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.

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.

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.

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.

[Surgical Treatment of Pseudotumours of the Lung]. / Chirurgische Therapie von Pseudotumoren der Lunge.

INTRODUCTION: Pseudotumour of the lung is a collective term for various subentities. Some subgroups are considered to be intermediary malignant tumours. A pseudotumour is a rare condition, which makes it difficult to estimate its incidence and prevalence. METHODS: Retrospective analysis of all surgically treated patients between 2008 and 2015 diagnosed with a pseudotumour of the lung. The primary endpoint of this study was to estimate the rates of local recurrence and metastasis. Secondary endpoints were to determine the nomenclature, medical history, treatment, and the perioperative course. RESULTS: Out of 27 patients (10 females and 17 males) with a median age of 58 years, 19 patients (70%) had an inflammatory pseudotumour (IPT), and four patients (15%) had an inflammatory myofibroblastoma (IMT). Two patients had a pneumocytoma/histiocytoma. A preoperative pulmonary infection was present in 12 (44%) patients. The average tumour size was 2.1 cm (0.8 - 5.3 cm), with the lower pulmonary lobes being mostly affected (52%). One enucleation, 20 atypical wedge resections and six anatomical resections were performed. This was done in a minimally invasive procedure (VATS) in 48% of cases (13/27). R0 resection was achieved in 93% of cases (25/27). Complications occurred in seven (26%) patients. The difference between the duration of hospital stay (mean duration 8 days) after open resection and VATS was minimal (8.8 vs. 7.2 days). Patients were followed up over a period of 4 years, during which time only one patient developed a tumour recurrence, which led to the patient's death, although she had had a R0 resection of an IMT. CONCLUSIONS: The treatment of choice for pseudotumours of the lung is R0 resection, preferably with VATS. Most patients have a benign course of disease, although relapses are possible in some cases, especially in IMT. Follow-up monitoring is necessary for IMT. The application of a uniform nomenclature and classification would be a sensible approach.

Successful sleeve lobectomy of pediatric inflammatory myofibroblastic tumor.

Inflammatory myofibroblastic tumor (IMT) is an uncommon solid tumor that was originally described in the lung. A 4-year-old girl was admitted to hospital with urticarial rash. On chest radiographs, an opacity was seen in the inferior zone of the left lung, and computed tomography showed a mass in the left lower lobe. Left lower sleeve lobectomy was performed, and the diagnosis was confirmed as IMT. Sleeve resection is the best option in lesions located in the mainstem bronchus or secondary carina. Herein, we present a rare case of IMT of the lung that was successfully treated with sleeve lobectomy. There have been fewer than 15 childhood cases of IMT reported in the literature, and the present 4-year-old patient is one of the youngest.

Sindrome de Blesovsky. A propósito de un caso / Anatomical and clinical implications of the humeral torsion

Introducción: La atelectasia redonda de pulmón, conocida como síndrome de Blesovsky, constituye un pseudotumor pulmonar de ubicación periférica asociado a la exposición al asbesto y al engrosamiento pleural. Los hallazgos imagenológicos pueden establecer el diagnóstico,pero no puede descartarse algún proceso neoplásico maligno. La precisión en el diagnóstico puede dificultarse por causas como la progresión radiológica y empeoramiento clínico del paciente. Objetivo: Presentación de caso clínico y revisión bibliográfica. Caso clínico: Paciente de 76 años, masculino, que consulta por síndrome de impregnación, pérdida de peso y anemia de 2 meses de evolución. Estudios imagenológicos muestran imagen nodular de 20 mm en lóbulo inferior derecho. Exploración videotoracoscópica: engrosamiento pleural de aspecto benigno (fibrosis) sin palparse nódulo pulmonar. TAC control a los 3 meses: aumento de la imagen nodular (30 mm) Se decide reexploración y resección pulmonar. Pieza de patología: Atelectasia redonda. Discusión: La atelectasia redonda es una entidad benigna, que como tal tiende a ser estable y en algunos casos evoluciona a la resolución espontanea. Aunque la seguridad diagnóstica mediante la Tac es alta, no existe un patrón individual que permita diferenciarla de un proceso maligno. Precisa de un seguimiento clínico estrecho y de la realización de pruebas diagnósticas invasivas si persisten las dudas y/o se detecta un crecimiento de la lesión. Se acepta como conducta diagnóstica y/o terapéutica la resección pulmonar lobar o sublobar.

