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Investigative and Clinical Urology Sep 2017To study clinical presentation, laboratory results, imaging findings and treatment options and outcomes of retroperitoneal fibrosis (RPF). To determine whether it...
PURPOSE
To study clinical presentation, laboratory results, imaging findings and treatment options and outcomes of retroperitoneal fibrosis (RPF). To determine whether it follows the same natural course and response to treatment in the Asian population as in the Western world.
MATERIALS AND METHODS
Medical records of patients diagnosed with RPF on imaging and histopathology between February 2010 and April 2016 were reviewed.
RESULTS
Of the 21 patients analyzed, mean age at presentation was 50.81 years. The male to female ratio was 0.9:1. Pain was most common presenting complaint (95.23% cases), almost 85% cases were idiopathic and rests were postradiation induced. The median creatinine level was 1.8 mg/dL. The mean erythrocyte sedimentation rate (ESR) was 53.2 mm/h. Hydronephrosis was present in all patients and 47.6% had atrophic kidneys. Diffuse retroperitoneal mass was present in 61.1%. Ureterolysis with lateralization, omental wrapping or gonadal pedicle wrap was done in 17 cases. Two patients underwent uretero-ureterostomy. One patient underwent ileal replacement of ureter, and one ileal conduit. Eighteen patients received concurrent medical treatment, 11 were given tamoxifen, 2 steroids (Prednisolone), and five were given both. Of the 20 patients with follow-up, 70% had complete symptomatic relief; ESR improvement was seen in 77.8%. Follow-up ultrasound showed resolved and decreased hydronephrosis in 20% and 55% respectively. One patient had treatment failure and 17.65% had disease recurrence.
CONCLUSIONS
RPF is a rare disease with varied presentation and outcomes. The male to female ratio may be equal in Asians and smoking could be lesser contributing factor. More Asian cohort studies are required to support same.
Topics: Adult; Aged; Atrophy; Blood Sedimentation; Creatinine; Female; Hemoglobins; Humans; Hydronephrosis; Kidney; Male; Middle Aged; Pain; Recurrence; Retroperitoneal Fibrosis; Tomography, X-Ray Computed; Treatment Outcome; Ultrasonography; Urography
PubMed: 28868510
DOI: 10.4111/icu.2017.58.5.371 -
BMC Women's Health Oct 2022Retroperitoneal fibrosis is a rare disease characterized by chronic nonspecific inflammation, which leads to clinical compression manifestations of retroperitoneal... (Review)
Review
BACKGROUND
Retroperitoneal fibrosis is a rare disease characterized by chronic nonspecific inflammation, which leads to clinical compression manifestations of retroperitoneal organs especially ureter. Approximately 70 percent of retroperitoneal fibrosis cases are idiopathic which has no clear etiology. This study reported a rare case of a 48-year-old woman presented with idiopathic retroperitoneal fibrosis and endometrial cancer.
CASE PRESENTATION
A 48-year-old woman presented with irregular vaginal bleeding without abdominal pain, bloating or discomfort. The patient was diagnosed iRPF after splenectomy 13 years ago. Then she took prednisone for 2 years and took tamoxifen for about 11 years. She stopped taking the medication from October 2019 to May 2020 and then started taking tamoxifen again until November 2020. Two weeks after she stopped taking tamoxifen, she presented with irregular vaginal bleeding. Gynecological ultrasound revealed a thick endometrium with uneven echo enhancement and blood flow signals. Then diagnostic curettage was performed with pathological examination showed endometroid carcinoma. Later, the patient was admitted to Peking University Third Hospital for surgery. Preoperative imaging examinations, including CT, MRI, and PET/CT, all showed pelvic enlarged lymph nodes and they were highly suspected to have lymph node metastasis. The patient underwent laparoscopic surgical staging and enlarged lymph nodes in the pelvic and aortic regions were removed. Finally, the pathology confirmed that endometrioid adenocarcinoma and fibrosis, but there was no tumor infiltration in these enlarged lymph nodes. The patient is now in good condition.
CONCLUSION
This case report stressed the difficulty to distinguish between lymph node metastasis and inflammatory hyperplasia by common imaging methods. Due to increased surgical difficulty among retroperitoneal patients, lymphadenectomy should be carefully evaluated to avoid additional surgical complications and over-treatment.
Topics: Endometrial Neoplasms; Female; Humans; Lymph Node Excision; Lymph Nodes; Lymphatic Metastasis; Middle Aged; Positron Emission Tomography Computed Tomography; Prednisone; Retroperitoneal Fibrosis; Tamoxifen; Uterine Hemorrhage
PubMed: 36183056
DOI: 10.1186/s12905-022-01968-8 -
Cureus Dec 2020Retroperitoneal fibrosis (RPF) is a rare condition characterized by fibroinflammatory tissue infiltrating and compressing retroperitoneal structures. While mostly...
