonetheless, never been studied in MCC. The purpose of this study was to evaluate the HLA-G expression and also to detect the MCPyV in MCC patients and correlate it with the clinical course of the disease. Forty-five MCC patients were included in a retrospective study. Formalin-fixed paraffin-embedded cutaneous skin biopsies were used by immunohistochemistry and RT-PCR to verify the HLA-G expression and MCPyV infection. HLA-G expression was found in 7 (15.6%), while the presence of MCPyV was detected in 28 (62.2%) of the studied patients. No significant association was found between HLA-G expression and MCPyV infection (p = 0.250). The presence of MCPyV was associated with areas of low sunlight exposure (p = 0.042) and the HLA-G expression with progression to death (p = 0.038). HLA-G expression was detected in MCC patients, as well as the MCPyV presence was confirmed. These markers could represent factors with a possible impact on patient survival; however, further studies with a greater number of patients are needed, to better elucidate the possible role in disease progression.
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Carcinoma de Célula de Merkel , Poliomavírus das Células de Merkel , Infecções por Polyomavirus , Neoplasias Cutâneas , Humanos , Idoso , Carcinoma de Célula de Merkel/genética , Carcinoma de Célula de Merkel/patologia , Poliomavírus das Células de Merkel/genética , Antígenos HLA-G , Neoplasias Cutâneas/genética , Estudos Retrospectivos , Infecções por Polyomavirus/complicações , Infecções por Polyomavirus/genéticaRESUMO
Abstract Viruses have been frequently identified in several human neoplasms, but the etiological role of these viruses in some tumors is still a matter of controversy. Polyomaviruses stand out among the main viruses with oncogenic capacity, specifically the Merkel cell polyomavirus (MCPyV). Recent revisions in the taxonomy of polyomaviruses have divided the Polyomaviridae family into six genera, including 117 species, with a total of 14 currently known human-infecting species. Although the oncogenicity of polyomaviruses has been widely reported in the literature since 1950, the first description of a polyomavirus as an etiological agent of a neoplasm in humans was made only in 2008 with the description of MCPyV, present in approximately 80% of cases of Merkel cell carcinoma (MCC), with the integration of its genome to that of the tumor cells and tumor-specific mutations, and it is considered the etiological agent of this neoplasm since then. MCPyV has also been detected in keratinocyte carcinomas, such as basal cell carcinoma and squamous cell carcinoma of the skin in individuals with and without immunosuppression. Data on the occurrence of oncogenic viruses potentially involved in oncogenesis, which cause persistence and tissue injury, related to the Merkel cell polyomavirus are still scarce, and the hypothesis that the Merkel cell polyomavirus may play a relevant role in the genesis of other cutaneous carcinomas in addition to MCC remains debatable. Therefore, the present study proposes to explore the current knowledge about the presence of MCPyV in keratinocyte carcinomas.
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INTRODUCTION: Merkel cell carcinoma (MCC) is an aggressive cutaneous cancer that frequently compromises the lymph nodes (LN) and distal organs. We sought to describe clinical and demographic characteristics of affected patients, analyze risk factors for LN compromise, metastasis, and death, and evaluate their impact on survival. MATERIALS AND METHODS: Patients with MCC were retrieved from the SEER database. Demographic, clinical and treatment data were analyzed. Logistic and Cox proportional hazard regression were used to analyze risk factors. Survival analysis was done with the Kaplan-Meier method. RESULTS: A total of 2010 patients were included, among which 288 (14.33%) had distant metastases at diagnosis. LN involvement occurred in 45.8% and 20.1% of patients with and without distant metastasis, respectively. Males were more likely to present LN compromise (OR = 1.33, p<0.001). Tumors >10 mm showed a significantly higher risk for LN involvement and distant metastasis, with those >20 mm showing the highest risk (OR = 2.76 p<0.001 and OR = 8.88 p<0.001 respectively). Location of the tumor in the trunk was a protective factor for overall death (OR = 0.27), while LN compromise was a risk factor (OR = 3.12). Only history of previous malignancy significantly affected disease-specific death (OR = 0.32, p = 0.01). One-year survival was 79.7% and 38.2% for patients with regional LN disease and distant metastasis, respectively. CONCLUSION: MCC is an aggressive cancer with high rates of LN involvement and distant metastases. Male gender and tumor size were risk factors for regional LN and metastatic disease. Tumor location in the trunk decrease the risk of overall death, while LN involvement increased it.
