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BACKGROUND: Pelvic exenteration (PE) is an extensive surgical treatment reserved for advanced or recurrent pelvic neoplasms, with potential impacts on patients' quality of life (QoL) poorly referenced in the literature. OBJECTIVES: This study aimed to evaluate QoL outcomes among three types of PE. METHODS: A cross-sectional study assessed 106 patients divided into anterior PE (APE), posterior PE (PPE), or total PE (TPE) groups. QoL was measured using e short form 36 version 2 (SF-36) and the European Organization for Research and Treatment of Cancer QoL Quality of Life Questionnaire Core 30 (QLQ-C30) QoL questionnaires. Descriptive and inferential analyses compared questionnaire scores. RESULTS: The findings unveiled a balance among the three groups concerning demographic variables and comorbidities, with the exception of a male predominance in the APE and TPE cohorts. Notably, the APE group exhibited elevated scores in overall health (assessed via SF-36) and social functioning and diarrhea domains (assessed via QLQ-C30). Moreover, in terms of the fatigue and nausea/vomiting domains (assessed via QLQ-C30), the APE group demonstrated superior QoL compared to the PPE group. Conversely, the PPE group manifested a notably lower QoL in the constipation domain (assessed via QLQ-C30) compared to the other two groups. Additionally, disease recurrence was significantly associated with diminished QoL across multiple domains. CONCLUSION: APE patients exhibited better QoL than PPE and TPE groups, with disease recurrence adversely affecting QoL.
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Ewing sarcoma in the head and neck is rare, and metastasis from other bones to the mandible accounts for 0.7% of cases. This report presents a case of oral metastasis in a 24-year-old male patient diagnosed with Ewing sarcoma of the femur (p53 gene mutation and EWSR1-ERG fusion). The chief complaint was numbness in the mandible and pain for 1 month and a hardened, ulcerated exophytic lesion in the right retromolar region. Imaging exams revealed an unspecified thinning of the cortical bone of the inferior alveolar canal in the right mandibular ramus, associated with erosion of the alveolar bone. Histopathological analysis confirmed metastasis of Ewing sarcoma. The patient presented an aggressive disease progression and died 1 month after the oral diagnosis. It is important to recognize the signs and symptoms compatible with rare clinical outcomes, leading to an early diagnosis that can improve patients' quality of life and survival.
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Desmoplastic small round cell tumor (DSRCT) is an extremely rare, aggressive sarcoma affecting adolescents and young adults with male predominance. Generally, it originates from the serosal surface of the abdominal cavity. The hallmark characteristic of DSRCT is the EWSR1-WT1 gene fusion. This translocation up-regulates the expression of PDGFRα, VEGF and other proteins related to tumor and vascular cell proliferation. Current management of DSRCT includes a combination of chemotherapy, radiation and aggressive cytoreductive surgery plus intra-peritoneal hyperthermic chemotherapy (HIPEC). Despite advances in multimodal therapy, outcomes remain poor since the majority of patients present disease recurrence and die within three years. The dismal survival makes DSRCT an orphan disease with an urgent need for new drugs. The treatment of advanced and recurrent disease with tyrosine kinase inhibitors, such as pazopanib, sunitinib, and mTOR inhibitors was evaluated by small trials. Recent studies using comprehensive molecular profiling of DSRCT identified potential therapeutic targets. In this review, we aim to describe the current studies conducted to better understand DSRCT biology and to explore the new therapeutic strategies under investigation in preclinical models and in early phase clinical trials.
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Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive mesenchymal malignancy, usually affecting young males. There is no consensus on the best therapeutic approach. We seek to characterize a cohort of nonpediatric patients with DSRCT treated at a large Brazilian cancer center. We performed a retrospective analysis of patients with histologically confirmed DSRCT referred to our institution (2007-2020). Clinical and imaging data were extracted and summarized with descriptive statistics. Survival analyses were conducted by the Kaplan-Meier method and compared with the log-rank test. We included 19 patients with DSRCT, the median age at diagnosis was 26 years (range: 15-41 years), and 68% were male. Ninety percent presented with abdominopelvic masses, and 32% had extra-abdominal metastasis at diagnosis. Eleven patients (58%) underwent surgery, four patients (21%) received whole abdominal adjuvant radiotherapy, and five patients (26%) had hyperthermic intraperitoneal chemotherapy. Median OS was 27 months (interquartile range: 18-51 m). The five-year OS rate was 12%. Our data confirm the aggressiveness of DSRCT despite intense multimodality treatment. Outcomes of patients treated in a reference cancer center in a developing country are similar to cancer centers in developed nations. Multicenter cooperation is urgent to the development of clinical trials and to improve diagnosis and treatment efficacy.