Retroperitoneal fibrosis (RPF) is a rare condition characterized by fibroinflammatory tissue infiltrating and compressing retroperitoneal structures. While mostly idiopathic (idiopathic retroperitoneal fibrosis or IRF), RPF is frequently associated with certain drugs, infections, and malignancies. It is thought to be immune-mediated because of response to steroids and RPF is commonly seen with other autoimmune diseases, especially IgG4-related disease (IgG4-RD). IRF is also a part of the chronic aortitis syndromes and the presence of aortic aneurysms is another characteristic of this disease. A 63-year old woman presented with left-sided flank pain. Computed tomography (CT) scan showed left hydronephrosis from compression of the ureter by a retroperitoneal mass. A thoracoabdominal aneurysm was also noted. A [F]- fluorodeoxyglucose positron emission tomography (FDG-PET) scan showed hypermetabolism in the mass, with no abnormally increased activity noted elsewhere. Within four months, the mass enlarged to involve the right ureter as well, leading to right hydronephrosis. She required bilateral ureteral stents and aneurysm repair. Biopsy of the mass showed dense fibrosis with a mononuclear cell infiltrate. The histology of the aneurysm specimen showed chronic periaortic inflammation. Laboratory investigations were significant for elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), with no evidence of monoclonal gammopathy. She was referred to the rheumatology clinic to receive steroid treatment for IRF. IRF commonly involves the ureters and is diagnosed on CT scans during a workup for obstructive uropathy. The treatment is high dose steroids, while in resistant cases, other immunosuppressants have been used. The presentation of a patient with IRF can commonly mimic that of urinary calculi and malignancy. While rare, IRF should not be forgotten when evaluating a patient for obstructive uropathy.
PubMed: 33489559
DOI: 10.7759/cureus.12147 -
World Journal of Gastroenterology Jan 2013Retroperitoneal fibrosis is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal... (Review)
Review
Retroperitoneal fibrosis is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs. Retroperitoneal fibrosis can be of 2 types: idiopathic and secondary. The recently advocated concept and diagnostic criteria of immunoglobulin G4 (IgG4)-related disease, derived from research on autoimmune pancreatitis (AIP), has led to widespread recognition of retroperitoneal fibrosis as a condition caused by IgG4-related disease. We now know that previously diagnosed idiopathic retroperitoneal fibrosis includes IgG4-related disease; however, the actual prevalence is unclear. Conversely, some reports on AIP suggest that retroperitoneal fibrosis is concurrently found in about 10% of IgG4-related disease. Because retroperitoneal fibrosis has no specific symptoms, diagnosis is primarily based on diagnostic imaging (computed tomography and magnetic resonance imaging), which is also useful in evaluating the effect of therapy. Idiopathic retroperitoneal fibrosis can occur at different times with other lesions of IgG4-related disease including AIP. Thus, the IgG4 assay is recommended to diagnose idiopathic retroperitoneal fibrosis. High serum IgG4 levels should be treated and monitored as a symptom of IgG4-related disease. The first line of treatment for retroperitoneal fibrosis is steroid therapy regardless of its cause. For patients with concurrent AIP, i.e., IgG4-related retroperitoneal fibrosis, the starting dose of steroid is usually 30-40 mg/d. The response to steroid therapy is generally favorable. In most cases, the pancreatic lesion and retroperitoneal fibrosis improve after the initial treatment. However, the epidemiology, treatment for recurring retroperitoneal fibrosis, and long-term prognosis are still largely unknown. Further analysis of such cases and research are necessary.
Topics: Autoimmune Diseases; Humans; Immunoglobulin G; Pancreatitis; Prevalence; Prognosis; Recurrence; Retroperitoneal Fibrosis; Steroids; Treatment Outcome
PubMed: 23326160
DOI: 10.3748/wjg.v19.i1.35 -
Internal Medicine (Tokyo, Japan) Oct 2021
Topics: Diagnosis, Differential; Humans; Lymphoma; Retroperitoneal Fibrosis
PubMed: 33867392
DOI: 10.2169/internalmedicine.7142-21 -
Therapeutic Advances in Urology Apr 2015Idiopathic retroperitoneal fibrosis (IRF) is a rare disease characterized by a fibrotic reaction that affects retroperitoneal organs, especially the urinary tract. In... (Review)
Review
INTRODUCTION
Idiopathic retroperitoneal fibrosis (IRF) is a rare disease characterized by a fibrotic reaction that affects retroperitoneal organs, especially the urinary tract. In this review we analyze the current imaging techniques, morphological characteristics, clinical aspects and therapeutic aspects of idiopathic retroperitoneal disease.