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Carcinoma de Célula de Merkel , Neoplasias Cutâneas , Humanos , Masculino , Carcinoma de Célula de Merkel/terapia , Carcinoma de Célula de Merkel/patologia , Modelos de Riscos Proporcionais , Linfonodos/patologia , Excisão de Linfonodo , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapiaRESUMO
Merkel-cell-carcinoma of the hand is rare. The Pathological and Immunohistochemical diagnosis helps us to focus the treatment. Immunotherapy has shown beneficial effects in unresectable/advanced/metastatic stages. The quantification of antibodies against Merkel-cell-polyomavirus (MCPyV) can be a useful for prognosis and follow-up. A wide margin in surgery and the sentinel node are the first option with Radiotherapy.
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Aims: The current study aims to present epidemiologic changes and clinical aspects of Merkel cell carcinoma (MCC) in Brazil. Methods: Data were collected from the Brazilian Population-Based Cancer Registries (2000-2015) and Hospital-Based Cancer Registries (2000-2017). Results: The average age-standardized incidence rates significantly increased in men between the years 2000 (0.31/1,000,000) and 2015 (1.21/1,000,000), with an annual percentage change of 9.4 (95% CI: 4.7-14.4; p < 0.001). In women, the incidence rates rose insignificantly in the period with an annual percentage change of 3.1 (95% CI: 0.0-6.2; p < 0.10). From the hospital-based database, 881 MCC patients were identified. Most of the patients were females (51.2%), aged >60 years (82.2%), White (67.6%) and diagnosed at stages III or IV (50.5%). Conclusions: A key aspect of public health promotion is to understand the incidence and morbidity of MCC.
Lay abstract Aims: The current study aims to present epidemiologic changes and clinical aspects of Merkel cell carcinoma (MCC) in Brazil. Methods: Data were collected from the Brazilian Population-Based Cancer Registries (20002015) and Hospital-Based Cancer Registries (20002017). Results: The incidence rates significantly increased in men between the years 2000 and 2015. In women, the incidence rates rose insignificantly in the same period. From the hospital-based database, MCC patients were identified. Most of the patients were females, aged >60 years, White and diagnosed with locally advanced and advanced stages. Conclusions: A key aspect of public health promotion is to understand the incidence and morbidity of MCC.
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Carcinoma de Célula de Merkel/epidemiologia , Neoplasias Cutâneas/epidemiologia , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Brasil/epidemiologia , Carcinoma de Célula de Merkel/diagnóstico , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Sistema de Registros/estatística & dados numéricos , Fatores de Risco , Fatores Sexuais , Neoplasias Cutâneas/diagnóstico , Adulto JovemRESUMO
BACKGROUND: Merkel cell carcinoma (MCC) is an aggressive malignancy, associated with poor outcomes in patients with metastatic disease (mMCC). Management has been dramatically altered as a result of incorporating immune checkpoint blockade agents. MCC data from Latin America (LATAM) come from case-series or individual records. Regional registries are lacking. A need for better registries to improve current knowledge about MCC is highlighted. Our objectives were to describe a real-world experience with avelumab as a second-line (or first-line in unfit patients) treatment in a subset of LATAM participants enrolled in a global Expanded Access Program (EAP) for patients with mMCC, and to evaluate its contribution to the resolution of the concerns described in a recent regional experts review. MATERIALS AND METHODS: We reviewed data of LATAM participants in an avelumab EAP for mMCC treatment (NCT03089658). EAP patient had unresectable or mMCC with progressive disease after one line of chemotherapy, and were ineligible for clinical trials or unfit for chemotherapy. RESULTS: 46 patients (median age: 71.6 years; 60.9% males; median treatment duration: 7.9 months) were included in the LATAM EAP. Physician-assessed objective responses were available for 19 patients. Complete response rate was 15.8% and partial response rate reached 42.1%, summarizing an objective response rate of 57.9%. Stable disease rate was 10.5%, with a disease control response of 68.4%. CONCLUSION: Avelumab showed robust efficacy and a safety profile consistent with global EAP data. Results are aimed to improve current knowledge about mMCC treatment and access to immunooncologic strategies for treating LATAM patients.
