Diagnosis Of Retroperitoneal Fibrosis Research Articles

Role of transduodenal endoscopic ultrasound-guided fine-needle aspiration/biopsy (EUS-FNA/FNB) for diagnosis of retroperitoneal fibrosis (Ormond's disease).

Retroperitoneal fibrosis (RPF), often referred to as Ormond's disease when it is of idiopathic origin, is a rare disease characterized by the presence of inflammatory infiltrates and periaortic masses in the retroperitoneum. For a definite diagnosis, a biopsy and subsequent pathological examination is required. Currently accepted methods for retroperitoneal biopsy include open, laparoscopic, or CT-guided approaches. However, transduodenal endoscopic ultrasound-guided fine-needle aspiration/biopsy (EUS-FNA/FNB) for diagnosis of RPF has attracted only little attention in the literature. We report two male patient cases who presented with leukocytosis, elevated C-reactive protein, and a suspicious retroperitoneal mass of unknown origin on computed tomography. One patient also reported left lower quadrant pain, whereas the other patient suffered from back pain and weight loss. In both patients, idiopathic RPF was successfully diagnosed by using transduodenal EUS-FNA/FNB with 22- and 20-gauge aspiration needles. Histopathology revealed dense lymphocytic infiltrates and fibrosis. The procedures lasted approximately 25 and 20 minutes, respectively, and in both patients no serious adverse events occurred. Treatment included steroid therapy and administration of Azathioprine. We demonstrate that using EUS-FNA/FNB to diagnose RPF is a feasible, fast, and safe method, which should always be considered as a first-line diagnostic modality. Hence, this case report emphasizes that gastrointestinal endoscopists are likely to play an important role in the setting of suspected RPF.

Characteristics of Adult Patients with Idiopathic Retroperitoneal Fibrosis and Assessment of Risk of Relapse at Diagnosis.

Objectives: To compare adult patients’ characteristics suffering from idiopathic retroperitoneal fibrosis between “relapse-free” and relapsing patients at the diagnosis and identify factors associated with relapse at initial presentation. Methods: We conducted a retrospective multicentric study in four hospitals in Eastern France, from 1993 to 2020, of adult patients suffering from idiopathic retroperitoneal fibrosis. We analyzed clinical, biological, and radiological features at diagnosis and during a forty-month follow-up. Results: Of 47 patients suffering from retroperitoneal fibrosis, 21 patients had idiopathic retroperitoneal fibrosis. Among them, 13 experienced one or more relapses during follow-up. At diagnosis, clinical characteristics, relevant comorbidities, biological and radiological features were similar between groups. Smoking cessation seems associated with decreased relapse risk (p: 0.0624). A total of 8 patients developed chronic renal failure during follow-up. Ureteral infiltration at diagnosis was associated with evolution to chronic renal failure (p: 0.0091). Conclusion: No clinical, biological, or radiological features could predict relapse at retroperitoneal fibrosis diagnosis, but smoking cessation may prevent relapse.

Retroperitoneal fibrosis and β-blocking agents: Is there an association?

Retroperitoneal fibrosis (RPF) is a rare chronic fibro-inflammatory disorder that may be secondary to certain drugs, including β-blocking agents (BBAs). However, their causative role is unclear. We aimed to investigate this association. Disproportionality analysis was carried out on cases from 1985 to 4 October 2020 in VigiBase, the World Health Organization pharmacovigilance database. The Bayesian-based IC025 metric and reporting odds ratio were used in order to assess the adverse event signal. We also analysed all published case reports from the literature regarding BBA-associated RPF to assess the value of suggested supportive clinical evidence. In total, 1599 individual case safety reports of RPF were reported to VigiBase, of which 132 (32%) concerned 16 different single BBA. For 12 of these agents (75%), reporting of RPF was disproportionate, indicating a potential safety signal. Line listing analysis of individual case safety reports showed no consistent time interval from start of BBA to RPF diagnosis (range 0.7-264 mo). Dechallenge was negative or unknown in the majority of cases (74%). In 18 published cases from the literature, time from start of BBA to RPF diagnosis varied widely (range 3-156 mo). BBA were discontinued 6months before (n=1) or at the time of RPF diagnosis (n=17). Most patients (84%) also received RPF specific treatment. Follow-up duration was short (median 5 mo [range 1-24 mo]) and in most cases (83%) relevant follow-up data were lacking. Although disproportionality analysis indicated a potential safety signal for RPF associated with BBAs, clinical evidence did not support a cause-and-effect relationship.

