A case of pulmonary inflammatory myofibroblastic tumor treated with bronchoscopic therapy plus lobectomy.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is a rare tumor with malignant potential. We presented a case of a young adult who was diagnosed with IMT and treated with loop electrocautery therapy to relieve airway obstruction, followed by lobectomy to complete resection. Recent studies have supported the use of such interventional resection methods. CASE PRESENTATION: A non-smoking 30-year-old woman presented with a 1-month history of progressive dyspnea and productive cough. The Chest X-ray showed a homogenous opacity invading the entire left hemithorax, and the mediastinum content was attracted to the left side. In an effort to avoid pneumonectomy and afford rapid palliation of dyspnea, loop electrocautery was selected as the most appropriate therapy. The left upper lobectomy by thoracoscopy was performed instead of left upper lobe sleeve resection in order to better prevent the recurrence of lung atelectasis. After 6 years of follow-up, no evidence of recurrence has been found till now. CONCLUSION: Interventional bronchoscopy coupled with surgical resection serves not only as a palliative management to bronchial obstruction but also a way to avoid pneumonectomy.

Recurrent pulmonary embolization of inflammatory myofibroblastic tumor: a case report.

We report a case of inflammatory myofibroblastic tumor affecting the pulmonary artery in a 15-year-old male, presenting with a clinical scenario of recurrent pulmonary embolisms. During diagnostic workup for persistent fever, a mass in main pulmonary artery was detected at echocardiography and confirmed at angio-CT scan. The patient underwent a first successful surgical resection and discharged home with no echocardiographic evidence of residual lesions, but, after 5 months, he was admitted for hemoptysis and an angio CT-scan showed a mass in right pulmonary artery with multiple distal perfusion defects, suspicious for both thrombotic and secondary lesions. To prevent further embolisms, the patient was scheduled for a second surgical procedure, which allowed a complete removal of the tumor from major branches of right pulmonary arteries. Our experience highlights that, despite of its intermediate malignancy, inflammatory myofibroblastic tumor may behave as an extremely dangerous condition, requiring multiple surgeries an integrated and multidisciplinary approach.

Afectación pulmonar bilateral. Opciones no quirúrgicas del pseudotumor inflamatorio, a propósito de un caso / Bilateral Pulmonary Involvement. Non-Surgical Options for Inflammatory Pseudotumor - A Case Report

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A Curious Case of Cough in a Young Woman

Inflammatory myofibroblastic tumour (IMT) is a rare neoplasm derived from tissue of mesenchymal origin. This tumour occurs predominantly in the lung, though extrapulmonary sites have been documented throughout the body.1 Presentation can be variable depending on location of the tumour and can include constitutional symptoms (fever, weight loss), thrombocytosis, hypergammaglobulinemia, anemia, and mass effect on local structures.2 Majority of patients with intrapulmonary IMT remain asymptomatic but can have symptoms including chest pain, dyspnoea, cough or haemoptysis.3 Most cases of IMT present in younger individuals (under age 40).4 Although the aetiology of IMT is unclear, current hypotheses suggest an inflammatory response to infection or an underlying malignancy could promote such cellular changes. Alternatively, the inflammatory component itself may be a consequence of the development of these mesenchymal tumours.

Inflammatory myofibroblastic lung tumor with dysphagia in a 4-year-old child.

Inflammatory myofibroblastic tumors of the lung are rare in adults but common among pediatric lung tumors. A 4-year-old girl was evaluated for respiratory symptoms, dysphagia, and weight loss. Radiology revealed a right hilar mass that persisted despite antibiotic treatment. On exploration, a hilar mass involving the pulmonary vasculature, diaphragm and gastroesophageal junction was found. Aggressive surgical excision including a right pneumonectomy was performed. Histopathology confirmed an inflammatory myofibroblastic tumor with no features of malignancy. At the 2-year follow-up, the child was clinically stable with no recurrence. Dysphagia is a very rare presentation of such tumors.

