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Inflammatory pseudotumor is a disease with unsettled pathogenesis. The brain is a rare site of occurrence. The aim of this study is to investigate ALK-1 protein expression and IgG4-positive plasma cells detection in 4 intracranial inflammatory pseudotumors. Three dural-based and 1 intraventricular inflammatory pseudotumors were retrieved from the hospitals' archive. The data on clinical presentation, radiological findings, procedure undertaken, and patients' progress were collected. Sections from the excised lesions were examined under hematoxylin and eosin, histochemical, and immunohistochemical staining including ALK-1 and IgG4. All 4 cases displayed typical histological features of inflammatory pseudotumor with dense lymphoplasmacytic infiltrate admixed with small number of benign-looking spindle cells in a collagenous stroma. Three cases exhibited high density of IgG4-positive plasma cells per high-power field. ALK-1 was negative. ALK expression was not found in any of our cases. On the contrary, the detection of significant number of IgG4-positive plasma cells in 3 inflammatory pseudotumors suggests that a considerable proportion of intracranial inflammatory pseudotumor may belong to the IgG4-related subgroup. Hence, a trial of corticosteroid after histological confirmation may be valid to avoid unnecessary risk-taking neurosurgical procedures or in cases with incomplete tumor removal.
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PMID:Inflammatory pseudotumors of the central nervous system. 1965 49

Inflammatory myofibroblastic tumor is a distinctive lesion composed of myofibroblastic spindle shaped cells accompanied by inflammatory infiltrate that may arise in various organs. It is believed to be a noneoplastic inflammatory condition, although this is still controversial. The recognition of inflammatory myofibroblastic tumor as an entity is important especially to avoid unnecessary surgery. A few cases have been reported in the oral cavity. This report primarily presents a case of inflammatory myofibroblastic tumor that arose in the floor of mouth of a 23-year-old woman. The proliferating spindle cells were immunoreactive for vimentin, smooth muscle actin, and muscle specific actin and negative for desmin, AE1/AE3, S-100, CD68, MyoD1 and caldesmon. In an attempt to assess the potential neoplastic nature of this lesion, immunohistochemical expression of ALK protein was performed, although no immunoreactivity was detected. Also, the presence of well differentiated myofibroblasts identified by fibronectin is discussed, as well as the importance in establishing an immunoprofile to better consolidate the diagnosis. We conclude that the study of fibronectin in case series may aid the diagnosis as well as the prediction of the tumor course.
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PMID:Fibronectin as an adjuvant in the diagnosis of oral inflammatory myofibroblastic tumor. 1968 Jan 89

Inflammatory pseudotumor (IPT) is a heterogeneous group of lesions occurring in various organs, which is histologically characterized by fibroblastic and myofibroblastic proliferation with inflammatory infiltrate. Inflammatory myofibroblastic tumor (IMT) is a neoplastic counterpart of IPT, which shows aberrant expression of ALK and its gene translocation. In contrast, the concept "immunoglobulin (Ig)G4-related IPT" in the lung, liver, and pancreas has recently been proposed as a member of IgG4-related sclerosing disease. In this study, we compared the histopathologic features with an emphasis on IgG4 expression between 22 cases of IMT and 16 cases of IgG4-related sclerosing disease, including chronic sclerosing sialadenitis (n=8), mass-forming autoimmune pancreatitis (n=3), sclerosing cholangitis (n=1), retroperitoneal fibrosis (n=2), and chronic sclerosing dacryoadenitis (n=2). Bland-looking spindle cell proliferation with fibrosis and inflammatory infiltrate of lymphocytes and plasma cells was the common morphologic feature in both lesions. Obstructive phlebitis was observed in all of the IgG4-related sclerosing lesions, but in only 1/22 (4.5%) of IMT. The immunohistochemical expression of ALK was observed in 15/22 (68.2%) of IMT and 0/16 (0%) of IgG4-related sclerosing disease. The number of IgG4-positive plasma cells and the ratio of IgG4+/ IgG+ plasma cells were each significantly lower in IMT than in IgG4-related sclerosing disease [mean 6.4/HPF vs. 178.3/HPF (P<0.0001), 3.0% vs. 67.5% (P<0.0001), respectively]. The results suggest that IgG4 does not play an important role in the pathogenesis of IMT. In addition, the evaluation of IgG4+ plasma cells and the ratio of IgG4+/IgG+ plasma cells and the presence of obstructive phlebitis may be useful for the differential diagnosis between IMT and IgG4-related sclerosing disease.
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PMID:Inflammatory myofibroblastic tumor versus IgG4-related sclerosing disease and inflammatory pseudotumor: a comparative clinicopathologic study. 1971 89

