Malformação congénita das vias aéreas pulmonares e sequestro pulmonar no lactente – um diagnóstico raro

Herein is described the case of a two-month-old female infant with recurrent respiratory infections, admitted to the Pediatric Emergency Department with productive cough, respiratory distress, and fever. Chest radiography showed an heterogenous hypotransparent image in the left pulmonary field and contralateral deviation of the mediastinum. Angiotomography revealed bilateral bronchopulmonary sequestration and a systemic vessel contributing to area irrigation. The patient was submitted to vessel occlusion and lacquering, left inferior lobectomy, and resection of a right lower lobe segment. Histological examination confirmed intralobar bronchopulmonary sequestration and type 2 congenital pulmonary airway malformation in the left lung, and extralobar bronchopulmonary sequestration in the right lung. With this report, the authors intend to emphasize the importance of clinical suspicion of congenital anomalies in infants with recurrent respiratory infections.info:eu-repo/semantics/publishedVersio

Pierwotny mięsak naczyniowy osierdzia: opis przypadku i przegląd piśmiennictwa

Primary cardiac tumours are rare entities and angiosarcoma is the most frequent primary cardiac malignant tumour. Mean survival is six months and the tumour responds poorly to chemotherapy. We present the case of a 50 year-old patient with localised pericardial angiosarcoma who survived 23 months after diagnosis with a combined approach of chemotherapy and surgery. Kardiol Pol 2010; 68, 7: 802-80

Metastização pulmonar na apresentação de angiossarcoma cardíaco: Caso clínico e discussão Pulmonary metastasis in a cardiac angiosarcoma: Case report and discussion

Apresenta-se um caso clínico referente a doente de 35 anos, do sexo masculino sem antecedentes pessoais relevantes, admitido no serviço de urgência por quadro de toracalgia e tosse produtiva com alterações electrocardiograficas sugestivas de pericardite. Inicialmente admitido pelo Serviço de Cardiologia, com melhoria do quadro clínico apos terapêutica anti-inflamatória; contudo, no internamento houve como intercorrencia pneumonia de provável etiologia bacteriana, complicada por derrame pleural. Após a alta, foi referenciado a consulta de pneumologia, onde se manteve o estudo etiológico do derrame persistente, tendo vindo a complicar-se o seu quadro com alterações das cavidades cardíacas e múltiplos nódulos pulmonares, sugestivos de endocardite subaguda com embolização séptica pulmonar. Internado no serviço de Pneumologia e submetido a videotoracoscopia, foi-lhe diagnosticado angiossarcoma cardíaco com metastização pulmonar. Assistiu-se a uma rápida evolução do quadro clínico, quase fulminante, com falência cardíaca e óbito do doente sem ter iniciado radioterapia ou quimioterapia adjuvante.We present a case report of a 35 year-old male without any relevant former pathology admitted to the emergency room with atypical chest pain, cough and sputum with ECG changes suggesting pericarditis. He was initially admitted to the cardiology ward and experienced clinical improvement after initiating anti- inflammatory treatment. As intercurrence he had bacterial origin pneumonia complicated by pleural effusion (PE). After discharge patient was referred to a pulmonology appointment where aetiological investigation of the PE was instigated. Investigation revealed changes in the cardiac cavities and multiple lung nodules, suggesting subacute endocarditis with septic pulmonary embolism. Admitted to the pulmonology unit patient underwent videothoracsopy which diagnosed cardiac angiosarcoma with pulmonary metastisation. Within a few days there was a rapid evolution of the clinical picture, with cardiac failure and death of the patient without radiotherapy or adjuvant chemotherapy being started

Metastização pulmonar na apresentação de angiossarcoma cardíaco â Caso clínico e discussão