Retroperitoneal fibrosis at the Ibn Sina University Hospital of Rabat: about 18 cases

La fibrose rétropéritonéale (FRP) est une pathologie rare caractérisée par la formation d´une plaque fibro-inflammatoire dans l´espace rétropéritonéale en avant de l´aorte abdominale, responsable d´un engainement des uretères. Le tableau clinique n´est pas spécifique et la pathologie est souvent révélée par une uropathie obstructive. Nous avons mené une étude descriptive rétrospective étendue sur 10 ans, de janvier 2006 à décembre 2016, réalisée dans les services d´urologie B et de néphrologie du Centre Hospitalier Universitaire Ibn Sina. Nous avons inclus 18 patients dans l´étude dont 11 hommes et 7 femmes, avec un âge moyen de 51,4 ans ± 11,2. Le diagnostic a été révélé par des douleurs lombaires chez 14 patients. L´insuffisance rénale obstructive était présente chez 15 de nos patients et le diagnostic de FRP a été posé par l´uroscanner. Le bilan étiologique notait des antécédents de néoplasies pour 2 patients, de pathologies inflammatoires pour 2 autres patients et une chirurgie rétropéritonéale chez un patient. On notait l´association de prises médicamenteuses chroniques chez plus de la moitié des patients. Le traitement comportait un double volet: une sonde double J systématique chez tous les patients et un traitement systémique par de la corticothérapie et des immunosuppresseurs selon le profil évolutif. Le traitement par urétérolyse a été réalisé d´emblée chez 3 patients. L´évolution a été favorable avec une nette amélioration de la fonction rénale chez 12 patients. La rechute est survenue chez 2 patients après 2 ans de suivi. L´association de signes généraux à une insuffisance rénale obstructive doit faire évoquer le diagnostic de FRP. La recherche de causes secondaires devrait être systématique, avec un accent particulier à mettre sur le syndrome d´hyper IgG4 et les pathologies néoplasiques.

An international patient-centred study of retroperitoneal fibrosis.

The impact that rare chronic disorders, such as retroperitoneal fibrosis (RPF), can have on the physical and psychological aspects of a patient's health is poorly understood. Patient-related outcome measures and experiences provide a unique opportunity to understand the impact rare chronic disorders have on a patient's life as well as allowing healthcare providers to compare and improve performance. To understand the physical and psychosocial impact that RPF has upon peoples' lives. An international online questionnaire was therefore created to gain insights into how patients with RPF, a rare fibro-inflammatory condition, viewed their health and experiences. An international online questionnaire comprising 62 questions/free text options, was designed in collaboration with two patient advocates and the multi-disciplinary Renal Association Rare Disease Registry (RaDaR) RPF Group the questionnaire was anonymous and freely accessible on a GOOGLE Form online platform for 6 months. A total of 229 patients from 30 countries across 5 continents responded. Four key issues were identified; (i) pain; (ii) therapy-related side effects; (iii) lack of informed doctors/information about their condition and its management; and (iv) psychological burden. Variations in diagnosis and management are highlighted with 55% undergoing a biopsy to reach a diagnosis of RPF; 75% of patients underwent a further interventional procedure with 60% concurrently treated medically. This study will guide further development of clinical and academic multi-disciplinary activity and shows the importance of trying to understand the impact of rare chronic disorders on the physical and psychological aspects of a patient's health.

