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Inflammatory Pseudotumor
According to the WHO classification, three lesional patterns can be observed * Inflammatory myofibroblastic tumour, that can be associated with an ALK gene rearrangement * Plasmocytic pattern (" plasma cell granuloma"), that can be linked to IgG4-related disease IgG4-related disease (IgG4-RD), formerly known as IgG4-related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, various degrees of fibrosis (scarring) and ... * Fibrous and hyalinizing pattern: Pulmonary hyalinizing granuloma References Lesion {{med-sign-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Inflammatory Myofibroblastic Tumour
Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm of the mesodermal cells that form the connective tissues which support virtually all of the organs and tissues of the body. IMT was formerly termed inflammatory pseudotumor. Currently, however, inflammatory pseudotumor designates a large and heterogeneous group of soft tissue tumors that includes inflammatory myofibroblastic tumor, plasma cell granuloma, xanthomatous pseudotumor, solitary mast cell granuloma, inflammatory fibrosarcoma, pseudosarcomatous myofibroblastic proliferation, myofibroblastoma, inflammatory myofibrohistiocytic proliferation, and other tumors that develop from connective tissue cells. Inflammatory pseudotumour is a generic term applied to various neoplastic and non-neoplastic tissue lesions which share a common microscopic appearance consisting of spindle cells and a prominent presence of the white blood cells that populate chronic or, less commonly, acute inflamed tissues. Inflammatory myofibrobl ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
ALK (gene)
Anaplastic lymphoma kinase (ALK) also known as ALK tyrosine kinase receptor or CD246 (cluster of differentiation 246) is an enzyme that in humans is encoded by the ''ALK'' gene. Identification Anaplastic lymphoma kinase (ALK) was originally discovered in 1994 in anaplastic large-cell lymphoma (ALCL) cells. ALCL is caused by a (2;5)(p23:q35) chromosomal translocation that generates the fusion protein NPM-ALK, in which the kinase domain of ALK is fused to the amino-terminal part of the nucleophosmin (NPM) protein. Dimerization of NPM constitutively activates the ALK kinase domain. The full-length protein ALK was identified in 1997 by two groups. The deduced amino acid sequences revealed that ALK was a novel receptor tyrosine kinase (RTK), having an extracellular ligand-binding domain, a transmembrane domain, and an intracellular tyrosine kinase domain. While the tyrosine kinase domain of human ALK shares a high degree of similarity with that of the insulin receptor, its extracel ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Plasma Cell Granuloma
Plasma cell granulomas (PCGs) are uncommon, non-neoplastic lesions of unknown etiology and are considered an entity of IgG4-related diseases. History/Introduction PCGs were first discovered and described in 1973 by Bahadori and Liebow. PCGs are characterized by the proliferation and infiltration of different inflammatory cells with the main cell observed in highest concentrations being plasma cells. More specifically, PCGs are a type of mass-forming lesion arising from the accumulation of polyclonal plasma cells surrounded in a swirling storiform orientation of fibrosis and spindle cell proliferation. It has been noted that these granulomas have the propensity to manifest on any organ or soft tissue. Plasma cell granulomas are generally found to be benign, but in some cases, the granulomas have the ability to initiate malignancy and become symptomatic regardless of location or size. Common sites of plasma cell granulomas are in the oral gingiva, lungs, vagina, larynx, orbit, spin ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
IgG4-related Disease
IgG4-related disease (IgG4-RD), formerly known as IgG4-related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, various degrees of fibrosis (scarring) and a usually prompt response to oral steroids. In approximately 51–70% of people with this disease, ''serum'' IgG4 concentrations are elevated during an acute phase. It is a relapsing-remitting disease associated with a tendency to mass forming, tissue-destructive lesions in multiple sites, with a characteristic histopathological appearance in whichever site is involved. Inflammation and the deposition of connective tissue in affected anatomical sites can lead to organ dysfunction, organ failure, or even death if not treated. Early detection is important to avoid organ damage and potentially serious complications. Treatment is recommended in all symptomatic cases of IgG4-RD and also in asymptomatic IgG4-RD involving certain anatomical site ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Pulmonary Hyalinizing Granuloma
Pulmonary hyalinizing granuloma is a lesional pattern of pulmonary inflammatory pseudotumor. Pathology Pulmonary hyalinizing granuloma is characterized by localized changes in lung architecture determined by deposition of hyaline collagenous fibrosis accompanied by sparse lymphocytic infiltrate that compresses and distorts the remaining bronchioles. A higher magnification, the mass is composed by hypocellular collagen lamellae. Associations * Morvan Syndrome * HIV/AIDS * Posterior uveitis * Castleman's disease Castleman disease (CD) describes a group of rare lymphoproliferative disorders that involve enlarged lymph nodes, and a broad range of inflammatory symptoms and laboratory abnormalities. Whether Castleman disease should be considered an autoimmu ... References Pulmonary lesion {{Symptom-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
