Search

Abstract Background Ovarian clear cell carcinomas (OCCCs) are rare, aggressive and chemoresistant tumors. Geographical and ethnic differences in the incidence of OCCC have been reported with a higher incidence in Asiatic countries. There is a paucity of information regarding OCCC in Latin America (LA) and other countries. Methods Here, we characterized two cohorts of 33 patients with OCCC from LA (24 from Brazil and 9 from Costa Rica) and a cohort of 27 patients from Spain. Genomic analysis was performed for 26 OCCC using the OncoScan platform. Tumors were classified according to their genomic landscapes into subgroups. Clinical parameters were related to the frequency of genomic aberrations. Results The median overall survival (OS) was not significantly different between the cohorts. Genomic landscapes were characterized by different homologous recombination deficiency (HRD) levels. No difference in the distribution of genomic landscapes profiles was detected between patients from the different cohorts. OCCCs with MYC-amplified tumors harboring a concomitant loss of a region in chromosome 13q12-q13 that includes the BRCA2 gene had the longest OS. In contrast, patients carrying a high number (> 30) of total copy number (CN) aberrations with no concomitant alterations in MYC and BRCA2 genes presented the shortest OS. Furthermore, amplification of the ASH1L gene was also associated with a shorter OS. Initial-stage OCCCs with early progression were characterized by gains in the JNK1 and MKL1 genes. Conclusions Our results provide new data from understudied OCCC populations and reveal new potential markers for OCCCs.

Abstract Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant disorder without a good genotype–phenotype correlation, characterized by tumor predisposition in the parathyroid gland, anterior pituitary, and pancreatic islet cells. Here, we describe a 37‐year‐old male with previous history of nephrolithiasis, with a 1‐year history of recurrent hypoglycemic episodes. Physical examination revealed the presence of two lipomas. Family history revealed primary hyperparathyroidism (PHPT), hyperprolactinemia, and multiple non‐functioning pancreatic neuroendocrine tumors. Initial laboratories revealed hypoglycemia and primary hyperparathyroidism. A fasting test was positive after 3 hours of initiation. An abdominal CT Scan demonstrated a 28 × 27 mm mass in the pancreatic tail and bilateral nephrolithiasis. A distal pancreatectomy was done. After surgery, the patient persisted with hypoglycemic episodes that were managed with diazoxide and frequent feedings. A parathyroid Tc‐99 m MIBI scan with SPECT/CT imaging demonstrated two hot uptake lesions compatible with abnormally functioning parathyroid tissue. Surgical treatment was offered; however, the patient decided to postpone the procedure. Direct sequence analysis of MEN1 gene revealed heterozygosity for a pathogenic insertion c.1224_1225insGTCC (p.Cys409Valfs*41). DNA sequence analysis was done to six of his first‐degree relatives. A sister with clinical diagnosis of MEN1 and a pre‐symptomatic brother were positive for the same MEN1 variant. To our knowledge, this is the first report of a genetically confirmed case of MEN1 in our country and is the first report in literature of the c.1224_1225insGTCC variant related to a clinically affected family.