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BACKGROUND: Human papillomavirus (HPV)-positive oropharyngeal squamous cell carcinoma (OPSCC) is associated with better tumor-response rates and survival outcomes. However, in some geographic regions, the impact of HPV infection on prognosis remains unclear. The aim of this study was to describe the patterns of recurrence and survival among patients treated for OPSCC in a geographic region with a reported low prevalence of HPV-related OPSCC. METHODS: We retrospectively evaluated 215 patients diagnosed with American Joint Committee on Cancer (AJCC) stages I to IV OPSCC who were treated with upfront surgery or radiation therapy with or without chemotherapy in a tertiary Cancer Center in Brazil. The collected data included demographic information, HPV status, tobacco and alcohol consumption, and pathologic and treatment variables. The patterns of recurrence were recorded according to HPV status. Disease-specific survival and recurrence-free survival were calculated. RESULTS: One hundred twenty-seven (59.1%) patients were diagnosed with HPV-positive OPSCC. According to the AJCC eighth edition, 34 (15.8%), 71 (33%), 47 (21.9%), and 60 (27.9%) patients had stage I, II, III, and IV disease, respectively. Surgery was performed in 109 (50.7%) cases, and upfront chemoradiation regimens were provided in 104 (48.4%, P = .69) patients. Overall, the 5-year cancer-specific survival was 73.5% and 68.1% for patients positive and negative to HPV, respectively. Tobacco status was considered the only independent prognostic factor for survival. Furthermore, HPV status was not associated with differences in recurrence rates (P = .68). While all distant relapses were found to be lung metastases in the HPV-negative group, we observed unusual sites of distant metastases in the HPV-positive group. CONCLUSIONS: HPV status was not associated with higher rates of survival among the investigated population. Moreover, smoking status was considered the only independent prognostic factor for survival. Furthermore, patients with HPV-positive tumors were more likely than patients with HPV-negative OPSCC to have unusual distant metastases.
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Alphapapillomavirus , Neoplasias de Cabeça e Pescoço , Neoplasias Orofaríngeas , Infecções por Papillomavirus , Brasil/epidemiologia , Neoplasias de Cabeça e Pescoço/terapia , Humanos , Recidiva Local de Neoplasia , Neoplasias Orofaríngeas/terapia , Papillomaviridae , Infecções por Papillomavirus/epidemiologia , Infecções por Papillomavirus/terapia , Prevalência , Prognóstico , Estudos RetrospectivosRESUMO
Introdução: A avaliação da resposta à quimioterapia de indução (QI) com regimes triplos, incluindo taxane, cisplatina e 5 fluorouracil (TPF) em carcinoma de células escamosas de cabeça e pescoço localmente avançado (CECCPLA) é geralmente realizada após 2 ciclos de quimioterapia usando critérios morfológicos. Preocupações em relação ao perfil de toxicidade do TPF sugerem um benefício potencial de uma abordagem de avaliação de resposta precoce. Objetivo: o objetivo deste estudo é avaliar a utilidade de se avaliar precocemente a resposta tumoral por método funcional e morfológico com uso do PET-SCAN em pacientes portadores de CECCPLA tratados com QI seguido de radioteapia após o primeiro ciclo de QI. Métodos: Pacientes com CECCPLA que se submeteram ao QI com TPF foram avaliados prospectivamente. Os procedimentos de estadiamento incluíram imagem locorregional e de tórax, exame endoscópico e PET-SCAN. Pacientes foram avaliados para resposta tumoral após o segundo ciclo da QI e ao término do tratamento, conforme conduta estabelecida para a prática clínica. No dia 14 do primeiro ciclo, um segundo PET- SCAN foi realizado e os médicos e pacientes foram cegados para os seus resultados. Todos os pacientes assinaram consentimento para participação do estudo. Resultados: Entre fevereiro de 2010 e julho de 2013, 49 pacientes portadores de CECCPLA estádio III / IVA-B CECCPLA foram recrutados. Após um seguimento mediano de 44,3 meses, pacientes cujos achados de PET-SCAN não registraram aumento no Stardard Uptake Value (SUV) máximo dos linfonodos regionais apresentaram melhor sobrevida livre de recidiva (HR = 0,18; IC95% 0,056-0,585; p = 0,004) e sobrevida global (HR = 0,14, IC 95% 0,040-0,498; p = 0,002) e foram considerados respondedores. Neste subgrupo, os pacientes que atingiram pelo menos 45% de redução no SUV máximo do tumor primário apresentaram melhor sobrevida livre de progressão tumoral (HR = 0,23, IC 95% 0,062-0,854; p = 0,028) e sobrevida global (HR = 0,11, IC 95% 0,013 -0,96; p = 0,046). Conclusão: Estes resultados sugerem um potencial papel da avaliação da resposta tumoral precoce com PET-SCAN em pacientes com CECCPLA submetidos a QI. Aumento no SUV máximo do linfonodo regional e diminuição insuficiente na captação do tumor primário predizem pior evolução clínica (AU)
