Enfisema lobar congênito: série de casos de 30 anos em dois hospitais universitários

Objetivo: Revisar os casos de enfisema lobar congênito (ELC) operados nos últimos 30 anos em dois hospitais universitários. Métodos: Foram revistos os prontuários médicos das crianças com ELC operadas no Hospital das Clínicas da Faculdade de Medicina de Botucatu e no Hospital da Universidade de Mogi das Cruzes entre 1979 e 2009. Dados sobre sintomas, exame físico, achados radiológicos, diagnóstico, tratamento cirúrgico e seguimento pós-operatório mediato e tardio foram analisados. Resultados: Durante o período estudado, 20 crianças com ELC foram submetidas a tratamento cirúrgico. A média de idade dos pacientes no momento da cirurgia foi 6,9 meses (variação, 9 dias a 4 anos). Todos os casos apresentaram sintomas ao nascimento ou nos primeiros meses de vida. Em todos os casos, a radiografia simples de tórax foi importante na definição do diagnóstico. Em casos de dificuldade respiratória moderada, a TC de tórax auxiliou no diagnóstico. Um paciente com desconforto respiratório grave foi diagnosticado erroneamente com pneumotórax hipertensivo e submetido a drenagem torácica. A broncoscopia só foi realizada nos pacientes com dificuldade respiratória moderada e não foram encontrados sinais de anomalias traqueobrônquicas. A abordagem cirúrgica foi realizada através de toracotomia lateral poupadora de músculo. O lobo superior esquerdo e o lobo médio foram os mais afetados, seguidos pelo lobo superior direito. A lobectomia foi realizada em 18 casos, enquanto a bilobectomia foi realizada em 2 casos (com ressecção de cisto broncogênico em 1 desses). Complicações pós-operatórias não foram observadas. O acompanhamento pós-operatório foi de pelo menos 24 meses (média, 60 meses) e não foram observadas complicações tardias nesta série. Conclusões: O ELC é uma doença pouco comum, ainda negligenciada, de etiologia incerta, mas de fácil diagnóstico radiológico e tratamento cirúrgico eficaz.

Quantificação de fibrose e enfisema em pacientes com paracoccidioidomicose (PCM) pulmonar, através de imagens de tomografia computadorizada de alta resolução (TCAR)

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Tomographic and radiographic aspects of an infiltrative mediastinal lymphoma in a domestic cat with acute paraparesis: case report

Lymphomas are malignant neoplasms resulting from clonal proliferations of lymphocytes, originated from lymphoid organs, but can develop in any organ by the migration of lymphocytes to the organ's tissue. Lymphoma constitutes one of the most common neoplasms in cats. Imaging techniques like radiology, ultrasound and tomography may help in diagnosing and classifying lymphomas. Our objective is to report the radiographic and tomographic characteristics of a mediastinal lymphoma in a cat, which occupied a large thoracic and abdominal area and infiltrated into the medullary canal causing acute paraparesis. The tomographic exam was essential for identifying the mass, its extension and its location, besides identifying how compromised the adjacent structures were, but the histopathological examination was crucial to diagnose the nature of the lesion.

Punção aspirativa guiada por ultrassom endobrônquico no diagnóstico e estadiamento de linfadenopatia mediastinal: experiência inicial no Brasil

OBJETIVO: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA, punção aspirativa por agulha guiada por ultrassom endobrônquico) é um método novo em diagnóstico e estadiamento linfonodal mediastinal. O objetivo do estudo foi avaliar os resultados preliminares obtidos com EBUS-TBNA no diagnóstico de lesões e no estadiamento linfonodal mediastinal. MÉTODOS: Foram avaliados pacientes com tumores ou adenopatias mediastinais e com diagnóstico ou suspeita de câncer de pulmão. Os procedimentos foram realizados com os pacientes sob sedação ou anestesia geral. O material coletado foi preparado em lâminas fixadas em álcool absoluto para citologia e em formol para bloco de células. RESULTADOS: Foram incluídos 50 pacientes (30 do sexo masculino), com média de idade de 58,3 ± 13,5 anos. Foram realizadas 201 punções em 81 linfonodos ou massas mediastinais (média de 2,5 punções). O material obtido foi considerado adequado para análise citológica em 37 pacientes (74%), dos quais 21 (57%) foram diagnosticados com malignidade. Nos 16 pacientes remanescentes, 1 teve diagnóstico de tuberculose, 6 tiveram seguimento clínico, e 9 foram submetidos a investigação adicional (2 diagnosticados com neoplasia - resultados falso-negativos). O rendimento do exame foi maior nos procedimentos com objetivo diagnóstico, em pacientes com lesões em múltiplas estações, e nas punções da estação linfonodal subcarinal. Um paciente apresentou sangramento endobrônquico resolvido com medidas locais. Não houve mortalidade na série. CONCLUSÕES: Esta experiência preliminar confirmou que o EBUS-TBNA é procedimento seguro, e que o nosso rendimento diagnóstico, inferior ao da literatura, foi compatível com a curva de aprendizado do método.

