Molecular Characterization of Inflammatory Tumors Facilitates Initiation of Effective Therapy.

Inflammatory myofibroblastic tumor (IMT) is a rare, mesenchymal tumor that has an increased incidence in childhood. Tumors are usually isolated to the chest, abdomen, and retroperitoneum, but metastatic presentations can be seen. Presenting symptoms are nonspecific and include fever, weight loss, pain, shortness of breath, and cough. Approximately 85% of IMTs harbor actionable kinase fusions. The diagnosis can be delayed because of overlapping features with inflammatory disorders, such as elevated inflammatory markers, increased immunoglobin G levels, fever, weight loss, and morphologic similarity with nonmalignant conditions. We present a girl aged 11 years with a TFG-ROS1 fusion-positive tumor of the lung that was initially diagnosed as an immunoglobin G4-related inflammatory pseudotumor. She underwent complete left-sided pneumonectomy and later recurred with widely metastatic disease. We then report the case of a boy aged 9 years with widely metastatic TFG-ROS1 fusion-positive IMT with rapid molecular diagnosis. In both children, there was an excellent response to oral targeted therapy. These cases reveal that rapid molecular testing of inflammatory tumors is not only important for diagnosis but also reveals therapeutic opportunities. Targeted inhibitors produce significant radiologic responses, enabling potentially curative treatment approaches for metastatic ROS1 fusion IMT with previously limited treatment options. Primary care pediatricians and pediatric subspecialists have a crucial role in the early consultation of a pediatric oncology center experienced in molecular diagnostics to facilitate a comprehensive evaluation for children with inflammatory tumors.

A rare cause of chronic dysphagia: pulmonary inflammatory myofibroblastic tumor with distal esophagus invasion.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is rare intermediate tumor, which happens mostly in children and young adults. CASE PRESENTATION: Reported is the successful treatment of a 29-year-old man presented with progressively dysphagia and weight loss. No other abnormal symptoms were observed. The contrast enhanced computed tomography (CT) revealed a dumbbell-shaped lesion between lung and esophagus. Finally, it was pathologically diagnosed as pulmonary IMT invading to the distal esophagus after operation. The patient underwent partial esophagectomy and left lower lobectomy, and was discharged on 10th postoperative day. CONCLUSIONS: IMT is a rare lesion that usually occurs in the lung, but pulmonary IMT with distal esophagus invasion has not been described previously. Discriminating untypical symptom, completed resection, pathological expertise and closed follow-up will reach the successful diagnosis and treatment.

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.

Syphilitic Pulmonary Inflammatory Pseudotumor: A Diagnostic Challenge.

A 46-year-old man presented with nonproductive cough and lower limb swelling. Chest radiograph showed a left lower lobe lung mass and multiple subpleural nodules. Other investigations revealed that he had nephrotic syndrome. Core biopsies of the left lower lobe lung mass showed features of inflammatory pseudotumor with endarteritis obliterans and a lymphoplasmacytic infiltrate. Immunohistochemical stain for Treponema pallidum was positive. Resolution of the lung mass and nephrotic syndrome was achieved after treatment with intramuscular benzathine benzylpenicillin. The differential diagnosis of pulmonary inflammatory pseudotumor, manifestations of pulmonary syphilis, and a literature review of secondary syphilis of the lung are discussed.

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.

Pseudotumor bronquial eosinofílico / Eosinophilic Bronchial Pseudotumor

Las eosinofilias pulmonares constituyen un grupo variado de entidades cuyo nexo común es la inflamación eosinofílica que puede o no asociarse a eosinofilia periférica. En ese reporte describimos una forma atípica y no descripta de presentación como "pseudotumor pulmonar" que remitió con tratamiento corticoesteroideo.

Pulmonary eosinophilia are a varied group of entities sharing the eosinophilic inflammation that may or may not be associated with peripheral eosinophilia. This report describes an atypical, undescribed presentation, the "pulmonary pseudotumor" which showed regression with corticosteroid treatment.

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.

Using CT texture analysis to differentiate between peripheral lung cancer and pulmonary inflammatory pseudotumor.

BACKGROUND: This study is to distinguish peripheral lung cancer and pulmonary inflammatory pseudotumor using CT-radiomics features extracted from PET/CT images. METHODS: In this study, the standard 18F-fluorodeoxyglucose positron emission tomography/ computed tomography (18 F-FDG PET/CT) images of 21 patients with pulmonary inflammatory pseudotumor (PIPT) and 21 patients with peripheral lung cancer were retrospectively collected. The dataset was used to extract CT-radiomics features from regions of interest (ROI), The intra-class correlation coefficient (ICC) was used to screen the robust feature from all the radiomic features. Using, then, statistical methods to screen CT-radiomics features, which could distinguish peripheral lung cancer and PIPT. And the ability of radiomics features distinguished peripheral lung cancer and PIPT was estimated by receiver operating characteristic (ROC) curve and compared by the Delong test. RESULTS: A total of 435 radiomics features were extracted, of which 361 features showed relatively good repeatability (ICC ≥ 0.6). 20 features showed the ability to distinguish peripheral lung cancer from PIPT. these features were seen in 14 of 330 Gray-Level Co-occurrence Matrix features, 1 of 49 Intensity Histogram features, 5 of 18 Shape features. The area under the curves (AUC) of these features were 0.731 ± 0.075, 0.717, 0.748 ± 0.038, respectively. The P values of statistical differences among ROC were 0.0499 (F9, F20), 0.0472 (F10, F11) and 0.0145 (F11, Mean4). The discrimination ability of forming new features (Parent Features) after averaging the features extracted at different angles and distances was moderate compared to the previous features (Child features). CONCLUSION: Radiomics features extracted from non-contrast CT based on PET/CT images can help distinguish peripheral lung cancer and PIPT.