Introduction: Evaluation of induction chemotherapy (IC) response with triplet taxane, cisplatin and 5 fluorouracil containing regimen (TPF) in locally advanced head and neck squamous cell carcinoma (LASCCHN) is usually performed after 2 cycles of chemotherapy using morphological criteria. Concerns regarding the TPF toxicity profile suggest a potential benefit of an early tumor response assessment approach. Objective: The objective of this study is to evaluate the usefulness of early evaluation of tumor response by functional and morphological method using PET-SCAN patients with LASCCHN treated with IC followed by radiotherapy after the first IC cycle. Methods: Patients with LASCCHN who underwent IC with TPF were prospectively evaluated. Staging procedures included standard primary neck tumor and chest imaging, endoscopic examination and PET-SCAN. Patients were evaluated for tumor response after the second cycle of IC and at the end of treatment, according to established practice guidelines. On day 14 of the first cycle, a second PET-SCAN was performed and physicians and patients were blinded to their exam findings. Results: Between February 2010 and July 2013, 49 patients staged AJCC III / IVA-B LASCCHN were recruited. After a median follow-up of 44.3 months, patients with no increase in the regional maximum lymph node SUV had better relapse-free survival (HR = 0.18, 95% CI 0.056-0.585, p = 0.004) and overall survival (HR = 0.14, 95% CI 0.040-0.498, p = 0.002) and were considered responders. Among cases considered responders, patients who achieved at least 45% reduction of SUV in the primary tumor presented improvement in progression-free (HR = 0.23, 95% CI 0.062-0.854, p = 0.028) and overall survival (HR = 0.11, 95% CI 0.013 -0.96, p = 0.046). All patients provided informed consent for study participation. Conclusion: These results suggest an important role of the evaluation of the early response with PET-SCAN in patients with LASCCHN undergoing IC. Increase in the regional SUV maximum and insufficient decrease in primary tumor uptake predict worse clinical outcome (AU)
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Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Papillomaviridae , Radioterapia , Carcinoma de Células Escamosas , Quimioterapia de Indução , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Neoplasias de Cabeça e PescoçoRESUMO
There is no established biomarker for cetuximab efficacy in recurrent head and neck squamous cell carcinoma (HNSCC). The aim of the present study was to evaluate the prognostic and predictive impact of PTEN, cMET, and p16 expression in recurrent HNSCC. In this retrospective study, 112 patients with recurrent HNSCC received chemotherapy (CT) alone (n = 37) or chemotherapy with cetuximab (n = 75). PTEN, cMET, and p16 protein expression were evaluated by immunohistochemistry. The median overall survival (mOS) for the patients treated with cetuximab + CT versus CT alone was 11.4 months and 7.0 months, (p = 0.949). The median progression-free survival (mPFS) was 6.2 months versus 3.0 months (p = 0.154). Patients with PTEN loss exhibited a mOS of 5.8 months versus 10.5 months (p = 0.002) and a mPFS of 3.2 months versus 4.7 months (p = 0.019). A multivariate analysis identified an independent association between PTEN loss and OS (HR 2.27; 95% confidence 95% CI 1.27-4.08; p = 0.006) and with PFS (HR 1.85; 95% CI 1.09-2.99; p = 0.022). A negative prognostic impact of PTEN loss was observed in the patients treated with cetuximab + CT, and not in the CT only group. Expression of cMET and p16 showed no impact on OS or PFS. The present findings confirm that PTEN is a prognostic factor for metastatic HNSCC and they support further studies of PTEN expression to evaluate its predictive value to cetuximab response.