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Anticorpos Monoclonais Humanizados/uso terapêutico , Antineoplásicos Imunológicos/uso terapêutico , Carcinoma de Célula de Merkel/tratamento farmacológico , Guias de Prática Clínica como Assunto/normas , Neoplasias Cutâneas/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma de Célula de Merkel/patologia , Feminino , Seguimentos , Humanos , América Latina , Masculino , Pessoa de Meia-Idade , Prognóstico , Neoplasias Cutâneas/patologiaRESUMO
Abstract Introduction Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine malignant cancer. It is an epidermal cancer common in the head and neck. Objectives Though there is limited number of cases described in the literature for the treatment difficult to obtain. Our purpose was to present the clinical course and treatment of four patients with MCC. Methods We conducted a retrospective analysis and obtained detailed clinical information for all 4 patients treated for MCC at the ENT Department of the SS Annunziata Hospital in Chieti, Italy, from 2013 through 2015. Results In our study, two patients presented with the tumor in a rare site (lower eyelid). All of the patients underwent surgical treatment: three patients had free excision margins and negative sentinel lymph nodes (SLNs) while 1 patient had free excision margins and positive SLNs. The latter patient underwent ipsilateral neck dissection. In another patient, the fluorodeoxyglucose positron emission topography (FDG PET)/computed tomography (CT) performed 6 months after the surgery has shown high metabolic activity in the left parotid gland, and the patient underwent total parotidectomy and a neck dissection. Conclusion Sentinel lymph node biopsy is a useful technique in small size MCCs of the head and neck. However, the parotid gland should be strictly controlled in patients with lower eyelid tumors.
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Introduction Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine malignant cancer. It is an epidermal cancer common in the head and neck. Objectives Though there is limited number of cases described in the literature for the treatment difficult to obtain. Our purpose was to present the clinical course and treatment of four patients with MCC. Methods We conducted a retrospective analysis and obtained detailed clinical information for all 4 patients treated for MCC at the ENT Department of the SS Annunziata Hospital in Chieti, Italy, from 2013 through 2015. Results In our study, two patients presented with the tumor in a rare site (lower eyelid). All of the patients underwent surgical treatment: three patients had free excision margins and negative sentinel lymph nodes (SLNs) while 1 patient had free excision margins and positive SLNs. The latter patient underwent ipsilateral neck dissection. In another patient, the fluorodeoxyglucose positron emission topography (FDG PET)/computed tomography (CT) performed 6 months after the surgery has shown high metabolic activity in the left parotid gland, and the patient underwent total parotidectomy and a neck dissection. Conclusion Sentinel lymph node biopsy is a useful technique in small size MCCs of the head and neck. However, the parotid gland should be strictly controlled in patients with lower eyelid tumors.
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Background: Little is known about the 2019 novel coronavirus disease (COVID-19) course and outcomes in patients receiving immunotherapy. Here we describe a metastatic Merkel cell carcinoma patient with a severe acute respiratory syndrome coronavirus 2 infection while receiving pembrolizumab. Case presentation: A 66-year-old man, with a metastatic Merkel cell carcinoma receiving pembrolizumab, presented with fever. Chest computed tomography (CT) showed pulmonary ground-glass opacities, suggesting viral or immuno-related etiology. On day 7, the patient was hospitalized due to dyspnea and worsening of the radiological findings. Real time polymerase chain reaction (RT-PCR) testing confirmed COVID-19. The patient developed acute respiratory distress syndrome and acute kidney injury. Hydroxychloroquine was administered for 5 days, but discontinued after supraventricular extrasystoles. Clinical improvement allowed the patient's discharge after 81 days of hospitalization. Conclusion: A careful evaluation of oncologic patients receiving immunotherapy during the COVID-19 pandemic is of utmost importance.