OBJETIVO: Avaliar a influência da técnica utilizada na dissecção das artérias torácicas na evolução de pacientes diabéticos submetidos a revascularização sem CEC. MÉTODOS: Setenta pacientes diabéticos submetidos a revascularização sem CEC com duas artérias torácicas foram avaliados. No grupo A, as artérias torácicas foram dissecadas de modo convencional, enquanto no grupo B foram esqueletizadas. RESULTADOS: A idade média do grupo A foi de 52,14±7,35 anos contra 55,71±8,1 anos no grupo B (p=0,057). No grupo A, seis (17,1%) pacientes eram diabéticos insulinodependentes contra nove (25,7%) no grupo B (p=0,561). O EUROSCORE foi de 3,97±2,49 para o grupo A contra 4,14±3,06 no grupo B (p=0,879). O número médio de anastomoses distais no grupo A foi de 3±0,77 contra 3,03±0,89 para o grupo B (p=0,981). Três (8,57%) dos pacientes do grupo A apresentaram mediastinite contra nenhum do grupo B (p=0,239). A diabetes insulino-dependente foi o único fator estatisticamente significativo (p=0,008) para mediastinite. Neste grupo, a utilização de artéria torácica interna esqueletizada diminuiu significativamente a incidência de mediastinite (p=0,044). CONCLUSÃO: A incidência de mediastinite foi menor no grupo onde ambas as artérias torácicas foram dissecadas de forma esqueletizada, apesar de, devido ao baixo número de casos, não apresentar diferença estatística. Nos portadores de diabetes insulino-dependente, 50% dos pacientes do grupo em que a artéria torácica foi obtida de forma convencional apresentaram mediastinite, sendo que a utilização de artéria torácica esqueletizada diminuiu significativamente a incidência de mediastinite.OBJECTIVE: To evaluate the influence of the technique used in the dissection of thoracic arteries in the evolution of diabetic patients submitted to OPCAB. METHODS: Seventy diabetic patients submitted to OPCAB using bilateral thoracic arteries were evaluated. In Group A, thoracic arteries were dissected as a pedicle, while in Group B they were skeletonized. RESULTS: The mean age of patients in Group A was 52.14 ± 7.35 years old versus 55.71 ± 8.1 years for Group B (p=0.057). In Group A, six patients (17.1%) were insulin dependent against nine (25.7%) in Group B (p = 0.561). The EUROSCORE was 3.97 ± 2.49 for Group A opposed to 4.14 ± 3.06 for Group B (p = 0.879). The number of distal anastomoses in Group A was 3 ± 0.77 versus 3.03 ± 0.89 in Group B (p = 0.981). Three patients (8.57%) from Group A presented with mediastinitis. Insulin dependence was the only significant risk factor (p=0.008) for mediastinitis. In this group the use of skeletonized internal thoracic arteries significantly decreased the incidence of mediastinitis (p = 0.044). Conclusion: The incidence of mediastinitis was lower in the group for which mammary arteries were dissected using skeletonization. Among insulin-dependent diabetics, 50% of the patients from the group in which the pedicled internal thoracic artery was utilized presented with mediastinitis; the utilization of skeletonized internal thoracic arteries significantly decreases the incidence of mediastinitis.

FUNDAMENTO: A fibrilação atrial é a mais comum complicação no pós-operatório de revascularização miocárdica, aumentando a incidência de morbi-mortalidade. OBJETIVO: O propósito deste estudo prospectivo e randomizado foi testar a hipótese de que a estimulação cardíaca temporária biatrial reduz a incidência da fibrilação atrial no pós-operatório de revascularização miocárdica. MÉTODOS: Em uma casuística de 98 pacientes não-consecutivos, submetidos a revascularização miocárdica sem circulação extracorpórea, foram implantados respectivamente dois eletrodos temporários em átrio direito e em átrio esquerdo e conectados a cada par de saída atrial do marcapasso, além dos eletrodos implantados no ventrículo direito. Foram randomizados dois grupos (controle: 49 pacientes sem a estimulação biatrial; terapêutico: 49 pacientes com a estimulação biatrial). As variáveis de interesse foram: fibrilação atrial (presença ou não), tempo de hospitalização. RESULTADOS: A incidência de fibrilação atrial foi de 36,73% no grupo controle e 14,29% no grupo terapêutico (p=0,0194). O tempo de hospitalização foi de 7,00±2,82 dias nos pacientes sem fibrilação atrial (n=73), e de 9,20±2,87 dias nos pacientes com fibrilação atrial (n=25) (p=0,0001). A idade foi importante preditor da arritmia, variou de 62,34±9,00 anos no grupo sem fibrilação atrial, e de 67,20±7,42 anos no grupo com fibrilação atrial (p=0,0170). CONCLUSÃO: A estimulação temporária biatrial profilática é efetiva na prevenção da fibrilação atrial, quando comparada ao grupo controle. Permanência hospitalar foi maior nos pacientes que apresentaram fibrilação atrial no pós-operatório e a idade foi importante preditor para o desenvolvimento da arritmia.BACKGROUND: Atrial fibrillation is the most common complication after myocardial revascularization, and it increases morbidity/mortality. OBJECTIVE: The purpose of this prospective randomized study was to test the hypothesis that temporary biatrial pacing is effective in reducing the incidence of postoperative atrial fibrillation after myocardial revascularization. METHODS: Ninety-eight non-consecutive patients who had undergone off-pump myocardial revascularization received two temporary electrodes attached to the right and left atria, which were connected to either pair of atrial pacemaker electrodes, in addition to the leads implanted in the right ventricle. Two groups of patients were randomized (control: 49 patients with no biatrial pacing; therapeutic: 49 patients with biatrial pacing). The variables of interest were atrial fibrillation (present or absent) and length of hospital stay. RESULTS: The incidence of atrial fibrillation was 36.73% in the control group and 14.29% in the therapeutic group (p=0.0194). Length of hospital stay was 7.00 ± 2.82 days for patients with no atrial fibrillation (n=73) and 9.20 ± 2.87 days for patients with atrial fibrillation (n=25) (p=0.0001). Age was an important predictor of arrhythmia and ranged between 62.34 ± 9.00 years in the group with no atrial fibrillation and 67.20 ± 7.42 years in the group with atrial fibrillation (p=0.0170). CONCLUSION: Compared to controls, prophylactic temporary biatrial pacing is effective in preventing atrial fibrillation. Hospital stay was longer for patients who developed postoperative atrial fibrillation, and age was an important predictor for the development of arrhythmia.