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Antineoplásicos Imunológicos/uso terapêutico , Cetuximab/uso terapêutico , Resistencia a Medicamentos Antineoplásicos/fisiologia , PTEN Fosfo-Hidrolase/metabolismo , Carcinoma de Células Escamosas de Cabeça e Pescoço/tratamento farmacológico , Adulto , Idoso , Biomarcadores Tumorais/análise , Inibidor p16 de Quinase Dependente de Ciclina , Inibidor de Quinase Dependente de Ciclina p18/metabolismo , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Prognóstico , Intervalo Livre de Progressão , Modelos de Riscos Proporcionais , Proteínas Proto-Oncogênicas c-met/metabolismo , Estudos Retrospectivos , Carcinoma de Células Escamosas de Cabeça e Pescoço/mortalidadeRESUMO
OBJECTIVE: The objective of this study was to assess the clinical value of 18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) after the first cycle of induction chemotherapy (IC) in locally advanced squamous cell carcinoma of the head and neck (LASCCHN). METHODS AND FINDINGS: A prospective, single-arm, single center study was performed, with patients enrolled between February 2010 and July 2013.Patients (n = 49) with stage III/IVA-B LASCCHN who underwent IC with taxanes, cisplatin, and fluorouracil were recruited. Staging procedures included loco-regional and chest imaging, endoscopic examination, and PET/CT scan. On day 14 of the first cycle, a second PET/CT scan was performed. Patients with no early increase in regional lymph node maximum 18F-FDG standard uptake value (SUV), detected using 18F-FDG PET/CT after first IC had better progression-free survival (hazard ratio (HR) = 0.18, 95%, confidence interval (CI) 0.056-0.585; p = 0.004) and overall survival (HR = 0.14, 95% CI 0.040-0.498; p = 0.002), and were considered responders. In this subgroup, patients who achieved a reduction of ≥ 45% maximum primary tumor SUV experienced improved progression-free (HR = 0.23, 95% CI 0.062-0.854; p = 0.028) and overall (HR = 0.11, 95% CI 0.013-0.96; p = 0.046) survival. CONCLUSIONS: These results suggest a potential role for early response evaluation with PET/CT examination in patients with LASCCHN undergoing IC. Increased regional lymph node maximum SUV and insufficient decrease in primary tumor uptake predict poorer outcomes.
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Neoplasias de Cabeça e Pescoço/diagnóstico por imagem , Neoplasias de Cabeça e Pescoço/tratamento farmacológico , Carcinoma de Células Escamosas de Cabeça e Pescoço/diagnóstico por imagem , Carcinoma de Células Escamosas de Cabeça e Pescoço/tratamento farmacológico , Adulto , Idoso , Feminino , Fluordesoxiglucose F18 , Neoplasias de Cabeça e Pescoço/metabolismo , Humanos , Quimioterapia de Indução , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Papillomaviridae/isolamento & purificação , Papillomaviridae/patogenicidade , Projetos Piloto , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Intervalo Livre de Progressão , Estudos Prospectivos , Compostos Radiofarmacêuticos , Carcinoma de Células Escamosas de Cabeça e Pescoço/metabolismo , Resultado do TratamentoRESUMO
INTRODUCTION: Soft tissue Sarcomas (STS) are rare malignances, with high mortality rates. Half of patients develop metastasis. The presence of isolated Circulating Tumor Cells (CTCs) and Circulating Tumor Microemboli (CTM) in the blood may be early markers of tumor invasion. Epidermal Growth Factor (EGF) family receptors can also influence this process. OBJECTIVES: to quantify CTCs and identify CTM as well as the EGF Receptor (EGFR) protein expression in these cells and correlate with clinical outcome in metastatic STS. MATERIALS AND METHODS: Approximately 8mL of blood was prospectively collected from patients with different types of high-grade STS, before the beginning of chemotherapy. The samples were processed and filtered by ISET (Rarecells, France) for the isolation and quantification of CTCs and CTMs. EGFR expression was analyzed by immunocytochemistry (ICC) on CTCs/ CTMs. RESULTS: We analyzed 18 patients with median age of 49 years (18-77 y). The positivity for EGFR protein expression in CTCs was observed in 93.75% of the patients. This result shows that targeting EGFR positive CTCs from STS origen can be translated in clinical benefit for some patients. In addition, if target therapy is chosen, the EGFR expression in CTCs can be used in follow-up to measure treatment effectiveness. CONCLUSIONS: This is the first study to demonstrate the expression of EGFR protein in CTCs from sarcoma patients. It may open an area for future investigations. The next step is to characterize CTCs in a larger cohort of patients to better understand the role of EGFR in sustaining tumor metastasis in sarcomas.
