Immunoglobulin-g4(IgG4)-rich Pseudotumor of the Kidney – A case report and review of the literature
IgG4-related disease is a newly identified fibroinflammatory condition characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells and storiform fibrosis. Elevated serum IgG4 levels are frequently observed. IgG4-related disease affects predominantly...
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| Format: | Article |
| Language: | English |
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Peytchinski Publishing
2018-01-01
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| Series: | Journal of IMAB |
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| Online Access: | https://www.journal-imab-bg.org/issues-2018/issue1/JofIMAB-2018-24-1p1872-1877.pdf |
| _version_ | 1818243187247939584 |
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| author | Orlin Savov Abbas Agaimy Ralf Rieker Ekkehardt Bismarck Joachim Dörsam Georg C. Rass Thomas Ebert Arndt Hartmann Bernd J. Schmitz-Dräger |
| author_facet | Orlin Savov Abbas Agaimy Ralf Rieker Ekkehardt Bismarck Joachim Dörsam Georg C. Rass Thomas Ebert Arndt Hartmann Bernd J. Schmitz-Dräger |
| author_sort | Orlin Savov |
| collection | DOAJ |
| description | IgG4-related disease is a newly identified fibroinflammatory condition characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells and storiform fibrosis. Elevated serum IgG4 levels are frequently observed. IgG4-related disease affects predominantly middle-aged and elderly patients, with male predominance and responds favorably to steroids. The patients present with symptoms referable to the involvement of one or more sites either simultaneously or serially, usually in the form of mass lesions affecting various organs including pancreas, biliary tree, salivary glands, periorbital tissues, kidneys, lungs, retroperitoneum and lymph nodes. The case of a 61-year old male referred for therapy of a 6.6 cm tumorous lesion of the right kidney is presented. Histological assessment of the resected mass showed IgG4-rich sclerosing pseudotumor. The available literature is summarized, and diagnostic and therapeutic options for this condition are discussed. Consideration of a non-malignant disease may be reasonable even in cases of CAT scan findings suggestive of renal cancer. |
| first_indexed | 2024-12-12T13:57:08Z |
| format | Article |
| id | doaj.art-f37950bce50b4573962accfe6c2b9b68 |
| institution | Directory Open Access Journal |
| issn | 1312-773X |
| language | English |
| last_indexed | 2024-12-12T13:57:08Z |
| publishDate | 2018-01-01 |
| publisher | Peytchinski Publishing |
| record_format | Article |
| series | Journal of IMAB |
| spelling | doaj.art-f37950bce50b4573962accfe6c2b9b682022-12-22T00:22:27ZengPeytchinski PublishingJournal of IMAB1312-773X2018-01-012411872187710.5272/jimab.2018241.1872Immunoglobulin-g4(IgG4)-rich Pseudotumor of the Kidney – A case report and review of the literatureOrlin Savov0Abbas Agaimy1Ralf Rieker2Ekkehardt Bismarck3Joachim Dörsam4Georg C. Rass5Thomas Ebert6Arndt Hartmann7Bernd J. Schmitz-Dräger8Urologie24 and Department of Urology, St. Theresien-Krankenhaus Nürnberg, GermanyInstitute of Pathology, Medical School, Philipp-Alexander-University Erlangen, GermanyInstitute of Pathology, Medical School, Philipp-Alexander-University Erlangen, GermanyUrologie24 and Department of Urology, St. Theresien-Krankenhaus Nürnberg, GermanyUrologie24 and Department of Urology, St. Theresien-Krankenhaus Nürnberg, GermanyUrologie24 and Department of Urology, St. Theresien-Krankenhaus Nürnberg, GermanyUrologie24 and Department of Urology, St. Theresien-Krankenhaus Nürnberg, GermanyInstitute of Pathology, Medical School, Philipp-Alexander-University Erlangen, GermanyDepartment of Urology, Philipp-Alexander-University, Erlangen, Germany.IgG4-related disease is a newly identified fibroinflammatory condition characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells and storiform fibrosis. Elevated serum IgG4 levels are frequently observed. IgG4-related disease affects predominantly middle-aged and elderly patients, with male predominance and responds favorably to steroids. The patients present with symptoms referable to the involvement of one or more sites either simultaneously or serially, usually in the form of mass lesions affecting various organs including pancreas, biliary tree, salivary glands, periorbital tissues, kidneys, lungs, retroperitoneum and lymph nodes. The case of a 61-year old male referred for therapy of a 6.6 cm tumorous lesion of the right kidney is presented. Histological assessment of the resected mass showed IgG4-rich sclerosing pseudotumor. The available literature is summarized, and diagnostic and therapeutic options for this condition are discussed. Consideration of a non-malignant disease may be reasonable even in cases of CAT scan findings suggestive of renal cancer.https://www.journal-imab-bg.org/issues-2018/issue1/JofIMAB-2018-24-1p1872-1877.pdfInterstitial nephritisImmune complexIgG4-related diseaseMembranous glomerulonephritisMembranous nephropathyRenal mass |
| spellingShingle | Orlin Savov Abbas Agaimy Ralf Rieker Ekkehardt Bismarck Joachim Dörsam Georg C. Rass Thomas Ebert Arndt Hartmann Bernd J. Schmitz-Dräger Immunoglobulin-g4(IgG4)-rich Pseudotumor of the Kidney – A case report and review of the literature Journal of IMAB Interstitial nephritis Immune complex IgG4-related disease Membranous glomerulonephritis Membranous nephropathy Renal mass |
| title | Immunoglobulin-g4(IgG4)-rich Pseudotumor of the Kidney – A case report and review of the literature |
| title_full | Immunoglobulin-g4(IgG4)-rich Pseudotumor of the Kidney – A case report and review of the literature |
| title_fullStr | Immunoglobulin-g4(IgG4)-rich Pseudotumor of the Kidney – A case report and review of the literature |
| title_full_unstemmed | Immunoglobulin-g4(IgG4)-rich Pseudotumor of the Kidney – A case report and review of the literature |
| title_short | Immunoglobulin-g4(IgG4)-rich Pseudotumor of the Kidney – A case report and review of the literature |
| title_sort | immunoglobulin g4 igg4 rich pseudotumor of the kidney a case report and review of the literature |
| topic | Interstitial nephritis Immune complex IgG4-related disease Membranous glomerulonephritis Membranous nephropathy Renal mass |
| url | https://www.journal-imab-bg.org/issues-2018/issue1/JofIMAB-2018-24-1p1872-1877.pdf |
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