SAT-088 RETROPERITONEAL FIBROSIS: DIAGNOSTIC AND THERAPEUTIC APPROACH

Retroperitoneal fibrosis (RPF), also named Ormond’s disease, is characterized by a fibrosis reaction that takes place in the retroperitoneal space around the abdominal aorta, compressing the ureters and others structures. It is a rare disease. Its incidence rate goes from 0.7 to 1.3/100000 inhabitants per year. The purpose of our work is to realize a descriptive, epidemiological, clinical, etiological, therapeutic and evolutive study of the RPF. We have conducted a retrospective study in our department between 1985 and 2019. A clear male predominance was noted with 32 men and 7 women. Ten patients were recieving treatments for more than 2 pathologies. The leading medical histories among our patients, which can be a predictive factor of occurrence of RPF, were breast tumor for 1 patient who was in remission at the time of the diagnosis of RPF and abdominal or pelvic surgery in 8 cases. One patient was operated for aortic aneurysm. Three patients were victims of abdominal or lumbar trauma as a result of a road accident. Lymphadenitis tuberculosis was noted in one case. One patient was recieving an antagonist beta adrenergic. Circumstances of discovery were dominated by lumbar pain in 31 cases. Abdominal pain was objectified in 29 patients. Twenty five patients presented a wasting syndrome with anorexia and / or weight loss, and three patients had legs‘ oedema. Acute kideny injury, high blood pressure, biological inflammatory syndrome have been found as a circumstance of discovery, respectively in 23.3 and 16 patients. One case of RPF was discovered following an hepatic cholestasis and another one after an operation for a testicular hydrocele. The abdominal scan confirmed the diagnosis in all the cases. We practiced the biopsy of the RPF for only 8 patients. It had showed an extended fibrosis relative to a chronic pancreatitis (1 case), a non specific inflammatory tissue (1 case). It was not interpretable in 1 case. A metastatic localization of a prostatic carcinoma was found in 1 case. The RPF was idiopahtic in 29 patients. It was due to: abdominal trauma (2 cases), tuberculosis (2 cases), pelvic and abdominal surgery (2 cases), sclerosing pancreatitis (1 case) prostatic tumor (1 case), aortic aneurysm (1 case) and mesenteric panniculitis (1 case). Twenty two patients had an urinary diversion by JJ catheter. No patient required hemodialysis. One patient had been referred to urology for the treatment of a prostatic tumor, 2 patients needed ureterolysis. Left nephrectomy because of pyonephrosis was practiced in one case.A tuberculosis treatment was prescribed for 2 patients. All our patients had a cortecosteroid therapy. We prescribed immunosuppressive therapy for only 7 patients who presented recurrence RPF. The JJ catheter was changed in 14 cases. Twenty patients presented a recurrence of their RPF during the follow up. Nineteen patients never had a recurrece. Eight patients kept a chronic kideny failure. Two patients have died. Because of its insidious nature and the irreversible development towards the chronic renal failure if an early diagnosis was not applied and the treatment did not immediately debuted, FRP requires all attention needed from the clinician. The important relapse rate proposes rethinking the actual therapeutic conduct.

Retroperitoneal fibrosis; a single-centre case experience with literature review

ObjectiveWe present 13 patients with retroperitoneal fibrosis, focusing on clinical features, radiological characteristics, treatments and their outcomes.MethodsRetrospective review of the medical records was performed of all retroperitoneal fibrosis patients diagnosed and treated in our department between 2012 and 2017.ResultsTwelve patients were male, with a median age of 64 years. Eleven patients presented with abdominal pain or back pain or both. Aetiologies varied from idiopathic to malignancy and vasculitis. Twelve patients had PET scans. These showed 18F-fluorodeoxyglucose-avid retroperitoneal soft tissue around the abdominal aorta in the vast majority, with five scans also demonstrating localized or generalized uptake by the aorta. In all cases except one, glucocorticoids were applied as the first-line therapy. Further immunosuppressive therapy was required in 10 cases.ConclusionOur patients were male and older in age compared with the existing literature. PET scans were very helpful in diagnosis of retroperitoneal fibrosis. Rituximab was found to be an effective treatment in six of our patients.

Pancreatic Retroperitoneal Fibrosis Masquerading as a Pancreatic Mass: A Diagnostic Quandary

Retroperitoneal fibrosis (RPF) is a cryptic condition affecting 0.1 per 100,000 patients per year. The pathophysiologic etiology is believed to be autoimmune in nature, resulting in fibrotic tissue deposition within multiple organs. This case highlights an unusual primary pancreatic manifestation of an already exceedingly rare disease. The patient is a 45 year-old male with no significant past medical history who presented with painless jaundice. Computerized tomography revealed a 3 cm pancreatic-head mass encasing the vasculature and ductal system. An endoscopic ultrasound with fine-needle-biopsies (EUS-FNB) of the mass and endoscopic retrograde cholangiopancreatography (ERCP) were performed. The EUS characterized the lesion as a 34 x 28 mm ill-defined heterogeneous mass with multiple collateral blood vessel in the pancreas, suggestive of underlying portal hypertension, correlating with an endoscopic finding of esophageal varices. On ERCP, the common bile duct was dilated to 14mm with a shelf-like distal stricture, treated with a 10 French, 5cm plastic biliary stent. Initial fine needle biopsy results, while inconclusive, could not rule out malignancy or autoimmune pancreatitis. Repeat EUS-FNB with 7 needle passes again returned with non-malignant findings. He subsequently underwent laparoscopic biopsy of the pancreatic mass which revealed sclerosing collagenous fibrosis, displaying lymphoplasmacytic inflammation, suggestive of RPF. Infectious and malignant etiologies for RPF were excluded. He was started on steroids with symptomatic improvement and mass stabilization on subsequent imaging. Through extensive review of the literature, RPF encasing the pancreas without ureteral or periaortic involvement has scarcely been documented. In this case, the mass effect of the fibrosis resulted in biliary obstruction and pre-sinusoidal portal hypertension, and highlights the difficulty in diagnosing an exceedingly rare condition masquerading as pancreatic malignancy. This patient had a protracted course involving multiple sub-specialties, numerous endoscopic procedures, and ultimately a diagnostic surgery. The seemingly elusive diagnosis of RPF conclusively requires histologic certainty prior to treatment. When finally diagnosed, steroids are the mainstay of therapy for RPF.1424_A.tif Figure 1: Dense fibrosis with keloid-type collagen fibers1424_B.tif Figure 2: PET scan showing avid FDG uptake at pancreatic lesion1424_C.tif Figure 3: No Caption available.