OBJETIVO: Avaliar os resultados imediatos das anastomoses mecânicas aorto-safena, comparando-as às anastomoses convencionais. MÉTODO: Foram analisados 12 pacientes, com idade média de 62,33 ± 7,30 anos, sendo 10 (83,3%) do sexo masculino. Os pacientes foram submetidos à operação sem circulação extracorpórea, com 33 anastomoses proximais avaliadas, sendo 21 anastomoses convencionais e 12 feitas com o conector aórtico Symmetry da St. Medical. Foram analisados tempo gasto para confecção da anastomose, fluxo livre com pressão arterial sistólica de 100 mmHg e patência no 4º dia de pós-operatório. RESULTADOS: A anastomose mecânica foi empregada com sucesso em todos os pacientes. Um paciente apresentou alterações eletrocardiográficas compatíveis com IAM, no 2º dia de pós-operatório, sendo encaminhado para reestudo angiográfico, que evidenciou oclusão de anastomose proximal convencional para ramo marginal. Três pacientes apresentaram fibrilação atrial. Não houve óbitos nesta série. O tempo médio gasto para realização da anastomose mecânica foi de 44,08 ± 9,26 segundos contra 3,86 ± 0,61 minutos da anastomose convencional (p = 0,0022). O fluxo livre médio observado nas anastomoses mecânicas foi de 302,75 ± 82,76 ml/min contra 190,75±51,53 ml/min (p = 0,0022). No reestudo angiográfico, feito no 4º pós-operatorio, detectou-se oclusão de três anastomoses mecânicas contra nenhuma nova oclusão das anastomoses convencionais (p = 0,2500). CONCLUSÃO: O presente estudo apresentou uma superioridade estatisticamente significativa em favor das anastomoses mecânicas da veia safena com aorta, quando avaliado tempo para confecção da anastomose e fluxo livre. Em relação à condição da artéria na angiografia pósoperatória, não se pode afirmar que exista diferença estatisticamente significativa entre os procedimentos estudados.OBJECTIVE: To evaluate the immediate results of mechanical aorto-saphenous anastomosis compared with conventional anastomosis. METHOD: We evaluated 12 patients. The mean age ranged from 62.33 ± 7.30 years. Of 12 patients submitted to surgery without extracorporeal circulation, 10 (83.3%) patients were male. Thirty-three proximal anastomoses were evaluated, 21 of them being performed through the conventional manner and 12 with the St. Jude Symmetry aortic connector. The time spent on anastomosis, and free flow and patency on the 4th day postoperative were analysed. RESULTS: The mechanical anastomosis was successfully performed in all patients. Electrocardiographic alteration compatible with myocardial infarctation (MI) on the 2nd day postoperative was observed only in one patient. The patient was referred to angiographic restudy, becoming evident a conventional proximal anastomosis occlusion for the marginal branch. Three patients had atrial fibrillation. The average time spent to perform the mechanical anastomosis was 44.08 ± 9.26 seconds against 3.86 ± 0.61 minutes of the conventional anastomosis (p = 0.0022). The average blood free flow observed in the mechanical anastomosis was 302.75 ± 82.76 mL/min versus 190.75 ± 51.53 mL/min (p = 0.0022). In the angiographic restudy performed on the 4th postoperative day, it was detected the occlusin of three mechanical anastomosis. There was no new conventional anastomosis (p = 0.2500). CONCLUSION: The present study showed a statistically significant superiority for mechanical anastomosis of the saphenous vein with the aorta when evaluated the blood free flow and the time to perform the anastomosis. In relation to the artery condition in the postoperative angiography, one cannot say there was statistically significant difference between the procedures studied.

