Cases reported "Retroperitoneal Fibrosis"

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1/17. Inflammatory abdominal aortic aneurysm treated by endovascular stent grafting: a case report.

    Despite complications inherent to open surgical repair of inflammatory abdominal aortic aneurysms, there is expected resolution of the retroperitoneal inflammatory process following graft replacement. An endovascular approach could also exclude the aneurysm while potentially avoiding injury to vital structures in the hostile operative field. However, data are limited regarding the role of endovascular stent grafts in the management of inflammatory abdominal aortic aneurysms. Furthermore, postoperative regression of perianeurysmal inflammation is rarely discussed in the few published accounts of endovascular repair of inflammatory aortic aneurysms. The case presented demonstrates successful endovascular treatment of an infrarenal inflammatory aneurysm with resolution of the retroperitoneal inflammation and hydronephrosis.
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2/17. Pseudotumor of the pancreatic head associated with idiopathic retroperitoneal fibrosis.

    A 68-year-old male presented with abdominal pain and obstructive jaundice. CT revealed a large mass in the pancreatic head that was initially interpreted as pancreatic carcinoma. Needle biopsy revealed only fibrous tissue with signs of chronic inflammation. Together with typical findings of an idiopathic retroperitoneal fibrosis, the final diagnosis of multifocal idiopathic fibrosclerosis with focal pseudotumorous pancreatic head fibrosis could be made.
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3/17. Riedel's thyroiditis and retroperitoneal fibrosis in multifocal fibrosclerosis: positron emission tomographic findings.

    PURPOSE: The authors describe F-18 fluorodeoxyglucose positron emission tomographic (FDG PET) imaging features of Riedel's thyroiditis and retroperitoneal fibrosis in a patient with multifocal fibrosclerosis. MATERIALS AND methods: A 41-year-old woman in whom Riedel's thyroiditis had been diagnosed 7 months earlier was examined for fatigue, anorexia, and lower back pain, irradiating to the abdomen. Abdominal sonography and computed tomography showed a retroperitoneal mass. A biopsy of this mass showed histopathologic findings of retroperitoneal fibrosis. FDG PET was performed to evaluate the activity of the retroperitoneal fibrosis and to screen for other areas of fibrosclerosis. RESULTS: The FDG-PET images showed an intense hypermetabolic abdominal mass surrounding the aorta and increased glucose metabolism in the thyroid. No other sites of abnormal FDG metabolism were noted. These abnormalities disappeared after 4 months of steroid therapy. CONCLUSIONS: Sites of multifocal fibrosclerosis can be demonstrated by FDG PET, probably as a result of active inflammation involving lymphocytes, plasma cells, and fibroblast proliferation. FDG PET can help to establish the diagnosis of multifocal fibrosclerosis and evaluate the activity and patient response to corticosteroid therapy.
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4/17. Idiopathic retroperitoneal fibrosis mimicking a pelvic tumor: a case of pericystitis plastica.

    retroperitoneal fibrosis was first described in 1905 by Albarran, a French urologist, who performed ureterolysis for ureteral compression produced by the disease. However, this disease became an established clinical entity by Ormond's account in the English literature in 1948. Pericystitis plastica has been used the define an extremely rare type of Idiopathic retroperitoneal fibrosis (IRF) constricting the bladder. In this study, we discussed the recovery of 29-year-old woman with pericystitis plastica who was misdiagnosed as pelvic malignancy or a chronic/subacut pelvic inflammation at the first evaluation.
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5/17. Non-aneurysmal inflammation of the aorta in combination with aortic valve disease.

    A 61-year-old man presented with aortic valve stenosis, coronary artery disease in combination with rheumatic arthritis, peri-aortic inflammation and fibrosis with aortitis. In the pathological literature this has been termed chronic periaortitis, while in the surgical literature, non-aneurysmal dilatation of the aorta associated with atherosclerosis and inflammation has been described as aortitis. The name chronic periarteritis refers to the associated involvement of coronary arteries. Although different terminologies have been used, it is suggested that all of them most likely represent the same disease entity.
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6/17. Laparoscopic diagnosis and management of perinephric adipose-containing lesions.

    We describe two patients in whom imaging and percutaneous biopsy of perinephric masses were insufficient for diagnosis. Laparoscopic biopsy permitted the diagnosis of fibrosis with chronic inflammation in one case and liposarcoma in the other.
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7/17. Hyper-IgG4 disease: report and characterisation of a new disease.

