Pericardial immunoglobulin G4-related inflammatory pseudotumor after right upper lobectomy for lung cancer.

A 75-year-old woman underwent thoracoscopic right upper lobectomy for lung cancer. A histopathological examination showed adenocarcinoma, pT1aN0M0 stage IA1. At six months after surgery, chest computed tomography (CT) revealed pericardial nodules that had not been detected before pulmonary resection. Postoperative CT performed two months later revealed that the nodules were growing and F18 fluorodeoxyglucose-positron emission tomography showed a maximum standardized uptake of 9.87. Blood tests revealed no elevated tumor markers, with the exception of a mildly elevated interleukin-2. Based on the above results, thoracoscopic biopsy was performed due to the suspected recurrence of lung cancer or malignant lymphoma. The histopathological examination of the nodule revealed immunoglobulin G4 (IgG4)-related inflammatory pseudotumor. The serum IgG4 levels were elevated (358 mg/dL, normal: 4.5-117.0 mg/dL). No additional treatment was required because all nodules were observed to have disappeared naturally on a follow-up CT scan performed two months after the surgical biopsy. The patient has been followed-up for two years without recurrence. KEY POINTS: SIGNIFICANT FINDINGS OF THE STUDY: We report a case of pericardial immunoglobulin G4-related inflammatory pseudotumor that appeared after right upper lobectomy for lung cancer, and which naturally disappeared without any treatment. WHAT THIS STUDY ADDS: There was an immunoglobulin G4-related inflammatory pseudotumor which appeared as multiple nodules in the pericardial space, and this should be kept in mind when considering the differential diagnosis of intrapericardial nodules.

Endobronchial Ultrasound-Guided Radiofrequency Ablation of Lung Tumors and Mediastinal Lymph Nodes: A Preclinical Study in Animal Lung Tumor and Mediastinal Adenopathy Models.

Radiofrequency ablation (RFA) can be a therapeutic option in medically inoperable lung cancer patients. In this study, we evaluated a prototype bipolar RFA device applicator that can be deployed from a standard endobronchial ultrasound (EBUS) bronchoscope to determine feasibility and histopathological analysis in animal models. Rabbit lung cancers were created by transbronchial injection of VX2 rabbit cancer cells. Once the tumors were developed, they were ablated transpleurally, under EBUS guidance using the prototype RFA device. The animals were then sacrificed for specimen resection. Pig inflammatory lung pseudo-tumors and lymphadenopathy were created by transbronchial injection of a talc paste and ablated transbronchially under EBUS guidance. Pigs were evaluated at 5 days, 2 weeks, and 4 weeks following ablation by bronchoscopy and cone beam computed tomography before necropsy. Nicotinamide adenine dinucleotide hydrogen diaphorase staining was employed to measure the ablation area. Twenty-four VX2 rabbit tumors were ablated. The total ablated area ranged from 0.6 to 3.0 cm2 (mean: 1.8 cm2), corresponding to a total energy range of 1 to 6 kJ. Six pig lung pseudo-tumors and 5 mediastinal lymph nodes were ablated. Adjacent airway ulceration was observed in 3 ablations of lymph nodes. These airway complications resolved within 4 weeks of RFA without any treatment. There was no hemoptysis, air embolism, respiratory distress, or other serious complication noted. In these 2 animal models, we provide evidence that EBUS-guided bipolar RFA is feasible and histopathology shows that can ablate lung tumors and mediastinal lymph nodes under real-time ultrasound guidance.

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.

Clinical and Histopathologic Correlates and Management Strategies for Inflammatory Myofibroblastic tumor of the lung. A case series and review of the literature.

Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm that may arise in soft tissues of nearly every organ. Although IMTs are the most common lung tumors in pediatric populations, these tumors are extremely rare in adults, constituting less than 1% of adult lung tumors. IMTs are characterized by proliferating spindle cells with variable inflammatory component. The biological behavior of lung IMTs in adults is highly unpredictable, which confounds diagnosis and treatment. We retrospectively investigated patients with pulmonary lesions and the histopathologic diagnosis of inflammatory myofibroblastic tumor or its synonymous names (Plasma Cell Granuloma, xanthogranuloma, inflammatory pseudotumor, fibroxanthoma, and fibrous histiocytoma) at the MD Anderson Cancer Institute in the period between August 2000 and August 2016. We describe 7 adult cases of IMT of the lung that were diagnosed at MD Anderson Cancer Center. These cases highlight the tumor's variability in terms of clinical presentation, histopathology, and biologic behavior, and underscore the challenges in the management of these rare lung neoplasms.