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Células Neoplásicas Circulantes/metabolismo , Sarcoma/enzimologia , Adolescente , Adulto , Idoso , Receptores ErbB/biossíntese , Receptores ErbB/genética , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Metástase Neoplásica , Sarcoma/genética , Sarcoma/patologia , Adulto JovemRESUMO
BACKGROUND: Circulating tumor microemboli (CTM) are clusters of circulating tumor cells (CTCs), involved in metastasis, as also transforming growth factor-ß (TGF-ß). The purpose of this study was to verify their role in progression-free survival (PFS). METHODS: Blood from patients with locally advanced head and neck squamous cell carcinoma (HNSCC; n = 53) was analyzed in 2 moments. TGF-ß receptor I (TGF-ßRI) expression was evaluated by immunocytochemistry. RESULTS: Comparing CTM1 (baseline) with CTM2 (first follow-up), patients with CTM1-positive disease who became CTM2-negative were classified as favorable (PFS 20 months). Patients with unfavorable evolution (CTM1-negative/CTM2-positive), had PFS of 17.5 months. Patients always CTM-negative showed PFS of 22.4 months, those always positive, 4.7 months (P < .001). The TGF-ßRI expression in the first follow-up correlated with poor PFS (12 × 26 months; P = .007), being an independent prognostic factor (hazard ratio [HR] = 6.088; P = .033). CONCLUSION: CTM1/2, TGF-ßRI expression, and unfavorable CTM kinetics may represent poor prognosis in locally advanced HNSCC.
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Carcinoma de Células Escamosas/metabolismo , Carcinoma de Células Escamosas/patologia , Neoplasias de Cabeça e Pescoço/metabolismo , Neoplasias de Cabeça e Pescoço/patologia , Células Neoplásicas Circulantes/metabolismo , Proteínas Serina-Treonina Quinases/metabolismo , Receptores de Fatores de Crescimento Transformadores beta/metabolismo , Adulto , Idoso , Brasil , Carcinoma de Células Escamosas/mortalidade , Estudos de Coortes , Intervalo Livre de Doença , Feminino , Neoplasias de Cabeça e Pescoço/mortalidade , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Modelos de Riscos Proporcionais , Receptor do Fator de Crescimento Transformador beta Tipo I , Carcinoma de Células Escamosas de Cabeça e PescoçoRESUMO
BACKGROUND: Identify in advance responder patients to chemotherapy in metastatic colorectal cancer (CRC) would allow prompt interruption of ineffective therapies in non-responder patients. Hence, predictive markers are sought in numerous trials to detect responder patients, including tumor shrinkage measured by imaging methods. Usually, Response Evaluation Criteria in Solid Tumors (RECIST) is used to evaluate tumor response in metastatic CRC, but these criteria are questionable with use of biological agents associated to chemotherapy. Our aim was correlate early metabolic response by (18)F-2-fluoro-2-deoxy-D-glucose positron emission tomography-computed tomography ((18)FDG-PET-CT) with long-term outcome in metastatic CRC in first-line therapy. METHODS: We prospectively evaluated 36 patients with metastatic CRC in first-line treatment with 5-fluorouracil, leucovorin (folinic acid), oxaliplatin (FOLFOX) or 5-fluorouracil, leucovorin (folinic acid), irinotecan (FOLFIRI) associated with cetuximab or bevacizumab. (18)FDG-PET-CT was performed at baseline and after two cycles of chemotherapy. The early metabolic response [standardized uptake value (SUV)] was measured to identify responder and non-responder patients and correlated with overall survival (OS) and progression-free survival (PFS). RESULTS: Median age was 58.5 years (range, 41-74 years). PFS was 15.5 months for responder and 13.3 months for non-responder (P=0.42), OS was 55.7 months for responder and not reached for non-responder. There was no correlation between delta-SUV and clinical and pathological variables analyzed. In the subgroup of patients who did not undergo resection of metastasis (45%), PFS was higher for responders (15.3×6.8 months, P=0.02). CONCLUSIONS: According to our findings, early response by (18)FDG-PET-CT was not a predictor of long-term outcome for patients with metastatic CRC treated in the first-line chemotherapy with a monoclonal antibody.