METHODS
A PubMed search was conducted in December 2013 to find original articles, bibliographic reviews and series reports published in the past 15 years on idiopathic retroperitoneal fibrosis, its management and outcomes by combining terms like retroperitoneal fibrosis, periaortitis, treatment and autoimmune. A total of 89 articles were included in this review that referred strictly to IRF. We analyzed the imaging tools used for diagnostic and the decision making protocol used by physicians in the management of IRF.
RESULTS
A computerized tomography (CT) scan represents the most commonly used imaging technique for diagnosis. Magnetic resonance imaging (MRI) is unable to differentiate more accurately between benign and malignant retroperitoneal fibrosis (RF) than a CT scan. Biopsy remains the most reliable diagnostic tool for IRF. However, the histological characteristics of IRF are not yet well-defined and the protocol for biopsy is not standardized in terms of template, number of biopsies and the immunohistochemical panel needed for positive diagnosis. The most common treatment reported is corticosteroid therapy alone or in combination with other immunosuppressants, whereas surgical treatment is reserved for severe cases. Indwelling ureteric stents represent the most common procedure for renal drainage, but their efficacy is questionable. Open ureterolysis remains the gold standard for surgical treatment, but its purpose is only to resolve the ureteric obstruction, not to treat the retroperitoneal fibrosis. Laparoscopic and robotic approaches have been reported to be feasible, but no prospective, comparative trials have been performed due to the rarity of the disease. Surgical technique is not standardized and the outcome of the treatment only evaluates the recovery of the renal function.
CONCLUSIONS
The imaging procedures available today are unable to accurately differentiate between idiopathic and malignant RF. A biopsy is mandatory to confirm the diagnosis, but there is no consensus regarding the template, timing and number of biopsies needed to exclude malignancy. Open ureterolysis represents the main surgical treatment for cases with severe IRF, and laparoscopic or robotic approach may be an option in selected cases. The recovery of the renal function is a surrogate for evaluating the success of the treatment. More clinical studies are needed in order standardize the protocol for diagnostic, treatment and follow up after medical or surgical management.
PubMed: 25829952
DOI: 10.1177/1756287214565637 -
Experimental and Therapeutic Medicine Jun 2017Retroperitoneal fibrosis (RPF) is a rare disease characterized by infiltration of inflammatory cells and deposition of thickened fibrous tissues. The present study...
Retroperitoneal fibrosis (RPF) is a rare disease characterized by infiltration of inflammatory cells and deposition of thickened fibrous tissues. The present study presents the case of a 53-year-old patient treated for generalized weakness and fatigue for 1 year prior to hospitalization. A cardiac ultrasound revealed pericardial effusion that required pericardiocentesis, during which 1,400 ml serous fluid with the characteristics of an exudate was aspirated. A pericardiectomy was performed due to persistent effusion and histological examination indicated pericardial fibrosis. A thoracic-abdominal computed tomography scan revealed the presence of retroperitoneal fibrosis. The patient was treated with corticosteroids and azathioprine. Follow-up examinations showed a significant reduction in the amount of abdominal fibrous tissue and no increase in pericardial effusion 1 year following the end of treatment. The patient continues to have regular follow-up control examinations with a cardiologist and nephrologist.
PubMed: 28588687
DOI: 10.3892/etm.2017.4425 -
Reviews in Urology 2014Retroperitoneal fibrosis (RPF) is a rare disease characterized by the replacement of normal tissue with fibrosis and/or inflammation. In this case, a 68-year-old man... (Review)
Review
Retroperitoneal fibrosis (RPF) is a rare disease characterized by the replacement of normal tissue with fibrosis and/or inflammation. In this case, a 68-year-old man presented with RPF in the pelvis, a rare location for this disease. Biopsies were performed, which showed elevated levels of C-reactive protein, erythrocyte sedimentation rate, and, most importantly, immunoglobulin G4 (IgG4). It has been postulated that IgG4-related sclerosing disease is a systemic disease. Treatment has been successful with systemic corticosteroids.
PubMed: 25009451
DOI: No ID Found -
Proceedings of the Royal Society of... Feb 1968
Topics: Humans; Male; Middle Aged; Prognosis; Retroperitoneal Fibrosis; Ureteral Diseases
PubMed: 5637819
DOI: No ID Found -
Canadian Medical Association Journal Sep 1961
Topics: Disease; Gastrointestinal Diseases; Retroperitoneal Fibrosis; Retroperitoneal Space
PubMed: 13696506
DOI: No ID Found