Imaging appearance of fibrosing diseases of the retroperitoneum: can a definitive diagnosis be made?

To assess the frequency with which previously reported characteristic findings of retroperitoneal fibrosis (RPF) (a circumferential or almost circumferential peri-aortic mass centered at L4, which does not displace the abdominal aorta or proximal common iliac arteries) are present in patients with RPF, in patients with other fibrosing diseases, and in cancer patients referred to a subspecialty clinic with a suspected diagnosis of RPF, in order to determine whether diagnostic percutaneous biopsy can be avoided in some patients. This HIPAA-compliant Institutional Review Board-approved retrospective study assessed clinical and CT and MR imaging abnormalities on imaging studies in 92 patients referred to a subspecialty clinic with suspected RPF over a 14-year period. Two reviewers, in consensus, determined the frequency of different CT and MRI findings in three groups of patients (Group 1: those with an eventual diagnosis of RPF, Group 2: those with a fibrosing disease associated with vascular or urologic abnormalities, and Group 3: those with cancer). Assessed imaging features included the presence of retroperitoneal masses, whether masses were single or multiple, whether such masses were circumferential or nearly circumferential, whether they displaced the aorta away from the spine (with the degree of such displacement measured), and whether there were abnormalities outside of the peri-aortic region of the retroperitoneum. The frequency with which findings previously reported as characteristic of RPF were present was determined for each of the three groups. Imaging results were correlated with the final diagnoses. Of 68 subjects eventually diagnosed with retroperitoneal fibrosis (RPF) (Group 1), 47 had peri-aortic retroperitoneal masses, 18 of which displaced the aorta anteriorly away from the spine. Of 12 subjects with fibrosing abnormalities related to vascular or urologic disease (Group 2), six had retroperitoneal masses, none of which displaced the aorta away from the spine. Of 12 subjects with malignancies (Group 3), six had peri-aortic retroperitoneal masses only two of whom had aortic displacement. Only 34 of 68 Group 1 subjects had peri-aortic masses characteristic of RPF, compared with six Group 2 subjects and one Group 3 subject. Subjects with characteristic retroperitoneal masses were significantly more likely to have benign disease than cancer (p=0.009). Many patients with RPF do not have characteristic imaging findings. Contrary to prior publications, absence of aortic displacement is not seen in all patients with RPF and is seen in some cancer patients. Nonetheless, when infiltrative peri-aortic retroperitoneal soft tissue that does not displace the aorta is encountered on CT or MRI, RPF can be diagnosed with a high degree of confidence, obviating the need for biopsy.

Ultrasonography for Preoperative Diagnosis of Retroperitoneal Fibrosis.

This study aimed to assess the application of ultrasonography for preoperative diagnosis of retroperitoneal fibrosis (RPF). A total of 51 patients with clinically suspected RPF underwent transabdominal ultrasonography and ultrasound-guided histopathologic biopsy (tAU-UGHB). Retroperitoneal fibrosis was diagnosed in 35 patients; of these, 31 cases (88.6%) received a diagnosis using tAU-UGHB. The ultrasonographic images mainly showed solid, irregular isoechoic masses, which were present behind the retroperitoneum and surrounded the abdominal aorta, inferior vena cava, and ureters and had clear borders with the encapsulated structures. Compared with other retroperitoneal lesions, RPF lesions were mainly located below the initial level of the renal artery, and their internal flow signals were not rich (P < 0.05). Retroperitoneal fibrosis had characteristic ultrasonographic features, and color Doppler ultrasound-guided biopsy could be positioned accurately, safely, and efficiently; therefore, tAU-UGHB can be used as an effective modality for preoperative diagnosis of RPF.