OBJETIVO: Analisar evolução hospitalar dos pacientes portadores de insuficiência renal crônica (IRC) em hemodiálise, submetidos a operação sem circulação extracorpórea (CEC). MÉTODO: Cinqüenta e um pacientes portadores de IRC foram submetidos à operação sem CEC. A hemodiálise foi realizada no dia anterior à operação e no dia seguinte. A revascularização do miocárdio foi realizada com ponto de LIMA e estabilizador por sucção. RESULTADOS: A idade média foi de 61,28±11,09 anos e 31 (58,8%) pacientes eram do sexo feminino. A classe funcional predominante foi a IV em 21 (41,1%) dos pacientes. A fração de ejeção do ventrículo esquerdo era ruim em 21 (41,1%) pacientes. O EUROSCORE médio desta série de pacientes foi de 7,65±3,83. O número médio de artérias coronárias revascularizadas foi de 3,1±0,78 por paciente. O tempo médio de ventilação mecânica foi de 3,78±4,35 horas. A permanência média na CTI foi de 41,9±13,8 horas, enquanto a média de permanência hospitalar foi de 6,5±1,31 dias. Quanto às complicações, nove (17,6%) pacientes desenvolveram FA e um (1,9%) apresentou quadro de AVC isquêmico, com boa recuperação durante a internação. Não houve óbito nesta série. CONCLUSÃO: Pacientes renais crônicos submetidos à hemodiálise sempre foram uma população de alto risco para revascularização do miocárdio. A ausência de CEC, aparentemente, cursa com baixos índices de morbi-mortalidade nesta população.OBJECTIVE: To analyze the hospital outcomes of patients, with chronic renal insufficiency in the hemodialysis, submitted to OPCAB. METHOD: Fifty-one patients with chronic renal insufficiency were submitted to OPCAB. Hemodialysis was performed on the day before and the day after the operation. Myocardial revascularization was performed using LIMA's suture and suction stabilization. RESULTS: Fifty-one patients, with an averageof 61.28±11.09 years, were analyzed. Thirty patients (58.8%) were female. The predominant functional class was IV in 21 (41.1%) of the patients. The left ventricle ejection fraction was dire in 21 (41.1%) patients. The mean EUROSCORE of this series was 7.65±3.83 and the mean number of distal anastomosis was 3.1±0.78 per patient. The average time of mechanical ventilation was 3.78±4.35 hours and the mean ICU stay was 41.9±13.8 hours, while the average hospitalization was 6.5±1.31 days. In respect to complications, nine (17.6%) of the patients developed atrial fibrilation, and one (1.9%) patient presented with a case of ischemic stroke but had a good recovery during hospitalization. There were no deaths in this series. CONCLUSION: Chronic renal patients submitted to hemodialysis were always a high risk population for myocardial revascularization. In this series, the absence of extracorporeal circulation appeared to be safe and efficient in this special subgroup of patients. The operations were performed with low indices of complications, absence of deaths and relatively low stays in the ICU and in hospital.