    BACKGROUND: We highlight a chronic inflammatory disease we call 'hyper-IgG4 disease', which has many synonyms depending on the organ involved, the country of origin and the year of the report. It is characterized histologically by a lymphoplasmacytic inflammation with IgG4-positive cells and exuberant fibrosis, which leaves dense fibrosis on resolution. A typical example is idiopathic retroperitoneal fibrosis, but the initial report in 2001 was of sclerosing pancreatitis. methods: We report an index case with fever and severe systemic disease. We have also reviewed the histology of 11 further patients with idiopathic retroperitoneal fibrosis for evidence of IgG4-expressing plasma cells, and examined a wide range of other inflammatory conditions and fibrotic diseases as organ-specific controls. We have reviewed the published literature for disease associations with idiopathic, systemic fibrosing conditions and the synonyms: pseudotumour, myofibroblastic tumour, plasma cell granuloma, systemic fibrosis, xanthofibrogranulomatosis, and multifocal fibrosclerosis. RESULTS: histology from all 12 patients showed, to varying degrees, fibrosis, intense inflammatory cell infiltration with lymphocytes, plasma cells, scattered neutrophils, and sometimes eosinophilic aggregates, with venulitis and obliterative arteritis. The majority of lymphocytes were T cells that expressed CD8 and CD4, with scattered B-cell-rich small lymphoid follicles. In all cases, there was a significant increase in IgG4-positive plasma cells compared with controls. In two cases, biopsies before and after steroid treatment were available, and only scattered plasma cells were seen after treatment, none of them expressing IgG4. review of the literature shows that although pathology commonly appears confined to one organ, patients can have systemic symptoms and fever. In the active period, there is an acute phase response with a high serum concentration of IgG, and during this phase, there is a rapid clinical response to glucocorticoid steroid treatment. CONCLUSION: We believe that hyper-IgG4 disease is an important condition to recognise, as the diagnosis can be readily verified and the outcome with treatment is very good.
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8/17. IgG4-associated multifocal systemic fibrosis complicating sclerosing sialadenitis, hypophysitis, and retroperitoneal fibrosis, but lacking pancreatic involvement.

    A 71-year-old man was admitted with malaise, mild fever, anorexia, body weight loss, lower back pain, thirst, and polydipsia. He showed bilateral swelling of the submandibular glands. Examinations showed panhypopituitarism and a high serum IgG4 concentration. Fluorodeoxyglucose positron emission tomography (FDG-PET) revealed uptake in the pituitary gland, bilateral submandibular gland, bilateral hilar and mediastinal lymph nodes, and a mass consistent with retroperitoneal fibrosis, but not in the pancreas. biopsy specimens from the submandibular gland and retroperitoneal mass indicated sialadenitis and retroperitoneal fibrosis respectively, and showed severe fibrosis and inflammation with marked lymphoplasmacytic infiltration and IgG4-positive plasma cell infiltration. hormone replacement therapy with hydrocortisone resulted in marked clinical improvement. Systemic involvement found in this patient possibly corresponded to the new concept of IgG4-associated multifocal systemic fibrosis.
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9/17. Characterisation of inflammatory cells associated with "idiopathic retroperitoneal fibrosis".

    During ureterolysis in a patient with "idiopathic retroperitoneal fibrosis", fresh samples of peri-ureteric and peri-aortic tissue were obtained. An abdominal CT scan confirmed the peri-aortic distribution of the inflammation associated with advanced abdominal aortic atherosclerosis. histology confirmed the presence of fibrosis and a variable chronic inflammatory cell infiltrate. Monoclonal antibodies were used to identify the inflammatory cells. B and T lymphocytes were present with the majority of T lymphocytes of the T helper phenotype. The majority of lymphocytes and macrophages and most vascular endothelial cells were HLA-DR positive. Ki67 and BerH2 staining was found in B cells and T helper cells, indicating that these cells were proliferating and activated. These findings compare with the characterisation of inflammatory cells associated with "inflammatory aneurysms" and with the inflammatory cells present in the spectrum of inflammation seen as a complication of advanced atherosclerosis--conditions known as "chronic peri-aortitis". It is suggested that our findings support the view that idiopathic retroperitoneal fibrosis represents clinical chronic peri-aortitis seen in an undilated aorta.
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10/17. Characterization of inflammatory cells in a patient with chronic periaortitis.

    During repair of an inflammatory abdominal aortic aneurysm, fresh samples of the aneurysm wall were obtained. histology confirmed the diagnosis of chronic periaortitis,- advanced atherosclerosis with medial attenuation, and periaortic inflammation. Monoclonal antibodies were used to identify the cells of the inflammatory infiltrate. The majority of the adventitial lymphocytes were B cells. No B cells were present in the atheroma. The majority of mature plasma cells contained IgG with a few plasma cells containing IgM. T cells, predominantly T helper cells, were found in the atheroma and adventitia. Positive DR staining was shown by the majority of lymphocytes, by macrophage-like cells in germinal centers of lymphoid follicles in the adventitia, by some vascular endothelial cells, and by some smooth muscle cells. The implications of these findings are discussed.
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