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OBJECTIVE: To evaluate changes in apparent diffusion coefficients (ADC) as measured by magnetic resonance imaging (MRI) before and after the treatment of primary tumors and cervical metastases in patients with squamous-cell carcinoma (SCC) of the head and neck, and to compare these values to the results of widely used morphological criteria and [18F]-FDG PET/CT findings. MATERIAL AND METHOD: This was a longitudinal, prospective, single-center nonrandomized trial involving patients with head and neck SCC treated with chemotherapy alone or in combination with radiotherapy. Imaging examinations ([18F]-FDG PET/CT and diffusion-weighted MRI) were performed on the same day, up to one day prior to the beginning of the first treatment cycle, and on the 14th day of the first chemotherapy cycle. Treatment response was evaluated based on the Response Evaluation Criteria in Solid Tumors (RECIST) and World Health Organization (WHO) morphological criteria, as well as PET Response Criteria in Solid Tumors (PERCIST) metabolic criteria. RESULTS: Seventy-five lesions were examined in 23 patients. Pre- and post-treatment comparisons of data pertaining to all target lesions revealed reductions in tumor size and SUV, as well as increases in ADC values, all of which were statistically significant. The increase in ADC following treatment was significantly higher in patients classified as complete responders by both morphological criteria than that observed in any of the other patient groups of response. Patients with a complete metabolic response also showed greater increases in ADC values as compared to the remaining groups. CONCLUSION: The assessment of tumor response based on diffusion-weighted MRI showed an increase in the ADC of cervical lesions following treatment, which was corroborated by morphological and metabolic findings. Associations between changes in ADC values and treatment response categories using morphologic criteria and [18F]-FDG PET/CT were only identified in complete responders.
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Carcinoma de Células Escamosas/terapia , Imagem de Difusão por Ressonância Magnética/métodos , Neoplasias de Cabeça e Pescoço/terapia , Tomografia por Emissão de Pósitrons/métodos , Tomografia Computadorizada por Raios X/métodos , Idoso , Carcinoma de Células Escamosas/diagnóstico por imagem , Quimiorradioterapia , Feminino , Fluordesoxiglucose F18 , Neoplasias de Cabeça e Pescoço/diagnóstico por imagem , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Ensaios Clínicos Controlados não Aleatórios como Assunto , Avaliação de Resultados em Cuidados de Saúde/métodos , Estudos Prospectivos , Indução de Remissão , Fatores de TempoRESUMO
Neurofibromatosis type 1 (NF1) has been associated with an increased risk for development of malignancy, especially malignant peripheral nerve sheath tumors. In addition, recently, literature has demonstrated an increased risk of breast cancer in women with NF1. The present paper shows a 53-year-old woman with NF1 who presented with metaplastic breast carcinoma and developed multiple metastases, including mandible. Furthermore, we reviewed the English literature, found 63 cases showing the association between NF1 and breast cancer, and added one more case. The present study demonstrated an important association between NF1 and breast cancer. Until the present time, there has been only one case of metaplastic breast carcinoma associated with NF1. Curiously, in our case the oral metastasis corresponded to sarcomatous component of metaplastic breast carcinoma.