Pseudotumor fibroso inflamatorio pulmonar / No disponible

Los pseudotumores inflamatorios, también conocidos como granulomas de células plasmáticas, son una entidad de naturaleza inflamatoria y de causa desconocida, que pueden afectar a varios órganos, siendo la localización pulmonar infrecuente. Representan, según la literatura, el 0,7% de todas las tumoraciones pulmonares. Se considera un proceso benigno, que se caracteriza por un crecimiento anormal descontrolado de células inflamatorias cuya mejor opción terapéutica es la resección quirúrgica con buenos resultados, aunque se han descrito casos de recidiva a pesar de resección completa. Presentamos el caso de un paciente de 67 años con una masa pulmonar con diagnóstico definitivo de pseudotumor inflamatorio

Inflammatory pseudotumors, also known as plasmatic cell granulomas, are an inflammatory entity, of unknown origin, that can affect many organs, with an infrequent pulmonary localization, representing, 0,7% of all the lung tumors. It is considered to be a benign process, characterized by an abnormal and uncontrolled growth of inflammatory cells. The better therapeutic choice is surgical resection with good results, although it has been described recurrence despite complete resection. We present the case of a 67-year-old patient with a lung mass with definitive diagnosis of Inflammatory pseudotumor

Función pulmonar y obesidad / Changes in lung function testing associated with obesity

La obesidad puede afectar, a través de mecanismos inflamatorios y alteraciones ventilatorias, la función pulmonar y la capacidad al ejercicio. Los sujetos obesos desarrollan, de esta forma, un tipo de respiración más rápida y superficial que la de personas sin obesidad. También a nivel de la vía aérea se han detectado cambios, observando mayores resistencias en los obesos. Sin embargo, todas estas alteraciones no siempre se han acompañado de un menor rendimiento en las pruebas de esfuerzo cardiorespiratorias. Probablemente la forma en que se expresa su capacidad al ejercicio pueda explicar parte de estos resultados contradictorios (AU)

Excess bodyweight has an important impact on the physiology of breathing. In fact, it affects resting lung volumes and exercise capacity. These effects appear as a consequence of ventilatory and inflammatory changes commonly associated to obesity. As a result, obese individuals have a rapid and shallow pattern of breathing, their respiratory compliance is reduced and the airway resistance tends to be higher. However, with respect to aerobic capacity, contradictory results have been reported depending on the way peak oxygen uptake is expressed. Moreover, the inability of this population to achieve maximal efforts during exercise testing also affects their cardiorespiratory fitness (AU)

Case report of anti-transcription intermediary factor-1-γ/α antibody-positive dermatomyositis associated with gastric cancer and immunoglobulin G4-positive pulmonary inflammatory pseudotumor.

Dermatomyositis is a rare connective tissue disease often associated with internal malignancy and interstitial pneumonitis. Serologically, various auto-antibodies (Ab) are associated with dermatomyositis. Anti-transcription intermediary factor-1-γ/α (TIF-1-γ/α) Ab was recently identified as an auto-Ab and was observed mostly in cancer-associated dermatomyositis. IgG4-related disease is a newly described entity characterized by increased serum IgG4 levels and IgG4-positive plasma cell infiltration with fibrosis in organs such as the pancreas and parotid gland. IgG4-related disease also includes inflammatory pseudotumors in various organs. We report herein a 59-year-old Japanese man who had dermatomyositis complicated with a gastric cancer and an IgG4-related pulmonary inflammatory pseudotumor. He manifested typical classical Gottron's papules on the fingers, V-sign erythema on the chest, flagellate erythema on the back, nail fold bleeding and facial erythema. Serum levels of anti-TIF-1-γ/α Ab were positive as assessed by immunoprecipitation assay. He also had bilateral swelling of the parotid gland, and an excised specimen of the lung showed inflammatory pseudotumor with IgG4-positive plasma cells. As far as we know, this case is the first to report the association of IgG4-related disease and TIF-1-γ/α-positive dermatomyositis. Further accumulation of such cases is required to elucidate the mechanism of this association.

Seudotumor inflamatorio en paciente pediátrico / Inflammatory pseudotumor in a five-year-old girl

El seudotumor inflamatorio es la masa pulmonar primaria más frecuente en niños, simulando en muchos casos una neumonía organizada desde el punto de vista de la imagen. Otra localización común de este proceso patológico es la órbita, aunque puede asentar en cualquier parte del cuerpo. Se trata de una lesión poco común y cuasi neoplásica, ya que radiológica y clínicamente se comporta como un tumor maligno. La patogenia, su historia natural, los hallazgos por imagen y las opciones de tratamiento todavía se discuten (AU)

Inflammatory pseudotumor is the most common primary lung mass in children. In many cases, it mimics organizing pneumonia on imaging tests. Another site often affected by inflammatory pseudotumors is the orbit, although they can be found in any part of the body. Inflammatory pseudotumors are rare and quasi-neoplastic, as radiologically and clinically they behave like malignant tumors. Consensus about their pathogenesis, natural history, imaging findings, and treatment options has yet to be reached (AU)

Postradiofrequency ablation inflammatory pseudotumor associated with pulmonary venoocclusive disease: case report and review of the literature.