Retroperitoneal fibrosis: about 12 cases

La fibrose rétropéritonéale (FRP) est une maladie rare. Elle se caractérise par la transformation progressive du tissu adipeux rétopéritonéal en une masse fibreuse qui enserre l'aorte, la veine cave inférieure et les voies urinaires responsable d'une altération progressive de la fonction rénale. Le mode habituel de présentation de cette maladie comporte l'association de douleurs lombaires, d'une insuffisance rénale, et d'un syndrome inflammatoire biologique. Nous rapportons 12 cas de fibrose rétropéritonéale dont nous précisons les particularités cliniques, radiologiques et thérapeutiques. Il s'agit d'une étude rétrospective portant sur douze cas de fibrose rétropéritonéale colligés au service d'urologie au CHU Hassan II de Fès durant une période de 9 ans (2005-2013). Il s'agissait de dix hommes et deux femmes. La symptomatologie clinique était très variable, dominée par la douleur lombaire qui était présente chez tous les malades et une hydrocèle chez un patient. Les explorations biologiques avaient montré une insuffisance rénale chez tous les malades et un syndrome inflammatoire chez dix patients. Le diagnostic de la maladie était suspecté dans tous les cas sur les données de l'échographie qui a montré une obstruction de la voie excrétrice supérieure sans obstacle visible chez tous les malades, et confirmé par la TDM abdominale sans injection du produit de contraste qui objectivait une lésion tissulaire rétropéritonéale engainant les vaisseaux et les voies urinaires. Dans notre série, la fibrose rétropéritonéale était idiopathique dans neuf cas. Elle était péri anévrysmale chez deux malades, et post radiothérapie chez un malade. Tous nos patients avaient bénéficié d'un drainage urinaire par sonde urétérale double J. Sept malades avaient reçu une corticothérapie. Une amélioration clinique et biologique, avec disparition de la douleur et amélioration de l'état général, a été observée chez 6 patients. A travers cette étude nous avons confirmé la rareté de la fibrose rétropéritonéale, la difficulté de son diagnostic, la fréquence de la douleur, du syndrome inflammatoire et de l'insuffisance rénale. La TDM abdominale sans injection du produit de contraste confirme le diagnostic. Le drainage urinaire est indispensable dans la plupart des cas et le suivi régulier des malades est nécessaire.

Retroperitoneal Fibrosis: Case Series of 20 Patients

Retroperitoneal fibrosis (RPF) is a rare disease. The objective of this paper is to study the prevalence, clinical, biological and radiological aspects of the RPFand to specify its therapeutic modalities. This study provides descriptive evidence for a series of 20 patients with RPF. We conducted a retrospective study of cases of RPF diagnosed in the Urology and Internal Medicine departments of the Ibn Sina Military Hospital in Marrakech over a five-year period from July 2010 to December 2015. The positive diagnosis of RPF was retained either on the basis of a histological analysis from a biopsy fragment or, in the absence of histological evidence, by the detection on a scan of an infiltrate or sleeve Regular and homogeneous tissue density, perivascular topography, surrounding the abdominal aorta, more or less extended to the iliac vessels and able to take the contrast after injection. They were 15 men and 5 women with an average age of 53 years with extremes of 41 to 74 years. Almost all patients had mainly lumbar and abdominal pain. An inflammatory syndrome existed in all cases and renal insufficiency in 15 cases. Radiological investigations showed unilateral or bilateral hydronephrosis in 16 cases. RPF was idiopathic in 14 patients. Secondary forms were present in 6 patients (three atheromatous diseases, one gastric tumor, one case associated with Riedel's thyroiditis and one case of systemic fibrosis associating RPF, Riedel's thyroiditis and liver fibrosis). The treatment consisted of corticosteroids in 19 cases, surgery in 17 cases associated with corticosteroid therapy or even immunosuppressant in the case of steroid-dependence. Two deaths were observed in this series; one of these two patients had developed cervix carcinoma with pleural and peritoneal metastases after 5 years of immunosuppressive therapy. The second died due to the complications of his gastric neoplasm. The general characteristics of patients included are similar to the other series of the literature. The frequency of secondary forms seems to be underestimated, hence the value of a rigorous etiological investigation. The therapeutic strategy and the use of the cortisone saving treatments should be specified by randomized therapeutic trials. The prognosis is generally good; however, the possibility of recurrences, the incidence of which is greater during the first five years, requires prolonged clinical, biological and radiological monitoring.