Pulmonary inflammatory myofibroblastic tumor: a case report.

Pulmonary inflammatory myofibroblastic tumor is a rare disease. Computed tomography in a 54-year-old woman showed a mass compressing the segmental bronchus of the left lung. Bronchoscopy indicated a mesenchymal tumor, hamartoma, or inflammatory myofibroblastic tumor. Because an inflammatory myofibroblastic tumor can potentially clog the segmental bronchus, we performed a left upper division segmentectomy. Pathologic examination revealed an inflammatory myofibroblastic tumor. In most cases, preoperative diagnosis is difficult because of the varied radiologic manifestations, and it can also be difficult to distinguish from malignancy in small tissue samples.

Complete resection of a huge hypervascular inflammatory myofibroblastic tumor in right hemithorax after embolization.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is a rare and mostly benign tumor that has the possibility of malignant change. METHODS: Radiological findings revealed a huge mass that filled most of the right hemithorax of a 17-monthold female infant. Tumor extirpation was stopped due to massive bleeding and limited exposure of the tumor. Embolization was conducted to obstruct the arteries feeding the mass. Complete resection was performed. RESULTS: Histopathologic examination led to the diagnosis of IMT. Postoperative recovery was uneventful. CONCLUSION: Hypervascularity of IMT should be considered. Preoperative embolization can be effective to reduce intraoperative blood loss and facilitate the surgical procedure.

Inflammatory Myofibroblastic Tumor of the Lung.

Inflammatory myofibroblastic tumor of the lung is a rare condition, with a reported incidence between 0.04 - 1.2% of all tumors of the lung. We present a case of inflammatory myofibroblastic tumor of the lung. A61-year man presented to the outpatient department complaining of cough and blood-streaked sputum for 5 days. The computed tomography scan of the chest demonstrated a 4.5 x 4 cm, calcified pulmonary mass in the anterior segment of the right upper lobe. Bronchoscophy and computed tomography-guided transthoracic fine needle aspiration was inconclusive. The tumor was removed via wedge resection. Histological and immunohistochemical findings were consistent with inflammatory myofibroblastic tumor of the lung.

'Inflammatory myofibroblastic tumour'-like dedifferentiation of anaplastic lymphoma kinase-rearranged lung adenocarcinoma.

AIMS: Anaplastic lymphoma kinase (ALK) functions as an oncogenic driver in a subset of haematopoietic, epithelial and mesenchymal neoplasms. Activation of ALK most commonly occurs through gene fusion events, the presence of which predicts response to ALK-targeted inhibitors in some tumour types. Echinoderm microtubule-associated protein-like 4 (EML4)-ALK fusions represent the majority of ALK rearrangements in lung adenocarcinomas and were, until recently, thought to be exclusive to that tumour type. However, recent work has identified EML4-ALK fusions in ~20% of inflammatory myofibroblastic tumours (IMTs), particularly in those arising in the lung. Here, we present a patient with an ALK-rearranged poorly differentiated lung adenocarcinoma with a predominant sarcomatoid component that was morphologically indistinguishable from IMT. METHODS AND RESULTS: Targeted next-generation sequencing revealed EML4-ALK rearrangements in both components, with identical fusion sequences. Copy number analysis demonstrated focal gain of the MYC gene in the IMT-like component. The findings support a diagnosis of ALK-rearranged lung adenocarcinoma with IMT-like dedifferentiation. CONCLUSIONS: Our findings suggest that ALK-driven epithelial and mesenchymal neoplasms exist on a morphological spectrum, and emphasize the need to consider translocation testing in pulmonary tumours with unusual sarcomatoid morphology.

[Case of pulmonary inflammatory pseudotumor with cysts].

We herein report a case involving a 58-year-old female patient with multiple cystic lesions in the right lobe of the lung. The lesions were revealed on chest computed tomography in 2002 and followed up. Transbronchial lung biopsy showed no malignancy in June 2013. The lesions gradually increased in size and thickness and were associated with fluid-filled cysts. We performed a right lower lobectomy in November 2013. Pathological examination revealed inflammatory pseudotumor. Such a case of inflammatory pseudotumor presenting as a pulmonary cyst has not been previously described. Intractable infection and inflammation are regarded as common causes of inflammatory pseudotumor. This condition should be considered in patients with a medical history consistent with infectious disease and a pulmonary cyst found on chest computed tomography.

[A rare tumor of the lung in childhood--inflammatory myofibroblastic tumor]. / Rijetki tumor pluca u djecjoj dobi --inflamatorni miofibroblasticni tumor.