Non-neoplastic bulky mediastinal mass presentation in an adolescent patient: a case report

Abstract : Introduction Mediastinal masses in pediatric patients are very heterogeneous in origin and etiology. In the first decade of life, 70% of the mediastinal masses are benign whereas malignant tumors are more frequent in the second decade of life. Among the mediastinal masses, lymph nodes are the most common involved structures and could be enlarged due to a lymphoma, leukemia, metastatic disease, or due to infectious diseases as sarcoidosis, tuberculosis and others. Case presentation. We report a case of a 13-year-old Caucasian girl who came to the emergency room with a history of intermittent fever, weight loss and night sweating for at least 1 month. A radiologic image work-up presented an anterior and posterior mediastinal mass. The 18F-fluorodeoxyglucose positron emission tomography presented a high maximum standard uptake value, which directed our decision for mediastinal biopsy for diagnostic elucidation. Histologic examination described the mass as granulomatous tuberculosis. The patient was treated with anti-tuberculosis therapy and developed a full clinical recovery. Conclusions . The present case report demonstrates that a bulky mediastinal lymphadenopathy detected on 18F-fluorodeoxyglucose positron emission tomography is not always a malignant lesion, and in countries where tuberculosis is endemic, this etiology should not be forgotten during clinical investigations. There is a need for more accurate cut-off values for this technology; meanwhile, the further investigation of patients with bulky mediastinal masses with procedures such as the open biopsy is indispensable.

Langerhans cell histiocytosis as differential diagnosis of a mediastinal tumor

We describe the case of a 55-year-old man who presented with parasternal swelling. The chest CT scan showed a large tumor of the chest wall infiltrating the subcutaneous tissue. To assume histologic diagnosis an open biopsy was performed. Between the myofibrils a coarse, white tumor with infiltrative growth was noted. Histopathologic examination revealed expanded atrophic skeletal muscle that was infiltrated by histiocytic cells. Numerous eosinophilic granulocytes and lymphocytes CD20 and CD3 positive could be detected and immunohistochemical staining was also positive for S-100 proteins and CD1a. Histologic findings were characteristic of Langerhans cell histiocytosis (LCH). To the best of our knowledge a LCH originating from the mediastinum in an adult as presented has not been previously described.

Treatment of mediastinal fibrosis with mycophenolate mofetil

We present the case of a 67-year-old male patient with mediastinal and retroperitoneal fibrosis. In Europe, this is a rare disease. Treatment was established to prevent complications due to strictures or compressions. Because of his diabetes, a therapy of low-dose prednisone combined with mycophenolate mofetil, known as steroid sparing agent, was applied. As a result, the radiological follow-up showed a marked decrease in the mediastinal and retroperitoneal masses.

Perioperative anaesthetic management of mediastinal mass in adults

Mediastinal mass syndrome remains an anaesthetic challenge that cannot be underestimated. Depending on the localization and the size of the mediastinal tumour, the clinical presentation is variable ranging from a complete lack of symptoms to severe cardiorespiratory problems. The administration of general anaesthesia can be associated with acute intraoperative or postoperative cardiorespiratory decompensation that may result in death due to tumour-related compression syndromes. The role of the anaesthesiologist, as a part of the interdisciplinary treatment team, is to ensure a safe perioperative period. However, there is still no structured protocol available for perioperative anaesthesiological procedure. The aim of this article is to summarize the genesis of and the diagnostic options for mediastinal mass syndrome and to provide a solid detailed methodology for its safe perioperative management based on a review of the latest literature and our own clinical experiences. Proper anaesthetic management of patients with mediastinal mass syndrome begins with an assessment of the preoperative status, directed foremost at establishing the localization of the tumour and on the basis of the clinical and radiological findings, discerning whether any vital mediastinal structures are affected. We have found it helpful to assign 'severity grade' (using a three-grade clinical classification scale: 'safe', 'uncertain', 'unsafe'), whereby each stage triggers appropriate action in terms of staffing and apparatus, such as the provision of alternatives for airway management, cardiopulmonary bypass and additional specialists. During the preoperative period, we are guided by a 12-point plan that also takes into account the special features of transportation into the operating theatre and patient monitoring. Tumour compression on the airways or the great vessels may create a critical respiratory and/or haemodynamic situation, and therefore the standard of intraoperative management includes induction of anaesthesia in the operating theatre on an adjustable surgical table, the use of short-acting anaesthetics, avoidance of muscle relaxants and maintenance of spontaneous respiration. In the case of severe clinical symptoms and large mediastinal tumours, we consider it absolutely essential to cannulate the femoral vessels preoperatively under local anaesthesia and to provide for the availability of cardiopulmonary bypass in the operating theatre, should extracorporeal circulation become necessary. The benefits of establishing vascular access under local anaesthesia clearly outweigh any associated degree of patient discomfort. In the case of patients classified as 'safe' or 'uncertain', a preoperative consensus with the surgeons should be reached as to the anaesthetic approach and the management of possible complications.