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Introdução Carcinoma Epidermóide (CE) de Cabeça e Pescoço corresponde a sexta neoplasia mais incidente no mundo, levando ao diagnóstico de 300.000 novos casos ao ano. Dentre os fatores ambientais associados reconhecem-se o uso de tabaco e álcool, assim como discute-se atualmente o papel de agentes virais, tais quais o vírus Epstein Bar (EBV) em carcinoma de nasofaringe e do Papiloma Vírus Humano (HPV). O carcinoma epidermóide de orofaringe tem seu tratamento baseado na extensão de doença de acordo com características do tumor primário, extensão linfonodal regional e presença de metástase a distância de acordo com o sistema TNM. O uso associado da quimioterapia e radioterapia nos modos neoadjuvantes e concomitantes é alternativa estabelecida em pacientes diagnosticados com carcinoma epidermóide de orofaringe em estádios clínicos (EC) locoregionais avançados III e IV (M0). Discute-se atualmente o papel prognóstico da presença do HPV com seu potencial para direcionar terapia destes pacientes ao se propor a associação de quimioterapia a radioterapia, em complemento ao uso exclusivo da extensão tumoral com este intuito. Objetivos: Analisar os marcadores moleculares associados a carcinogênese pelo HPV por imunoistoquímica p16 e p53, correlacionando com variáveis clínicas e demográficas da população estudada e seu potencial papel prognóstico em pacientes tratados com associação de quimioterapia antineoplásica e radioterapia em pacientes diagnosticados com CE EC III-IV não metastáticos a distância.Pacientes e Métodos: O presente estudo analisa retrospectivamente 75 pacientes diagnosticados e tratados por carcinoma de orofaringe no Hospital A.C. Camargo entre 1995 e 2010 pela associação de quimioterapia e radioterapia nos estádios III e IV (M0), considerando variáveis sociodemográficas e clínicas e o potencial papel prognóstico das proteínas de regulação do ciclo celular associadas a infecção e carcinogênese pelo HPV p16 e p53 avaliadas por imunoistoquímica. Resultados: Analisados 75 pacientes, idade mediana 55 anos, 86,7% sexo masculino, 13,3% sexo feminino. Tabagismo e etilismo estavam presentes em 58,6% e 54,7% respectivamente. Estádio IV foi observado em 80% dos pacientes, e EC III em 20%. Taxa de resposta de 86,6% e probabilidade de sobrevida em 5 anos de 70,2% foram observadas. Extensão do tumor primário T4 versus T1 a T3, tabagismo e etilismo e obtenção de resposta completa ao tratamento demonstraram valor prognóstico significativo. Trinta pacientes tiveram variáveis analisadas à imunoistoquímica associadas ao HPV, p16 e p53 não apresentaram valor prognóstico nesta população. Conclusão: As taxas de resposta ao tratamento e sobrevida foram elevados. As variáveis preditoras significativamente associadas ao prognóstico foram extensão do tumor primário, uso de tabaco e álcool, e obtenção de resposta completa.
Background: Squamous-cell carcinoma of Head and Neck is the sixth most frequent neoplasia in the world accounting 300.000 new cases a year. Alcohol consumption and tobacco are widely recognized as risk factors, besides the viral infeccion by epistein-barr virus in nasopharyngeal cancer and human papillomavírus (HPV) in oropharyngeal carcinoma. Staging tumor by it extension according the TNM / AJCC system is currently the most important tool to access rognosis and guide treatment. Chemotherapy with radiotherapy is widely accepted as an treatment choice to locally advanced stage III-IV non-metastatic oropharyngeal cancer. Besides the TNM prognostic evaluation, there is a growing interest in the role of HPV infeccion as a prognostic factor in these tumors. The imunohistochemistry study of HPV related proteins p16 and p53 is currently discussed as a prognostic marker. Objectives: to evaluate molecular tumor markers correlated to HPV carcinogenesis by imunohistochemistry p16 and p53 testing, correlating to medical information and studying it potencial role as a prognostic factor in patients treated with chemoradiation for locally advanced oropharyngeal cancer. Patients and methods: It was retrospectively evaluated 75 stage III and IV nonmetastatic oropharyngeal cancer patients treated with chemotherapy and radiotherapy in the Hospital AC Camargo from 1995 to 2010. Sociodemographic and clinical information and p16 and p53 imunohistochemistry were evaluated as a potencial markers for prognostic information and association with a different patient profile. Results: Seventy five patients were evaluated for sociodemographic and clinical profile. Median age recorded was 55, male patients were 86.7%, female 13.3%. Tabacco and alcohol consumption were present in 58.6% and 