Inflammatory myofibroblastic tumor (IMT) is a rare benign neoplasm, mainly involving the lungs of the children. It represents 0.7% of all lung tumors. It was first described by Brunn in 1939.2 Diagnosis is very difficult and often only possible after resection of the tumor. We would like to present a case of pulmonary IMT in a 13-year-old girl who presented with symptoms like cough, shortness of breath, and chest discomfort. Chest X ray and computed tomography revealed the presence of a right lower lobe lung mass. Its clinical and radiological findings were diverse and non specific. The mass was removed in toto, histopathology confirmed the inflammatory myofibroblastic tumor of the lung. Intraoperative and postoperative courses were uneventful. The patient has been without any signs of relapse 2 years after the surgery.

[Pseudotumor phenotype of sarcoidosis: about two cases]. / Phénotype pseudotumoral de la sarcoïdose: à propos de deux cas.

INTRODUCTION: Sarcoidosis is a multisystem benign granulomatous disease of unknown etiology. It can sometimes cause diagnostic confusion by presenting in the form of a pseudotumor, thus constituting a trap for the unwary. COMMENTS: The authors report two cases of pseudotumor sarcoidosis. In the first, the pseudotumor occurred in the context of multisystem disease in a 48-year-old man. The response to treatment with systemic corticosteroids was complicated by the development of disseminated tuberculosis, which was rapidly fatal. The second case, by contrast, was about a 58-year-old woman and the disease was self-limiting, resolving spontaneously in less than 3 months. CONCLUSION: Through these two cases, the authors focus on the particularities of the pseudotumor manifestation of pulmonary sarcoidosis. The diagnosis is often difficult. The mismatch between the clinical picture and the extent of radiological lesions should, however, suggest the diagnosis. Histological evidence is needed to eliminate other etiologies including malignant tumors. The condition usually resolves either spontaneously or after treatment with systemic corticosteroids. However, relapses are possible, including on discontinuation of corticosteroid therapy.

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.

Spontaneous resolution of an inflammatory pseudotumour of the lung subsequent to wedge biopsy.

Spontaneous, complete resolution of inflammatory pseudotumour (IPT) of lungs is exceptionally rare. A 44-year-old male was referred for evaluation for «non resolving pneumonitis¼. He had cough and minimal expectoration for 5 months, chest pain, haemoptysis and fever for a fortnight. Computed tomography of thorax (CT-thorax) confirmed the presence of a homogenous mass with irregular borders in right middle lobe with areas of breakdown and air bronchogram. Transbronchial lung biopsy was suggestive of «plasma cell granuloma¼. Thoracotomy disclosed a hard mass in right middle lobe adherent to lower lobe, chest wall and mediastinum which could not be removed. A wedge biopsy confirmed IPT. Chest radiograph after 4 weeks revealed significant resolution. CT-thorax a year later showed fibrotic scar. Till date, there are only five reports documenting 6 patients with spontaneous resolution of IPT of lungs and in 4 this occurred within 3 months of an invasive diagnostic intervention.

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 myofibroblastic tumor of the lung: a benign lesion with aggressive behavior.

Inflammatory myofibroblastic tumor is a rare solid tumor that most often affects children and young adults. They present as myofibroblastic cell proliferations accompanied by inflammatory cells made up mostly of plasma cells. Although benign, the tumor may be very aggressive locally. In this report we describe a 22-year-old woman with primary invasive myofibroblastic tumor of the left lower lobe leading to a left pneumonectomy.

A case of endobronchial inflammatory pseudotumor invading the mediastinum.

A 50-year-old-male was admitted to our hospital in March 2007, complaining of cough and hemoptysis for 3 months. Postero-anterior chest X-ray showed an opacity on right upper zone. Computed tomography of the thorax showed a mass lesion occupying the right upper lobe and superior segment of the lower lobe and invading the mediastinum. Fiberoptic bronchoscopy showed total occlusion of the right upper lobe bronchus by the mass and infiltration of the bronchus intermedius. Bronchoscopic biopsies were nondiagnostic. PET-CT revealed SUVmax of 18.8. Right thoracotomy was performed. Vena cava superior and right pulmonary artery was invaded by the mass. Biopsies were performed. Histopathologic examination demonstrated an inflammatory pseudotumor. Corticosteroid treatment was started. The tumor was clinically and radiologically unresponsive to corticosteroids. He was referred to oncology department for radiotherapy. The patient died on November 2007.