Inflammatory myofibroblastic tumour is a distinctive mesenchymal neoplasm, composed of a variable admixture of myofibroblastic spindle-shaped and inflammatory cells which were originally described in the lung, as a nonneoplastic lesion and designated as an inflammatory pseudotumour. The lack of certainty of the IMTs pathogenesis is reflected in the large number of terms which have been attributed to this lesion. Recent genetic and molecular studies of IMTs have showed chromosomal abnormalities of 2p23 resulting in a rearrangement of the anaplasic lymphoma kinase gene and have also provided evidence for a monoclonal, noeplastic origin for IMT.Occurrence of IMT in the kidney is very rare, and to our knowledge, only 30 such cases have been described in the literature.This report describes an original case of an ALK positive IMT of the kidney associated with renal pelvic calculus which we believe has never been reported. The differential diagnosis of IMTs will also be discussed.
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PMID:Anaplasic lymphoma kinase positive inflammatory myofibroblastic tumour with renal pelvic calculus: a case report. 1991 81

Inflammatory myofibroblastic tumour (IMT) or inflammatory pseudotumour is a histologically distinctive lesion occurring primarily in the viscera and soft tissue of children and young adults. We report an unusual case of IMT which had undergone malignant transformation in the chest wall at the pacemaker site. A 64-year-old male presented with a history of high fever, loss of appetite and weight loss of three months duration. He had a dual chamber pacemaker reinserted in the left infraclavicular region in the previous year. This was followed by a gradually enlarging hard swelling at the insertion site. The CT-scan showed a soft tissue mass encasing the pacing box, without intrathoracic extension. The trucut biopsy was suspicious of soft tissue sarcoma. A well encapsulated hard mass, with pacemaker embedded within it was resected en-bloc ensuring wide resection margins. Histology revealed fascicles of spindle cell proliferation with prominent inflammatory component, occasional spindle cells with prominent nucleoli and scattered atypical mitotic figures, with areas of focal necrosis. The lesional cells were negative for CD21, smooth muscle actin, ckit, cytokeratins and anaplastic lymphoma kinase 1. A diagnosis of IMT with malignant transformation i.e. inflammatory fibrosarcoma was made. He had adjuvant radiotherapy and uneventful recovery.
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PMID:Inflammatory myofibroblastic tumour at the pacemaker site. 2004 Apr 80

We report a case of a 28-year-old man with a dry cough and chest pain. Chest X-ray film showed a huge mass in the right lung field. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a huge mass which occupied most of the right thoracic cavity and invaded the superior vena cava, heart atrium and right pulmonary vein. Positron emission tomography (PET) showed metastasis of bone, right adrenal grand and lymph nodes. A tumor specimen was biopsied percutaneously, and the diagnosis was pathologically confirmed as an inflammatory myofibroblastic tumor. Immunohistochemical staining also showed an overexpression of ALK in the tumor. He was treated with a non-steroid anti-inflammation drug and steroid, but they were ineffective. He underwent chemotherapy with bleomicin, etoposide and cisplatin. After two cycles of chemotherapy, the tumor slightly reduced in size, but was eventually refractory to the regimen finally. He also underwent with paclitaxel and carboplatin. At present, if operative extirpation is not possible, there is no way to treat an inflammatory myofibroblastic tumor. In the future, new therapy incorporating ALK inhibitors would be expected to treat those cases of IMT in which local recurrences and distant metastases occur.
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PMID:[A case of rapidly growing inflammatory myofibroblastic tumor in the lung]. 2005 97