Retroperitoneal fibrosis: retrospective descriptive study on clinical features and management.

IntroductionRetroperitoneal fibrosis (RPF) is a rare condition characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encases the ureters or abdominal organs. This study describes the clinical characteristics, diagnostic methods, and treatments and their effects on renal function.MethodsWe conducted a retrospective analysis of patients diagnosed with RPF at Maisonneuve-Rosemont Hospital.ResultsWe identified 17 patients with RPF between 1998 and 2013. Eight patients were females (47%), and the mean age was 62±18 years. Eleven patients were idiopathic. Back pain was the most common symptom. All diagnoses were made based on the finding of a retroperitoneal mass on the computed tomography scan. Three patients had histological diagnosis of RPF and seven patients had unspecific changes on their biopsy. Twelve patients needed double-J stents, three patients had a temporary percutaneous nephrostomy, two patients had to have a nephrectomy for refractory ureteral obstruction, and one patient required hemodialysis. Ten patients with idiopathic RPF received medical treatment. In the treated group, only two patients had complete remission of the disease and five patients had improvement of their lesions. There were no deteriorations and only one relapse. Seven patients did not receive any treatment; two of them achieved complete remission, one of them deteriorated, and two of them had no changes.ConclusionMost of our cases of RPF were idiopathic. Almost all treated patients received prednisone and seemed to respond, at least partially. There was a lot of heterogeneity in patient management, which makes it difficult to compare treatment effects. However, treated patients seemed to have more favorable outcomes than those who were not.

A Case of Acute Renal Failure Associated with IgG4-Related Disease Presenting Both Tubulointerstitial Nephritis and Retroperitoneal Fibrosis

We report a case of IgG4-related disease presenting both tubulointerstitial nephritis and retroperitoneal fibrosis causing acute renal failure in a 63-year-old male. He was admitted to our hospital because of acute renal failure requiring emergent hemodialysis. Computed tomography showed a soft-tissue density mass with an irregular border in the retroperitoneum. The mass involved bilateral ureters and had caused acute renal failure by bilateral hydronephrosis. Because of a history of uveitis and high IgG4 levels, we considered a diagnosis of retroperitoneal fibrosis, IgG4-related disease. Kidney biopsy revealed IgG4-related kidney disease with interstitial nephritis. After relief of urinary obstruction by inserting ureteral catheters into the bilateral ureters, renal function recovered.

A rare cause of benign retroperitoneal mass mimicking adnexial malignancy in a postmenopausal woman: retroperitoneal fibroma

© 2014 Akpak et al; licensee Herbert Publications Ltd. This is an Open Access article distributed under the terms of Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0). This permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Correspondence We read with great interest the article by G Bassi et al., [1] in which the authors presented a case named “An incidental retroperitoneal solitary fibrous tumor”. They mentioned about the clinicopathological characteristics of retroperitoneal solitary fibrous tumor and analyse the diagnosis of retroperitoneal fibrosis. Unfortunately, there are only a few cases of retroperitoneal fibroma reported in the literature. When we search “retroperitoneal fibroma” in PubMed we found only five literatures in english about this rare condition. The total number of reported cases has been fewer than thirteen. Therefore there is limited information about the retroperitoneal fibromas. Fibromas are stromal tumours composed of spindle, oval or round collagen-producing cells that resemble fibroblasts [2]. Herein, we present a patient with pelvic retroperitoneal fibroma which was confirmed histopathologically following surgery. Imaging findings of the tumor mimicked an adnexial mass preoperatively. A 54-year-old postmenopausal multiparous woman presented us with a complaint of lower abdominal pain, dyspareunia and urinary retention for 3 years. The patient had a history of total abdominal hysterectomy operation 16 years ago. A solid, movable mass was noted in the middle and left lower quadrant of abdomen by abdominopelvic examination. Transvaginal ultrasonography revealed a smooth surfaced solid tumoral mass with calcific degeneration measuring 86x82x88 mm located in the middle region near the vagina cuff, occupying the left adnexial area (Figure 1A). We did not see ureteral obstruction or ectasia of renal pelvis by abdominal ultrasonography. Laboratory examinations including tumor markers (AFP, CA 125, CEA, CA199, CA 15-3) were unremarkable. Pelvic magnetic resonance imaging demonstrated about 8x8 cm in size pelvic solid mass with heterogeneous internal structure, showing no significant enhancement, located in the middle and left adnexial region (Figures 1B and 1C). However, a malignant lesion could not be ruled out. One month after the initial examination, laparotomy *Correspondence: yasamaster@gmail.com