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Carcinoma de Célula de Merkel/terapia , Infecções por Coronavirus/diagnóstico , Imunoterapia , Pneumonia Viral/diagnóstico , Neoplasias Cutâneas/terapia , Idoso , Anticorpos Monoclonais Humanizados/uso terapêutico , Antineoplásicos Imunológicos/uso terapêutico , Betacoronavirus , COVID-19 , Carcinoma de Célula de Merkel/complicações , Carcinoma de Célula de Merkel/patologia , Infecções por Coronavirus/complicações , Infecções por Coronavirus/patologia , Infecções por Coronavirus/terapia , Humanos , Imunoterapia/efeitos adversos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Masculino , Pandemias , Pneumonia Viral/complicações , Pneumonia Viral/patologia , Pneumonia Viral/terapia , Receptor de Morte Celular Programada 1/antagonistas & inibidores , SARS-CoV-2 , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/patologia , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
OBJECTIVE: To summarize the features of the Merkel cell carcinoma (MCC) and to sistematyze its diagnosis and therapeutic management. METHOD: We performed a literature review in PubMed, obtaining a total of 3,308 articles, selecting 10 for its complete reading and 22 for the reading of the summary according to the content. RESULTS: In none of our patients, the MCC was the first suspected diagnosis. The treatment consisted in surgical excision with tumor free margins and lymphadenectomy. We offered ad-juvant RT which they rejected. They remain disease-free at the present time. CONCLUSIONS: MCC is a rare and aggressive disease which presents as a fast-growing solitary asymptomatic erythematous nodule in those areas of skin which are exposed to sunlight in elderly patients. The main risk factors include radiative ultraviolet, immunosuppression and merkel cell polyomavirus. Surgery is the main loco-regional treatment. Lymph node metastases in the course of the disease is one of the main prognostic factors. If there are no adenopaties, sentinel lymph node biopsy must be done; if there are adenopaties or a positive biopsy, lymphadenectomy is indicated. Radiotherapy is indicated in all stages of disease since it has shown to improve loco-regional control. In distant metastatic disease, immunotherapy and participating in clinical trials are the first choice.
OBJETIVO: Resumir las características del carcinoma de células de Merkel (CCM) y sistematizar su manejo diagnóstico-terapéutico. MÉTODO: Realizamos una búsqueda bibliográfica en PubMed y aparecieron 3,308 artículos, de los que seleccionamos 10 para lectura completa y 22 para lectura del resumen acorde con su contenido. RESULTADOS: En ninguno de nuestros pacientes el CCM fue la primera sospecha diagnóstica. El tratamiento consistió en la extirpación quirúrgica con márgenes libres y linfadenectomía. Se les ofreció radioterapia adyuvante, que rechazaron. Se encuentran libres de enfermedad tras 1 año del tratamiento. CONCLUSIONES: El CCM es una condición rara y agresiva que se presenta como un nódulo eritematoso de rápido crecimiento y asintomático en zonas fotoexpuestas de pacientes añosos. Los principales factores de riesgo son la exposición ultravioleta, la inmunosupresión y el poliomavirus asociado al carcinoma de Merkel (MCPyV, Merkel cell polyomavirus). La cirugía es el pilar fundamental del tratamiento locorregional. La afectación ganglionar en el transcurso de la enfermedad es uno de los principales factores pronósticos. Si no existen adenopatías reconocibles, debe realizarse biopsia selectiva de ganglio centinela; si existen adenopatías o la biopsia es positiva, está indicada la linfadenectomía. La radioterapia adyuvante está indicada en todos los estadios y ha demostrado un mejor control locorregional. En la enfermedad a distancia es de primera elección la inmunoterapia y participar en ensayos clínicos.
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Carcinoma de Célula de Merkel , Neoplasias Faciais , Hallux , Neoplasias Cutâneas , Idoso , Idoso de 80 Anos ou mais , Carcinoma de Célula de Merkel/diagnóstico , Carcinoma de Célula de Merkel/mortalidade , Carcinoma de Célula de Merkel/patologia , Carcinoma de Célula de Merkel/cirurgia , Neoplasias Faciais/diagnóstico , Neoplasias Faciais/mortalidade , Neoplasias Faciais/patologia , Neoplasias Faciais/cirurgia , Feminino , Humanos , Excisão de Linfonodo , Metástase Linfática , Masculino , Margens de Excisão , Radioterapia Adjuvante , Fatores de Risco , Biópsia de Linfonodo Sentinela , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/mortalidade , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/cirurgiaRESUMO
PURPOSE: Immunosuppressed (IS) patients are at increased risk for developing Merkel cell carcinoma (MCC) with worsened outcomes compared to immunocompetent (IC) patients. We sought to determine the effects of immune status on the efficacy of adjuvant RT regarding OS for patients with stage I, II or III (localized) MCC of the head and neck. METHODS/PATIENTS: The National Cancer Database was queried for patients with resected, localized MCC of the head and neck with known immune status. Kaplan-Meier methods were used to describe OS. Log-rank tests, multivariable Cox regression models and interaction effect testing were used to compare OS by subgroup categorized by patient and treatment factors including immune status and adjuvant RT receipt. RESULTS: A total of 892 (89.6%) IC and 104 (10.4%) IS patients with MCC of the head and neck were included. Adjuvant RT was associated with improved 3-year OS rate for both IS patients (49.4% vs. 35.5%, p = 0.0467) and stage I/II IC patients (72.4% vs. 62.9%, p = 0.0092). Adjuvant RT was associated with decreased hazard of death (HR 0.77, 95% CI 0.62-0.95). Interaction effect testing did not demonstrate a difference in the efficacy of adjuvant RT on OS between IC and IS status (p = 0.157). CONCLUSIONS: In this NCDB analysis, adjuvant RT was associated with decreased hazard of death for patients with localized MCC of the head and neck regardless of immune status and should be considered for both IS and IC patients.