Introduction: Rounded atelectasis lung, known as Blesovsky syndrome, is a pulmonary pseudotumor of peripheral location associated with asbestos exposure and pleural thickening. Image findings may establish the diagnosis but a malignant neoplastic process can not be ruled out. The accuracy of the diagnosis canbe difficult for reasons such as radiological progression and clinical worsening of the patient.Objective: Clinical case presentation and literature review.Case Report: 76 years old male patient, who consults forimpregnation syndrome, weight loss and anemia 2 months ofevolution. Imaging studies show 20 mm nodular image in the rightlower lobe. Laparoscopic exploration: pleural thickening withbenign aspect (fibrosis) without palpable image. CT 3 monthslater: Increased nodular image (30 mm) It decides relaparoscopyand pulmonary resection. Piece lobectomy: rounded atelectasis.Discusion: Rounded atelectasis is a benign entity, and as suchtends to be stable and in some cases resolve spontaneously.Although security diagnosed by tomography is high, there is nosingle pattern that allows to differentiate from malignancy.Requires close clinical monitoring and invasive diagnostic tests ifdoubts persist and/or injury growth is detected. Lobar or sublobarlung resection is accepted as diagnostic and therapeutic conduct.

Imaging of childhood inflammatory myofibroblastic tumor.

BACKGROUND: Inflammatory myofibroblastic tumor is a rare benign neoplasm and most commonly involves the lung but occurs in extrapulmonary locations. OBJECTIVE: To present imaging findings in inflammatory myofibroblastic tumors in children based on a single-centre experience. MATERIALS AND METHODS: We retrospectively reviewed CT and MRI findings of children diagnosed with inflammatory myofibroblastic tumor in a single institution. RESULTS: We identified 15 children (range: 1-17 years) with inflammatory myofibroblastic tumor. The tumor was localized to the lung (n = 5), mediastinum (n = 3), trachea (n = 1), bronchus (n = 1), abdomen (n = 2) and orbit (n = 3). All the extraorbital tumors were solid masses with homogeneous or heterogeneous enhancement. Four lung tumors and one posterior mediastinal tumor contained calcification. Local recurrence following surgical removal occurred in two children with invasion of the esophagus and of the left atrium in one. Localized masses were seen in all children with orbital tumour. Two of these had episcleritis and perineuritis; one had episcleritis, tendonitis, perineuritis, myositis and dacryoadenitis. CONCLUSION: The locations and imaging features of inflammatory myofibroblastic tumors are variable.

Inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT): a representative literature review occasioned by a rare IMT of the transverse colon in a 9-year-old child.

AIMS AND BACKGROUND: Inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT) represent different entities. However, it is only in recent years that this has been taken into increasing consideration. Some authors still use both terms synonymously or interchangeably. Inflammatory myofibroblastic tumor is a real neoplasm because of the proliferation of myofibroblastic cells. Inflammatory pseudotumor is a more inflammatory reactive or regenerative entity and shows an overlapping with immunoglobulin G4-related disease. METHODS AND STUDY DESIGN: To analyze the current situation, 443 publications from the last 5 years (2009 to February 2014) were included. Reports involved 938 patients and 956 organ sites. The age distribution is twin peaked with one maximum in childhood and the other between 50 and 60 years of age. This distribution is questionable due to the more frequent occurrence of IPT in the liver and of IMT in the lung. Inflammatory pseudotumors mainly occur in older patients; IMTs in children and young adults. RESULTS AND CONCLUSIONS: The liver and biliary tract were the most commonly affected of all body regions, at 32%. This was followed by the lung, including the respiratory tract, at 27%, and by the gastrointestinal tract, at 10%. Lesions of the large bowel, as in the present case of a 9-year-old boy, are very rare. There were organ-related as well as nonspecific clinical symptoms, such as fever, weight loss, and fatigue. Laboratory test results revealed anemia and elevated inflammation-dependent parameters. The patterns in medical imaging are variable and nonspecific. Morphology often suggests a malignant process. For this reason, therapy in most cases is surgical, but this is required more often in IMTs. Many IPTs could be treated conservatively.

Synchronous posttransplant lymphoproliferative disorder and inflammatory myofibroblastic tumor of the lung in a 2-year-old liver transplanted boy: a case report.

Inflammatory myofibroblastic tumor is a rare benign lesion found mostly in children and young adults. It originates from the lung, abdominopelvic region, and retroperitoneum. The tumor is composed of myofibroblasts and inflammatory cell infiltration in the tissue. The cause and pathogenesis of this tumor are not completely understood. Epstein-Barr virus has been held responsible in some reported cases of inflammatory myofibroblastic tumors. Another tumor (which is known to be related to the Epstein-Barr virus) is posttransplant lymphoproliferative disorder. We report the case of a 2-year-old boy who underwent a liver transplant at the age of 9 months (the donor was his mother) because of biliary atresia. At 11 months after transplant, we detected posttransplant lymphoproliferative disorder and inflammatory myofibroblastic tumor concurrently. This entity is presented to highlight possible Epstein-Barr virus involvement in inflammatory myofibroblastic tumor of lung.