OBJETIVO: Recentemente, o uso de múltiplos enxertos arteriais nas cirurgias coronarianas vem conquistando grande interesse da comunidade médica diante da perspectiva de melhores resultados a longo prazo em relação às veias safenas. O presente estudo tem por objetivo analisar os resultados imediatos da associação da operação de revascularização do miocárdio sem circulação extracorpórea, com o uso exclusivo de enxertos arteriais. MÉTODO: Entre junho de 2000 a dezembro de 2004, 300 pacientes foram submetidos a operação para revascularização do miocárdio sem circulação extracorpórea, usando-se apenas enxertos arteriais. A artéria torácica interna esquerda foi o enxerto de primeira escolha, seguida da artéria radial do membro superior não-dominante, artéria torácica interna direita e artéria radial do membro dominante. RESULTADO: A idade dos pacientes variou entre 33 e 77 anos. Duzentos e trinta e quatro pacientes eram do sexo masculino e 66 do sexo feminino. Com relação a fatores de risco coronarianos, 77% tinham história de hipertensão arterial, 66% história de tabagismo, 53% níveis elevados de colesterol e 21% eram diabéticos. Oitenta e quatro (28%) pacientes apresentavam antecedente de infarto do miocárdio e 77 (25,6%) necessitaram de nitroglicerina endovenosa no período pré-operatório. A fração de ejeção era inferior a 30% em 77 (25,6%) pacientes e a cineangiocoronariografia mostrou lesão em três ou mais vasos em 63% dos casos. O EuroSCORE variou de 0 a 12 pontos, com uma mortalidade esperada para este grupo de 3,7%. O número total de anastomoses distais foi de 838, com média de 2,79±0,97 por paciente. Houve seis (2%) óbitos nesta série, sendo dois por mediastinite, um por insuficiência renal após choque séptico, um por causas metabólicas, um por sangramento e um por broncopneumonia. Diabetes foi o único fator associado com aumento na mortalidade. CONCLUSÃO: A utilização de enxertos arteriais nas operações para revascularização do miocárdio sem circulação extracorpórea não acarretou aumento da morbi-mortalidade imediata nesta série. Os resultados obtidos estão dentro do previsto através do EuroSCORE. O uso de enxertos arteriais em diabéticos deve ser realizado de maneira bastante criteriosa.OBJECTIVE: The present study reviews our immediate experience of off-pump coronary artery bypass grafting using arterial grafts alone. METHOD: Between June 2000 and December 2004, 300 patients were submitted to off-pump myocardial revascularization using only arterial grafts. The left internal mammary artery was the first-choice graft, followed by radial artery and the right mammary artery. RESULTS: The ages of the patients ranged from 33 to 77 years, with 234 male and 66 female. In respect to risk factors for coronary disease, 77% had hypertension, 66% had history of smoking, 53% had high levels of cholesterol and 21% had diabetes. Eighty-four patients (28%) had a history of myocardial infarction and 77 (25.6%) were using endovenous nitroglycerin in the preoperative period. The ejection fraction was less than 30% in 77 (25.6%) patients. A total of 189 patients had multi-vessel disease. The EuroSCORE ranged from 0 to 12 points with an expected mortality rate of 3.7%. The total numbers of distal anastomoses were 838, with a mean of 2.79±0.97 anastomoses per patient. There were six deaths in this series, one caused by renal failure, one caused by metabolic disorders, two caused by mediastinitis, one caused by pneumonia and one caused by bleeding. Diabetes was the only factor associated with mortality. CONCLUSION: The use of arterial grafts in off-pump myocardial revascularization did not increase the immediate mobidity and mortality in this series. The results were similar to the results predicted by EuroSCORE. The use of arterial grafts alone in diabetics patients must be carefully evaluated.

Objective: To assess off-pump myocardial revascularization in patients with significant left ventricular dysfunction., Methods: Four hundred and five patients with an ejection fraction less than 35% underwent myocardial revascularization without extracorporeal circulation. The procedure was performed with the aid of a suction stabilizer and the LIMA stitch. The distal anastomoses were performed first., Results: A total of 405 patients were evaluated whose mean age was 63.4 +/- 9.78 years. Two hundred and seventy-nine patients were men (68.8%). With regard to risk factors, 347 patients were hypertensive, 194 were smokers, 202 were dyslipidemic, and 134 had diabetes. Two hundred and sixty patients were classified as NYHA functional class III and IV. Twenty patients suffered from chronic renal disease and were under dialysis. Fifty-one underwent emergency surgery, and 33 had been previously operated on. The mean ejection fraction was 27.2 +/- 3.54%. The mean EuroSCORE was 8.46 +/- 4.41. The mean number of anastomoses performed was 3.03 +/- 1.54 per patient. Forty-nine patients (12%) needed an intra-aortic balloon inserted after induction of anesthesia, whereas 73 (18%) needed inotropic support during the perioperative period. As to complications, 2 patients (0.49%) had renal failure, 2 had mediastinitis (0.49%), 7 (1.7%) needed to be reoperated because of bleeding, 5 patients (1.2%) suffered acute myocardial infarction, and 70 patients (17.3%) experienced atrial fibrillation. Eighteen (4.4%) patients died., Conclusion: Based on the data above, we concluded that myocardial revascularization without extracorporeal circulation in patients with left ventricular dysfunction is a safe and effective technique, and an alternative for high-risk patients. Results obtained were better than those predicted by EuroSCORE.