Retroperitoneal Fibrosis: Role of Imaging in Diagnosis and Follow-up

Retroperitoneal fibrosis (RPF) encompasses a range of diseases characterized by proliferation of aberrant fibroinflammatory tissue, which usually surrounds the infrarenal portion of the abdominal aorta, inferior vena cava, and iliac vessels. This process may extend to neighboring structures, frequently entrapping and obstructing the ureters and eventually leading to renal failure. The idiopathic form of RPF accounts for more than two-thirds of cases; the rest are secondary to factors such as drug use, malignancies, or infections. If promptly diagnosed and treated, idiopathic and most other benign forms of RPF have a good prognosis. In contrast, malignant RPF, which accounts for up to 10% of cases, has a poor prognosis. Therefore, the most important diagnostic challenge is differentiation of benign from malignant RPF. Imaging plays a key role in diagnosis of RPF. Cross-sectional imaging studies, particularly multidetector computed tomography (CT) and magnetic resonance (MR) imaging, are considered the imaging modalities of choice. Imaging features may help distinguish between benign and malignant RPF, but in some cases histopathologic examination of the retroperitoneal tissue is needed for definitive diagnosis. CT and MR imaging, along with positron emission tomography with fluorine 18 fluorodeoxyglucose, also play an important role in management and follow-up of idiopathic RPF.

The Role of Multidetector CT in the Diagnosis of Retroperitoneal Fibrosis: Report of a Case

Herein, we report a 40-year old man who presented with flank and abdominal pain with dilatation of the bilateral pyelocalyceal system detected in ultrasonography. Computed Tomography (CT) scan showed a soft tissue mass at the level of the fourth and fifth lumbar vertebrae in the retroperitoneal region. There were no blood flow signals in 64-slice multidetector CT (MDCT) which confirms the Retroperitoneal Fibrosis (RPF). Pathological examination showed infiltration of plasma cells, macrophages, lymphocytes and eosinophils accompanied by fibrosis, which is consistent with idiopathic RPF. In conclusion, 64-slice MDCT imaging is useful in the diagnosis of RPF.

Retroperitoneal Fibrosis in a Patient with Gastric Cancer Manifested by Lower Extremity Edema and Hydrocele

Herein we report a 57-year-old man with lower extremity edema and swelling in the scrotum who was found to have a periaortic soft tissue mass and hydronephrosis by computed tomography. With the most plausible diagnosis of retroperitoneal fibrosis, corticosteroid therapy was initiated; however, it did not improve his symptoms. Upper gastroscopy performed on day 20 post admission showed ulcerative regions with an irregular border and fusion of thickened rugae at the gastric angle; the diagnosis of gastric adenocarcinoma was confirmed histologically. It is important to always be aware of unrecognized malignancies that are accompanied by retroperitoneal fibrosis.

Idiopathic Retroperitoneal Fibrosis

Retroperitoneal fibrosis (RPF) is a rare disorder of unknown etiology. Its incidence is unknown, and the insidious and nonspecific nature of symptoms may contribute to considerable diagnostic delay. We conducted the current study to assess the incidence and clinicoradiologic characteristics of idiopathic RPF. For this, we evaluated prospectively 53 consecutive patients with a diagnosis of idiopathic RPF at our tertiary care referral center from April 1998 through January 2008.Calculated annual incidence of RPF was 1.3/100,000 inhabitants. Mean age was 64 +/- 11.1 (SD) yr; male-female ratio was 3.3:1.0. Median duration of symptoms was 6.0 mo (IQR 3.0-12.0). Abdominal, flank, and/or back pain and discomfort were the major symptoms, with visual analogue scale scores of 49 +/- 27.2 mm and 43 +/- 29.4 mm for pain and discomfort, respectively. Female patients had higher erythrocyte sedimentation rate (ESR), higher white blood cell count, and lower hemoglobin content than male patients at presentation. Computed tomography-documented maximal mass thickness amounted to 35 +/- 16.6 mm; craniocaudal length amounted to 137 +/- 48.8 mm. RPF mass extension up to or above the level of the renal vessels was noted in 3 patients (6%). Six patients (11%) presented with atypical RPF localization and/or bulky mass. Localized lymphadenopathy adjacent to the RPF mass was observed frequently (25%). Patients with hydronephrosis (56%) presented earlier than patients without hydronephrosis, with higher creatinine and greater mass thickness but similar pain severity. Patients were typically at high cardiovascular risk with increased-often aneurysmal-infrarenal aortic diameter (25.0 mm, IQR 22.0-30.0). RPF mass distribution was similar in patients with or without aneurysmal dilation. Occupational asbestos exposure (20%) and asbestos-related pleural changes (17%) were frequent among males. Previous or concurrent chronic inflammatory disease and/or autoimmune disease was noted in 8 patients (15%). Multivariate analysis revealed an independent association of ESR values with severity of pain and discomfort. Smoking was independently associated with infrarenal aortic diameter.In summary, annual RPF incidence is higher than previously assumed. Age at diagnosis and male-female ratio seem to have changed over time. RPF typically affects patients at high cardiovascular risk, including increased aortic diameter. Clinical presentation is influenced by sex, severity of inflammation and presence of hydronephrosis. Prolonged asbestos exposure and asbestos-related pleural changes were frequent among males. Localized lymphadenopathy adjacent to the RPF mass occurs frequently and should not confuse RPF diagnosis.