Pulmonary inflammatory myofibroblastic tumour after Hodgkin's lymphoma.

Positron emission tomography (PET)/CT plays a major role in staging, assessing response to treatment and during follow-up of paediatric Hodgkin's lymphoma (HL). Owing to high sensitivity to detect viable tumoural tissue, negative PET/CT is highly predictive of survival. However, (18)F-FDG is not specific for malignant disease and may concentrate in numerous benign/inflammatory lesions that may cause 'false-positive' results and follow-up PET/CT studies should be interpreted with caution. We report a case of pulmonary inflammatory myofibroblastic tumour, which developed during follow-up in a young patient with complete remission of a stage IIB HL and was fully treated with surgical resection.

Pulmonary inflammatory myofibroblastic tumor and IgG4-related inflammatory pseudotumor: a diagnostic dilemma.

IgG4-related inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT) share morphological features like a prominent fibroblastic/myofibroblastic proliferation and the presence of inflammatory cells. Since IPT is managed conservatively and IMT is treated by surgical excision, it is important to differentiate these two lesions. The aim of this study is to highlight morphological and immunohistochemical features that distinguish IPT and IMT. Clinicopathological characteristics of cases diagnosed as pulmonary IPT or IMT from 1997 to 2013 were reviewed. The histological features were studied on hematoxylin and eosin-stained sections. Immunohistochemistry was done for IgG, IgG4, ALK-1, SMA, desmin, and CD34 for classification into IPT and IMT. Of the ten patients, seven were male and the age ranged from 4 to 58 years. The tumor size ranged from 1.5 to 4.0 cm in diameter. Histologically, proliferation of bland-looking spindle cells along with fibrosis and an inflammatory infiltrate comprising of lymphocytes and plasma cells were the common morphological features of both lesions. The spindle cell proliferation was more marked in IMT whereas lymphoplasmacytic infiltrate was more prominent in IPT. Obstructive phlebitis was observed only in cases of IPT. IgG4 expression was noted in IPT, and the number of IgG4-positive plasma cells and the ratio of IgG4+/IgG+ plasma cells were significantly lower in IMT than in IgG4-related IPT. Expression of anaplastic lymphoma kinase (ALK) was observed only in IMT, but not in IgG4-related IPT. The proportion of proliferating spindle cells, lymphoplasmacytic infiltrate, obstructive phlebitis, IgG4+ plasma cells and the ratio of IgG4+/IgG+ plasma cells, and ALK expression are helpful in differentiating these morphologically similar but biologically different lesions, which require different treatment modalities.

A rare case of lung tumor -- pulmonary inflammatory pseudotumor.

Pulmonary inflammatory pseudotumor (PIP) is a rare condition of unknown etiology. It is still a matter of debate if it represents an inflammatory lesion characterized by uncontrolled cell growth or a true neoplasm. Although mostly benign, these tumors are diagnosis and therapeutic challenges. Preoperative diagnosis can rarely be established. The treatment of choice is surgical resection which has both diagnostic and therapeutic value. We report the case of a 63-year-old male presented with clinical and imagistic picture suggestive of malignancy in the thorax. Lobectomy was performed with histological diagnosis of PIP. No evidence of tumor recurrence.

Pulmonary inflammatory pseudotumor observed by bronchoscopy and resected using video-assisted thoracic surgery.

Pulmonary inflammatory pseudotumor is rare. A 34-year-old woman visited our hospital due to an abnormal chest shadow. Computed tomograhy showed a nodule in the right upper lobe. Bronchoscopy showed a polypoid endobronchial nodule obstructing most of the orifice of B2a. The nodule was white, glossy, and smooth, and it seemed to be covered with bronchial mucosa. However, transbronchial biopsy could not facilitate a diagnosis. To obtain a definitive diagnosis, we performed lobectomy of the right upper lobe using video-assisted thoracic surgery and removed the nodule completely. The pathologic diagnosis made during surgery was inflammatory pseudotumor. Immunohistochemical examination showed proliferating spindle cells were positive for vimentin and smooth muscle actin, but negative for epithelial markers. These findings were consistent with the staining pattern of inflammatory pseudotumor previously reported. Careful follow-up is necessary to detect any sign of local recurrence and distant metastases.