Resumo: Apresenta-se um caso clínico referente a doente de 35 anos, do sexo masculino sem antecedentes pessoais relevantes, admitido no serviço de urgência por quadro de toracalgia e tosse produtiva com alterações electrocardiográficas sugestivas de pericardite. Inicialmente admitido pelo Serviço de Cardiologia, com melhoria do quadro clínico após terapêutica anti-inflamatória; contudo, no internamento houve como intercorrência pneumonia de provável etiologia bacteriana, complicada por derrame pleural. Após a alta, foi referenciado à consulta de pneumologia, onde se manteve o estudo etiológico do derrame persistente, tendo vindo a complicar-se o seu quadro com alterações das cavidades cardiacas e múltiplos nódulos pulmonares, sugestivos de endocardite subaguda com embolização séptica pulmonar. Internado no serviço de Pneumologia e submetido a videotoracoscopia, foi-lhe diagnosticado angiossarcoma cardíaco com metastização pulmonar. Assistiu-se a uma rápida evolução do quadro clínico, quase fulminante, com falência cardíaca e óbito do doente sem ter iniciado radioterapia ou quimioterapia adjuvante.Rev Port Pneumol 2009; XV (6): 1175-1184 Abstract: We present a case report of a 35 year-old male without any relevant former pathology admitted to the emergency room with atypical chest pain, cough and sputum with ECG changes suggesting pericarditis. He was initially admitted to the cardiology ward and experienced clinical improvement after initiating anti-inflammatory treatment. As intercurrence he had bacterial origin pneumonia complicated by pleural effusion (PE). After discharge patient was referred to a pulmonology appointment where aetiological investigation of the PE was instigated. Investigation revealed changes in the cardiac cavities and multiple lung nodules, suggesting subacute endocarditis with septic pulmonary embolism. Admitted to the pulmonology unit patient underwent videothoracsopy which diagnosed cardiac angiosarcoma with pulmonary metastisation. Within a few days there was a rapid evolution of the clinical picture, with cardiac failure and death of the patient without radiotherapy or adjuvant chemotherapy being started.Rev Port Pneumol 2009; XV (6): 1175-1184 Palavras-chave: Angiossarcoma cardíaco, metástases pulmonares, derrame pleural, pericardite, Key-words: Heart angiosarcoma, pulmonary metastasis, pleural effusion, pericarditi

Surgical resection of Masaoka stage III thymic epithelial tumours with great vessels involvement : a retrospective multicentric analysis from the European Society of Thoracic Surgeons thymic database

Objectives: The aim of this study was to analyse the outcomes of an international cohort of patients affected by Masaoka stage III thymic epithelial tumours with vascular involvement and treated by surgery. Methods: Study design was the observational multicentre retrospective cohort study. Data were extracted from the European Society of Thoracic Surgeons thymic database; additional variables were collected. Inclusion criteria were as follows: stage III (Masaoka-Koga) thymic epithelial tumours; surgery with radical intention; clinical or pathological great vessels involvement; and radiologically suspected or diagnosed intraoperatively. Outcome items were analysed. Results: Sixty-five patients submitted to surgery from 2001 to 2017 fulfilled inclusion criteria. Thymoma and thymic carcinoma patients did not differ for demographics and clinical characteristics. The majority of great vessel treated were superior vena cava or innominate veins (72.3%). Eleven patients (16.9%) had postoperative cardiopulmonary complications; vascular stenosis was observed in 3 patients (4.6%). The multivariable Cox analysis for disease-free survival showed an increased hazard of recurrence for thymic carcinoma (hazard ratio = 3.59; 95% confidence interval: 1.66-7.78, P = 0.001). The 1-, 3-, 5- and 10-year overall survival rates were 0.86, 0.84, 0.81, and 0.53, respectively. There was no significant difference in overall survival according to resection status or between thymoma and thimic carcinoma. The univariable Cox regression model did not show an increased hazard of death for myasthenic patients considering all resection status and for patients who received neoadjuvant therapy. Conclusions: We observed that clinical outcomes of patients treated for stage III thymic epithelial tumours with vascular involvement are satisfactory suggesting to increase the confidence in dealing with these complex surgeries. Complete resection should be achieved, even though extensive vascular reconstructions are required

Thymomectomy plus total thymectomy versus simple thymomectomy for early-stage thymoma without myasthenia gravis : a European Society of Thoracic Surgeons Thymic Working Group Study

OBJECTIVES: Resection of thymic tumours including the removal of both the tumour and the thymus gland (thymothymectomy; TT) is the procedure of choice and is recommended in most relevant articles in the literature. Nevertheless, in recent years, some authors have suggested that resection of the tumour (simple thymomectomy; ST) may suffice from an oncological standpoint in patients with early-stage thymoma who do not have myasthenia gravis (MG) (non-MG). The goal of our study was to compare the short- and long-term outcomes of ST versus TT in non-MG early-stage thymomas using the European Society of Thoracic Surgeons thymic database.METHODS: A total of 498 non-MG patients with pathological stage I thymoma were included in the study. TT was performed in 466 (93.6%) of 498 patients who had surgery with curative intent; ST was done in 32 (6.4%). The completeness of resection, the rate of complications, the 30-day mortality, the overall recurrence and the freedom from recurrence were compared. We performed crude and propensity score-adjusted comparisons by surgical approach (ST vs TT).RESULTS: TT showed the same rate of postoperative complications, 30-day mortality and postoperative length of stay as ST. The 5-year overall survival rate was 89% in the TT group and 55% in the ST group. The 5-year freedom from recurrence was 96% in the TT group and 79% in the ST group.CONCLUSION: Patients with early-stage thymoma without MG who have a TT show significantly better freedom from recurrence than those who have an ST, without an increase in postoperative morbidity rate