54.7% respectively. Stage IV accounted 80% and EC III 20% of patients. Response rate of 86.6% and 5-year probability survival of 70.2% were recorded.Primary tumor extension T4 versus T1 to T3, tobacco and alcohol consumptions and complete response achievement were associated with significant survival benefit. Thirty of 75 patients had immunochemistry analysis for p16 and p53, wich demonstrated no difference in clinical outcome regarding efficacy evaluation and it role as a prognostic role in this patient population. Conclusion: High response rate and 5 year overall survival were recorded. Primary tumor extension T4, Tabacco and alcohol consumption and achievement of complete response were significantly associated with prognosis.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Carcinoma de Células Escamosas de Cabeça e Pescoço , Neoplasias de Cabeça e Pescoço , Radioterapia , Terapêutica , Tratamento FarmacológicoRESUMO
BACKGROUND: Gastric cancer is the second leading cause of death due to cancer worldwide and is particularly prevalent in Brazil. Promising new therapeutic agents have already shown activity in some gastrointestinal malignancies and their role in gastric cancer will need to be evaluated. Determining the prognostic factors of survival for patients with gastric cancer can help in identifying patients with a worse prognosis after treatment with the current chemotherapeutic regimens. METHODS: A retrospective chart review of 186 patients diagnosed with gastric cancer and treated at a single institution in Brazil from January 1994 to December 2004 was carried out. Univariate and multivariate analyses were performed to identify patient- and tumor-related characteristics associated with peritoneal metastasis at diagnosis and with overall survival. RESULTS: Of the 186 patients, 76 were alive at the time of this analysis. The median survival for all patients was 30.1 months. Two independent factors associated with the presence of peritoneal metastasis at diagnosis were identified by multivariate analysis: signet-ring cell type (odds ratio [OR], 10.8; 95% confidence interval [CI], 3.1 to 37.5), and visceral metastasis (OR, 51.8; 95% CI, 12.4 to 215.4). The prognostic factors for poor survival were tumor stage T3 or T4 (hazard ratio [HR], 1.87; 95% CI, 1.09 to 3.22) and visceral metastasis (HR, 4.98; 95% CI, 3.02 to 8.20). CONCLUSION: Two factors correlated with peritoneal metastasis and two prognostic factors for survival were identified. These findings may contribute to clinical decision-making, treatment tailoring, and the design of future trials.
Assuntos
Adenocarcinoma/mortalidade , Adenocarcinoma/terapia , Neoplasias Peritoneais/mortalidade , Neoplasias Peritoneais/terapia , Neoplasias Gástricas/mortalidade , Neoplasias Gástricas/terapia , Adenocarcinoma/secundário , Adulto , Antineoplásicos/uso terapêutico , Brasil/epidemiologia , Carcinoma de Células em Anel de Sinete/mortalidade , Carcinoma de Células em Anel de Sinete/secundário , Carcinoma de Células em Anel de Sinete/terapia , Quimioterapia Adjuvante , Feminino , Gastrectomia , Humanos , Estimativa de Kaplan-Meier , Masculino , Estadiamento de Neoplasias , Razão de Chances , Cuidados Paliativos , Neoplasias Peritoneais/secundário , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Neoplasias Gástricas/patologia , Fatores de Tempo , Resultado do TratamentoRESUMO
BACKGROUND: Peritoneal carcinomatosis is a common pattern of recurrence in gastric cancer and is associated with a poor prognosis. Determining predictive factors for peritoneal recurrence can help the selection of patients suitable for more aggressive treatment strategies. METHODS: A retrospective chart review of 162 patients diagnosed with gastric cancer with no peritoneal carcinomatosis and treated at a single institution in Brazil from January 1994 to December 2004 was carried out. Univariate and multivariate analyses were performed to identify patient and tumor-related characteristics associated with the development of peritoneal metastasis. RESULTS: Twenty-three (14.2%) patients developed peritoneal carcinomatosis. Three independent factors associated with the development of peritoneal metastasis were identified by multivariate analysis: signet-ring cell histology (odds ratio [OR] = 4.9; P = 0.018), the presence of vascular invasion (OR = 4.8; P = 0.022), and the presence of visceral metastasis at diagnosis (OR = 5.1; P = 0.011). Tumor stages T3 or T4 showed a trend towards significance (P = 0.062). CONCLUSIONS: Patients with gastric cancer presenting with signet-ring histology, vascular invasion, or visceral metastasis appear to be at higher risk for the development of peritoneal carcinomatosis.