A rare case of a large spinal meningioma with mediastinal extension and malignant behavior classified histologically as benign

AIM To report a rare case of a spinal WHO grade I meningioma extending through intervertebral foramina C7 to D4 with an extensive mediastinal mass and infiltration of the vertebrae, and to discuss the malignant behavior of a tumor classified as benign. METHODS (Clinical Presentation, Histology, and Imaging): A 54-year-old man suffered from increasing lower back pain with gait difficulties, weakness and numbness of the lower extremities, as well as urge incontinence. CT scan of the thorax and MRI scan of the spine revealed a large prevertebral tumor, which extended to the spinal canal and caused compression of the spinal cord at the levels of C7 to D4 leading to myelopathy with hyperintense signal alteration on T2-weighted MRI images. The signal constellation (T1 with and without contrast, T2, TIR) was highly suspicious for infiltration of vertebrae C7 to D5. Somatostatin receptor SPECT/CT with (111)In-DTPA-D: -Phe-1-octreotide detected a somatostatin receptor-positive mediastinal tumor with infiltration of multiple vertebrae, dura, and intervertebral foramina C7-D4, partially with Krenning score >2. Percutaneous biopsies of the mediastinal mass led to histopathological findings of WHO grade I meningioma of meningothelial subtype. RESULTS (Therapy): C7 to D4 laminoplasty was performed, and the intraspinal, extradural part of the tumor was microsurgically removed. Postoperative stereotactic radiation therapy was done using the volumetric modulated arc therapy (VMAT) technique (RapidArc). No PRRNT with (90)Y-DOTA-TOC was done. CONCLUSIONS Due to the rare incidence and complex presentation of this disease not amenable to complete surgical resection, an individualized treatment approach should be worked out interdisciplinarily. The treatment approach should be based not only on histology but also on clinical and imaging findings. Close clinical and radiological follow-up may be mandatory even for benign tumors.

Novel radiotherapy techniques for involved-field and involved-node treatment of mediastinal Hodgkin lymphoma: when should they be considered and which questions remain open?

PURPOSE Hodgkin lymphoma (HL) is a highly curable disease. Reducing late complications and second malignancies has become increasingly important. Radiotherapy target paradigms are currently changing and radiotherapy techniques are evolving rapidly. DESIGN This overview reports to what extent target volume reduction in involved-node (IN) and advanced radiotherapy techniques, such as intensity-modulated radiotherapy (IMRT) and proton therapy-compared with involved-field (IF) and 3D radiotherapy (3D-RT)- can reduce high doses to organs at risk (OAR) and examines the issues that still remain open. RESULTS Although no comparison of all available techniques on identical patient datasets exists, clear patterns emerge. Advanced dose-calculation algorithms (e.g., convolution-superposition/Monte Carlo) should be used in mediastinal HL. INRT consistently reduces treated volumes when compared with IFRT with the exact amount depending on the INRT definition. The number of patients that might significantly benefit from highly conformal techniques such as IMRT over 3D-RT regarding high-dose exposure to organs at risk (OAR) is smaller with INRT. The impact of larger volumes treated with low doses in advanced techniques is unclear. The type of IMRT used (static/rotational) is of minor importance. All advanced photon techniques result in similar potential benefits and disadvantages, therefore only the degree-of-modulation should be chosen based on individual treatment goals. Treatment in deep inspiration breath hold is being evaluated. Protons theoretically provide both excellent high-dose conformality and reduced integral dose. CONCLUSION Further reduction of treated volumes most effectively reduces OAR dose, most likely without disadvantages if the excellent control rates achieved currently are maintained. For both IFRT and INRT, the benefits of advanced radiotherapy techniques depend on the individual patient/target geometry. Their use should therefore be decided case by case with comparative treatment planning.

Anaesthesiological management of mediastinal tumors

The perioperative management of patients with mediastinal masses is a special clinical challenge in our field. Even though regional anaesthesia is normally the first choice, in some cases it is not feasible due to the method of operation. In these cases general anaesthesia is the second option but can lead to respiratory and haemodynamic decompensation due to tumor-associated compression syndrome (mediastinal mass syndrome). The appropriate treatment begins with the preoperative risk classification on the basis of clinical and radiological findings. In addition to anamnesis, chest radiograph, and CT, dynamical methods (e.g. pneumotachography and echocardiography) should be applied to verify possible intraoperative compression syndromes. The induction of general anaesthesia is to be realized in awake-fiberoptic intubation with introduction of the tube via nasal route while maintaining the spontaneous breathing of the patient. The anaesthesia continues with short effective agents applied inhalative or iv. If possible from the point of operation, agents of muscle relaxation are not to be applied. If the anaesthesia risk is classified as uncertain or unsafe, depending on the location of tumor compression (tracheobronchial tree, pulmonary artery, superior vena cava), alternative techniques of securing the respiratory tract (different tubes, rigid bronchoscope) and cardiopulmonary bypass with extracorporal oxygen supply are prepared. For patients with severe clinical symptoms and extensive mediastinal mass, the preoperative cannulation of femoral vessels is also recommended. In addition to fulfilling technical and personnel requirements, an interdisciplinary cooperation of participating fields is the most important prerequisite for the optimal treatment of patients.