Leuco-Thrombocytopenia Due to Splenomegaly in a Patient Affected by Retroperitoneal Fibrosis

Idiopathic retroperitoneal fibrosis (RPF) is a rare clinical entity characterized by the progressive proliferation of connective tissue in the retroperitoneum. The chronic inflammatory process can entrap the retroperitoneal structures, mainly the ureters and the great vessels. Although the causes are unknown, immunogenetic factors and immunopathologic/autoimmune mechanisms are probably involved. The pathogenesis appears to be related to IgG4 autoimmune mechanisms (“hyper-IgG4 disease”). Surgical treatments are frequently performed such as ureterolysis and aneurysm repair; steroids and immunosuppressive agents are also used. In January 2008 a 62-year-old Caucasian man was referred to our institution for mild leucopenia and progressive thrombocytopenia. In 1999 he had a diagnosis of RPF treated surigically and complicated by chronic renal failure. He did not undergo regular follow-ups during the following years. When he came to our attention his peripheral blood count showed: Hb 14.5 g/dl, WBC 3.800/uL (N 60%, L 28%, M 8%), Plt 79.000/uL, confirmed by numerous measurements. He did not present signs or symptoms of cutaneous or mucosal bleeding and he did not report any recurrence of infectious episodes. At physical examination the spleen was palpable at 5 cm from the ribs, neither liver enlargement nor superficial lymphoadenopathies were noted. A peripheral blood (PB) smear showed only anisocytosis of red blood cells. To exclude an underlying hematologic disorder a bone marrow (BM) aspiration and a BM trephine biopsy were performed, both demonstrating a regular representation of the three hematopoietic series without any pathologic patterns. Cytogenetic examination revealed normal karyotype and molecular biology analyses resulted negative for Bcr/Abl rearrangement and for mutation V617F of JAK2 gene. Routine laboratory tests including liver function, LDH, Beta2 microglobulin serum levels, autoimmune tests, PB mononuclear cell immunophenotyping, viral sierology resulted within normal range. Abdominal ultrasound showed about 95 cm2 homogeneous splenomegaly and revealed an hyperechogenic rounded mass measuring 5–6 cm that completely embraced the splenic hilum. Splenic vein at the source was not visible and splenic flow was not measurable because of the hilum compression. Splenic vein in the retropancreatic site was measurable only partially: it presented thin caliber with normal directed blood flow inside. The abdominal CT exam confirmed the presence of a 5.6×7 cm solid tissue mass embracing the pancreatic tail and the splenic vessels and it extended across the spleno-pancreatic ligament. Moreover, the mass also involved the upper part of the left adrenal gland expanding until peri-renal space. Radiologically the mass was considered as RPF. In this case the retroperitoneal fibrotic tissue compresses the splenic hilum reducing the blood flow through the spleen thus causing congestive splenomegaly. The increase size of the spleen is responsible for thrombocytopenia as well as for leucopenia. The mechanism underlying splenomegaly-related thrombocytopenia is an induction of a reversible pooling of up to 90 percent of total body platelets. Both platelet production and the survival of platelets within the spleen are normal. Pooling is the major factor responsible for thrombocytopenia in uncomplicated splenomegaly. In this patient no hematologic specific treatment is indicated since neither thrombocytopenia nor leucopenia have clinical relevance. The aim of the therapy should be to reduce the progression of the fibrotic tissue to prevent further organ-related damages.