Inflammatory myofibroblastic tumor (IMT) is a soft tissue lesion which can be found in almost all parts of the human body, but shows the highest incidence in the abdomen. It is a spindle cell lesion with prominent inflammatory infiltrate which consists predominantly of plasma cells. Using immunohistochemistry a myofibroblastic differentiation can be demonstrated. ALK is expressed in approximately 50% of tumors. The tumors are associated with a benign behavior but recurrences occur in about one third of abdominal cases. Malignant cases are rare.
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PMID:[Inflammatory myofibroblastic tumor]. 2006 99

Inflammatory myofibroblastic tumor (IMT) is an uncommon benign neoplasm with locally aggressive behavior but malignant change is rare. We report an unusual case of pelvic-abdominal inflammatory myofibroblastic tumor with malignant transformation in a 14-year-old boy presenting with abdominal pain and 9 kg body weight loss in one month. Computed tomography revealed a huge pelvi-abdominal mass (30 cm), possibly originating from the pelvic extraperitoneal space, protruding into the abdomen leading to upward displacement of the bowel loops, downward displacement of the urinary bladder, massive central necrosis, a well-enhanced peripheral solid component with prominent peritumoral vascularity. Subsequent examination confirmed the computed tomographic findings. Histopathologic examination revealed proliferative epitheloid and spindle cells, inflammatory cell infiltration and high mitotic counts. Immunohistochemistry was strongly positive for anaplastic lymphoma kinase and revealed a high proliferative index (ki-67 = 40%). DNA sequencing and electronic microscopy further confirmed the primitive fibroblastic cell phenotype of the tumor and a final diagnosis of inflammatory myofibroblastic tumor with malignant transformation was established. Rapid tumor recurrence was noted 20 d after radical tumor resection. To our knowledge, this is the largest documented case of IMT in a pediatric patient and the first report of IMT with malignant transformation originating from the pelvic extraperitoneal space.
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PMID:Huge pelvi-abdominal malignant inflammatory myofibroblastic tumor with rapid recurrence in a 14-year-old boy. 2051 95

Inflammatory myofibroblastic tumor (IMT) is an unusual tumor composed of differentiated myofibroblastic spindle cells usually accompanied by numerous plasma cells and lymphocytes. IMT was originally described in the lung; occurrence in a long bone is rare. We present two examples of IMT arising in a long bone: one in the humerus and one in the femur. In both cases, imaging shows a poorly delineated osteolytic lesion with cortical bone destruction that aggressively extends into surrounding soft tissue. Histologically, the lesion is dominated by differentiated spindle cells with aprominent collagenous stroma and an inflammatory component including plasma cells and lymphocytes, and with positive immunoreactivity for anaplastic lymphoma kinase. The absence of cytologic atypia helps differentiate this lesion from malignant spindle cell tumors.
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PMID:Inflammatory myofibroblastic tumor of bone: two cases occurring in long bone. 2065 41

Inflammatory Myofibroblastic Tumor (IMT) is a rare spindle cell neoplasm with a relatively indolent course. Its morphology may be confused with both reactive processes and/or malignant neoplasms on FNA specimens. Herein we discuss the cytologic features and IHC studies of IMT. The archives of the Department of Pathology at the Johns Hopkins Hospital were searched for IMT. A total of 257 cases were identified over a period of 11 years. Among them, 20 cases had cytology material. The patients' ages ranged from 7 to 81 years old with a median age of 54 years. The locations of the tumor in descending order were: liver (9/20, 45%), lung (8/20, 40%), abdomen (1/20, 5%), pelvis (1/20, 5%), and kidney (1/20, 5%). On FNA, the majority of tumors consisted of bland spindle cells with oval nuclei and small prominent nucleoli in a background of lymphocytes and plasma cells. Focal cytological atypia and "ganglion-like" cells were identified in 7 cases, likely related to the risk of metastases and malignant transformation. The lesional cells expressed ALK (8/17, 47.1%) and actin (10/10, 100%), but with variable expression of cytokeratin. Ki-67 showed low proliferative indices. ALK gene rearrangement was detected by FISH in three out of three cases and correlated with ALK protein expression by IHC. The cytologic diagnosis of IMT is challenging. When encountering a spindle cell lesion with prominent inflammatory component, a high index of suspicion in combination with the use of ancillary studies increases the diagnostic yield of IMT.
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PMID:Cytology of fine-needle aspiration of inflammatory myofibroblastic tumor. 2073 Aug 98


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