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Carcinoma de Célula de Merkel/radioterapia , Neoplasias de Cabeça e Pescoço/radioterapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma de Célula de Merkel/imunologia , Carcinoma de Célula de Merkel/mortalidade , Feminino , Neoplasias de Cabeça e Pescoço/imunologia , Neoplasias de Cabeça e Pescoço/mortalidade , Humanos , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Radioterapia Adjuvante , Estudos RetrospectivosRESUMO
Merkel cell carcinoma (MCC) is a rare skin tumour of neuroendocrine origin with aggressive behaviour. The aims of this study were to investigate the association of p63 + MCC with clinicopathological features and to estimate survival through a systematic review and meta-analysis. A comprehensive search of PubMed, Embase, Scopus and Virtual Health Library following the PRISMA guidelines was conducted on September 2017. DerSimonian and Lard random-effects models were used to calculate survival-weighted means and their corresponding 95% confidence intervals (CI) among studies. Five studies met our inclusion criteria after screening 77 citations and 36 full-text articles. The included studies enrolled 413 patients with MCC. We observed that p63 + MCC was significantly associated with mortality with OR 2.92 (95% CI [1.66-5.13]). The summary hazard ratio of multivariate analysis was 1.99 (95% CI [1.32-3.01]). The only clinicopathological feature associated with p63 + MCC with statistical significance was the Merkel cell polyomavirus (MCPyV) status. The presence of MCPyV was associated as a protective factor for the expression of p63 (OR 0.25, 95% CI [0.08-0.73]). These results support that p63 + MCC evaluated by immunohistochemistry has a poor outcome. Therefore, we suggest p63 to be performed when staging MCC.
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Carcinoma de Célula de Merkel/metabolismo , Carcinoma de Célula de Merkel/patologia , Proteínas de Membrana/metabolismo , Neoplasias Cutâneas/metabolismo , Neoplasias Cutâneas/patologia , Humanos , Estadiamento de Neoplasias , Taxa de SobrevidaRESUMO
BACKGROUND: Merkel cell carcinoma (MCC) is a rare neuroendocrine skin tumor. In our knowledge, only 30 cases of brain metastasis were reported in literature. The authors report a case of 57-year-old male with elevated intracranial pressure signs, which a frontal mass with pathological diagnosis of MCC. CASE DESCRIPTION: A 57-year-old male was admitted with a 3-month history of progressive headache, associated with nausea and dizziness. The magnetic resonance imaging showed a left frontal lobe, parasagittal, and nodular lesion with perilesional edema. The patient underwent complete surgical resection with success. The adjuvant treatment was radiotherapy and chemotherapy. CONCLUSION: In our knowledge, there is a little number of cases of MCC reported in literature. Surgical management is considered in cases with intracranial hypertension or focal signs. The adjuvant treatment options are immunotherapy and radiotherapy.
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Introducción: El carcinoma de células de Merkel (MCC) es un tumor cutáneo maligno agresivo y de mal pronóstico. La incidencia es mayor en adultos hombres, caucásicos, con edad promedio de 70 años. Feng et al, lograron aislar un nuevo virus en muestras de este tumor, que denominaron virus polioma de células de Merkel (MCPyV). Se ha intentado establecer una relación causal entre el virus y MCC. El virus está integrado al genoma y produciría mutaciones específicas. En muestras de MCC, se ha detectado expresión de oncoproteinas virales (antígenos T) que promueven la replicación viral y tumorogénesis
Introduction: Merkel cell carcinoma (MCC) is an aggressive malignant cutaneous tumor with poor prognosis. Most cases affect elder patient with an average of 70 years of age. Feng et al isolated a new virus, the Merkel cell carcinoma polyoma virus (MCPyV). A causal relationship between MCPyV y MCC has been established. The virus is integrated in the genome and pro-duces specific mutations. MCC samples show ex-pression of viral oncoproteins (T antigens) that promote viral replication and tumorogenesis.