Radiofrequency ablation of pulmonary veins is a common therapeutic intervention for atrial fibrillation. Pulmonary vein stenosis and venoocclusive disease are recognized complications, but the spectrum of pathologies postablation have not been previously reviewed. A recent case at our hospital showed a left hilar soft tissue mass in association with superior pulmonary vein stenosis in a patient 4 years postablation. On resection, this proved to be an inflammatory pseudotumor composed of myofibroblasts in an organizing pneumonia-type pattern with adjacent dendriform ossifications. Pulmonary venoocclusive change was a prominent feature. Literature on the histopathology of postradiofrequency ablation complications is limited. The severity of vascular pathology appears to increase with the postablation interval. Although pulmonary vascular changes are the most common late finding, fibroinflammatory changes including pulmonary pseudotumor formation, attributable to thermal injury, should be considered in the differential diagnosis of these cases.

Granuloma pulmonar hialinizante / Pulmonary Hyalinizing Granuloma

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A rare tumor of the lung: inflammatory myofibroblastic tumor.

Inflammatory myofibroblastic tumor is a rare benign lesion whose tumor origin is now proven. It represents 0.7% of all lung tumors. We report the case of a three-year-old child who suffered from a chronic cough with recurrent respiratory infections. Chest X-ray and computed tomography revealed the presence of a left lower lobe lung mass. After pneumonectomy, histological examination combined with immunohistochemical study discovered an inflammatory myofibroblastic tumor.The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/8722069326962972.

Inflammatory pseudotumor arising from the right ventricular outflow tract causing pulmonary stenosis.

Inflammatory pseudotumor (also known as inflammatory myofibroblastic tumor) is an uncommon spindle cell lesion that was initially recognized in the lung and is now known to occur in virtually every major organ of the body. We report a case of a seven-year-old male who had an inflammatory pseudotumor of the right ventricular outflow tract involving the pulmonary valve causing pulmonary stenosis.

Un caso excepcional de seudotumor inflamatorio calcificado transmural cardiaco / An unusual case of transmural calcifying inflammatory pseudotumor of the heart

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Inflammatory pseudotumor suspected of lung cancer treated by thoracoscopic resection.

We report a relatively rare surgical treatment for two cases of inflammatory pseudotumors of the lung. In case 1, a 52-year-old male with a history of left chest pain was admitted to our hospital for an abnormal nodule with an irregular margin that was detected in the left upper lung field. The nodule, measuring 15 mm in diameter, was larger than the one observed six months earlier, which had been removed by a thoracoscopic resection. In case 2, a 64-year-old female with a history of chronic cough and hemoptysis was admitted to our hospital, and an abnormal nodule with pleural indentation was detected in the lower left lung field. The nodule, measuring 8 mm in diameter, was also removed by a thoracoscopic resection. In both cases, the histologic examination enabled us to diagnose the lesion as an inflammatory pseudotumor. In general, it is very difficult to differentiate inflammatory pseudotumors from malignant tumors of the lung. The best treatment for inflammatory pseudotumors is usually early and complete surgical resection, since it can lead to improved survival. Therefore, we consider thoracoscopy-aided surgery to be less invasive and more useful than other surgical methods in the diagnosis and treatment of inflammatory pseudotumor of the lung.

Immunoglobulin G4-related inflammatory pseudotumor of the lung.

An 82-year-old man presented with a nodule in the right S(2)a of the lung as seen by chest computed tomography (CT). He had undergone treatment for chronic obstructive lung disease. He had a 53-year history of smoking 20 cigarettes a day. Subsequent to the appearance of the nodule in the right S(2)a, the CT images revealed consolidations in the right S(2)b, right S(3), and left S(5). The nodule in the right S(2)a was diagnosed as squamous cell carcinoma after performing video-assisted thoracoscopic wedge resection of the lung. After 4 months, the size of the consolidation in the right S(2)b increased. Recurrence of lung cancer was suspected. Using transbronchial lung biopsy, the consolidation in the left S(5) was diagnosed as organizing pneumonia; therefore, right upper lobectomy was performed. The consolidations in the right S(2)b and right S(3) were diagnosed as inflammatory pseudotumors with infiltrations of immunoglobulin G4-positive plasma cells.