Pulmonary hyalinizing granuloma with hydronephrosis.
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A 49-year-old man was admitted for the evaluation of a bilateral mass shadow in his chest X-ray film. No definitive diagnosis was established either by brushing cytology or biopsy through bronchoscopy. No malignancies were suggested by general work-up. Both masses were surgically removed, and were diagnosed as pulmonary hyalinizing granuloma (PHG). Fifteen months later, low grade fever continued and the renal function decreased. Laboratory examinations revealed bilateral hydronephrosis with polyclonal hypergammaglobulinemia. The findings of abdominal CT and urography were compatible with retroperitoneal fibrosis. Steroid treatment completely reversed the initial abnormality in laboratory data and the symptoms disappeared. (+info)
Retroperitoneal fibrosis.
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Retroperitoneal fibrosis (RPF) is an uncommon collagen vascular disease of unclear aetiology. It is characterised by a chronic non-specific inflammation of the retroperitoneum, which can entrap and obstruct retroperitoneal structures, notably the ureters. Because of the protean manifestations of RPF, awareness of the disease is important. It is still not uncommon to detect RPF only after severe renal failure is present. This comprehensive review deals with the various aspects of RPF and tries to provide a framework for the diagnosis, treatment and follow-up of this intriguing condition. Although it may have various causes, chronic periaortitis appears to be an increasingly encountered form of secondary RPF in patients with advanced atherosclerosis. Irrespective of its cause, most cases of non-malignant RPF - if in the active 'cellular' stage - will respond to treatment with corticosteroids, thereby obviating the need for surgical treatment. The clinical and radiographic improvement seen after starting steroid therapy is often impressive and reassuring as to the diagnosis. Treatment with corticosteroids may also make aneurysmectomy, if indicated, feasible in the patient who presents with perianeurysmal fibrosis and renal failure. Accumulating data suggest alternative treatment strategies for steroid-resistant cases (i.e. intensive immunosuppression) or when steroids are not feasible (i.e. other forms of immunosuppression or hormonal treatment, particularly tamoxifen). Although early diagnosis and treatment provide excellent renal and patient outcome, long-term follow-up is mandatory in all cases. (+info)
Endovascular treatment of iliocaval occlusion caused by retroperitoneal fibrosis: late results in two cases.
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We report two cases of iliocaval occlusion caused by retroperitoneal fibrosis; one presented acute symptoms and one chronic. Both were treated by use of transluminal angioplasty and stenting with excellent clinical, hemodynamic, and imaging results at 36 and 51 months. These cases confirm the benefit of endovascular techniques in the treatment of nonmalignant obstructive disease of large veins, and specifically in the case of retroperitoneal fibrosis. (+info)
Azygous vein to right atrium bypass graft in a patient with idiopathic fibrosing mediastinitis and symptomatic superior and inferior vena cava obstructions.
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We report an unusual case of a patient with diminished cardiac output caused by systemic venous occlusions of unclear etiology with a history of idiopathic fibrosing mediastinitis and retroperitoneal fibrosis. On thoracic, abdominal, and pelvic venograms, the patient was previously known to have superior vena caval and infrarenal inferior vena caval occlusions. Subsequent studies revealed retrograde flow through the azygous and hemiazygous veins and numerous caval-portal anastomoses providing venous return from the upper and lower extremities. After an extensive hypercoagulable state work-up, no abnormalities were found. To relieve the symptoms, the patient underwent an elective right thoracotomy with azygous vein to right atrial bypass graft. The patient has had complete amelioration of the disabling symptoms and participates in many activities that he could not tolerate previously. Because this condition and procedure are unreported, we present a novel surgical treatment option to palliate symptoms caused by concurrent superior and inferior caval obstructions by improving venous return to the heart. (+info)
Analysis of 4.3 kilobases of divergent locus B of macaque retroperitoneal fibromatosis-associated herpesvirus reveals a close similarity in gene sequence and genome organization to Kaposi's sarcoma-associated herpesvirus.
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We previously identified retroperitoneal fibromatosis-associated herpesvirus (RFHV) as a simian homolog of Kaposi's sarcoma-associated herpesvirus (KSHV) in a fibroproliferative malignancy of macaques that has similarities to Kaposi's sarcoma. In this report, we cloned 4.3 kb of divergent locus B (DL-B) flanking the DNA polymerase gene from two variants of RFHV from different species of macaque with a consensus degenerate hybrid oligonucleotide primer approach. Within the DL-B region of RFHV, viral homologs of the cellular interleukin-6, dihydrofolate reductase, and thymidylate synthase genes were identified, along with a homolog of the gammaherpesvirus open reading frame (ORF) 10. In addition, a homolog of the KSHV ORF K3, the modulator of immune recognition-1, was identified. Our data show a close similarity in sequence conservation, gene content, and genomic structure between RFHV and KSHV which strongly supports the grouping of these viral species within the same RV-1 rhadinovirus lineage and the hypothesis that RFHV is the macaque homolog of KSHV. (+info)
Inflammatory aneurysm in the infrarenal portion of thoracoabdominal aneurysms: an uncommon variant.
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INTRODUCTION: We present an unusual variant of type IV thoracoabdominal aneurysm with inflammatory changes in part of the aortic wall. MATERIAL AND METHODS: Between January 1990 and December 2000, 5 male patients (mean age, 68 years) with inflammatory aneurysms of 29 with type IV thoracoabdominal aneurysms (17.2%) underwent surgery. All five had arterial hypertension. The diagnosis of inflammatory aneurysm was made on the basis of clinical suspicion supported by characteristic features on CT scans. Endoaneurysmorraphy was performed in all cases; a tube graft was inserted in three cases, and bifurcation was performed in 2. The macroscopic appearance of periaortic fibrosis was detected at the infrarrenal portion of the aneurysm in the 5 patients, but no retroperitoneal fibrosis was found at the level of the distal thoracic aorta in any case. RESULTS: There was no operative mortality. Paraparesis was not detected in any patient. One patient had acute kidney failure, and another required an extended stay in the intensive care unit because of respiratory insufficiency. Pathologic examination confirmed the diagnosis of inflammatory aneurysm in all 5 patients, with characteristic adventitial thickening caused by lymphoplasmacytic infiltrate and fibrosis around the ganglionic and nerve structures. CONCLUSIONS: Type IV thoracoabdominal aneurysm with inflammatory changes can be treated at surgery with a retroperitoneal approach. Usually the fibrotic response is confined to the infrarenal portion of the aneurysm. (+info)
Idiopathic retroperitoneal fibrosis mimicking a pelvic tumor: a case of pericystitis plastica.
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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. (+info)
Sclerosing cholangitis associated with multifocal fibrosis: a case report.
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The association of sclerosing cholangitis, retroperitoneal fibrosis, and Riedel's thyroiditis has been reported twice before, and on both occasions the authors successfully used steroids to control the fibrotic process. A further case of fibrosis with this triad of organ involvement in which a combination of surgery and steroid treatment has arrested disease progression is described. This suggests an inappropriate immune response in this type of fibrotic overlap syndrome. (+info)