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.

Pulmonary Mycobacterium Spindle Cell Pseudotumor in Patient With Liver Transplant.

We report a case of liver transplant patient who presented with lung masses, found to be Mycobacterium spindle cell pseudotumors. The masses demonstrated hypermetabolic activities on positron emission tomography. Core biopsy revealed sheets of spindle histiocytic cells with abundant acid-fast bacilli identified as Mycobacterium avium-intracellulare complex. This finding is a rare presentation of Mycobacterium infection, mainly nontuberculous Mycobaterium. It is characterized by a benign, spindle cell mass-forming reaction. Most of the reported cases had acquired immune deficiency syndrome or organ transplant. Histopathology illustrating the proliferation of spindle cell shaped histiocytes containing numerous acid-fast bacilli is the gold standard for diagnosis. The standard treatment has not been well established; previously reported cases followed the standard treatment for Mycobacterium based on organ involvement. Our case is the first case to our knowledge that reports pulmonary Mycobacterium spindle cell pseudotumors in a liver transplant recipient.

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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Inflammatory Myofibroblastic Tumor After Lung Transplant-A Rare and Aggressive Complication: A Case Report.

INTRODUCTION: Malignant diseases are well-known complications after lung transplantation (LT). Among these, inflammatory myofibroblastic tumor (IMT) is a rare neoplasm with a not well-known and often aggressive biological behavior. MATERIAL AND METHODS: We hereby describe 2 cases of cystic fibrosis patients who underwent bilateral sequential LT (BSLT) complicated by IMT. RESULTS: A 26-year-old man presented a right endobronchial lesion 6 months after BSLT. Two consecutive fiber bronchoscopic biopsies showed granulation tissue. For the persistent lesion growth, the patient underwent a transthoracic biopsy showing histologic diagnosis of IMT. Therefore, he underwent to right pneumonectomy that was unfortunately complicated after 6 months with a late bronchopleural fistula and empyema with exitus 6 months later. A 31-year-old woman 1 year after BSLT presented with a left voluminous pleural-parenchymal lesion; the histologic examination after biopsy revealed an IMT. She underwent a removal of the lesion with a macroscopic R0 resection. Histologic, immunophenotypic, and cytogenetic examinations showed a strong overexpression of anaplastic lymphoma kinase requiring biological adjuvant therapies; however, the patient refused it. Four years later, she presented a recurrence treated with debulking procedure and adjuvant radiotherapy. At last follow-up, the patient was alive with stable disease and optimal graft function. CONCLUSIONS: Although IMT is a rare complication after lung transplant, to obtain a careful diagnosis, an early and aggressive treatment is mandatory.

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.

Varón con síndrome constitucional y adenopatías mediastínicas. A propósito de un caso / Patient with constitutional syndrome and mediastinal adenopathies. A case report

La sarcoidosis es una enfermedad granulomatosa sistémica de causa desconocida, caracterizada por la presencia de granulomas no caseificantes en los órganos afectados. Afecta típicamente al pulmón de adultos de mediana edad. Se basa en un diagnóstico de exclusión, ya que no existe una prueba diagnóstica definitiva. Como tratamiento de primera línea se recomiendan los corticoesteroides, teniendo como alternativas los inmunosupresores, fundamentalmente el metotrexato, y las terapias biológicas. Presentamos el caso de un paciente de 43 años sin antecedentes de interés, enviado a consultas de neumología por pérdida de peso e imágenes de agrandamiento hiliar bilateral y ensanchamiento mediastínico derecho en la radiografía de tórax

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology, characterized pathologically by the presence of noncaseating granulomas in involved organs (most frecuently the lung of middle aged adults). The most important part of the diagnosis is the exclusion of alternative possibilities, because a definitive diagnostic test for sarcoidosis does not exist. The essence of the treatment are corticosteroids. We present a 43- years-old male patient with no medical history of interest, who was sent to the pneumology outpatient department because of weight loss and bilateral hilar adenopathies and right paratracheal node enlargement on the radiography

Inflammatory Myofibroblastic Tumor Diagnosed by Transbronchial Needle Aspiration / Tumor inflamatorio miofibroblástico diagnosticado por punción aspirativa transbronquial

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