Idiopathic retroperitoneal cyst in an adult male.
Ontology highlight
ABSTRACT:
SUBMITTER: Jha AK
PROVIDER: S-EPMC5532897 | biostudies-other | 2017 Jul-Sep
REPOSITORIES: biostudies-other
Similar Datasets
Project description:Retroperitoneal fibrosis (RPF) is a very rare disease that is even more rare in the pediatric population. Even less common are idiopathic pediatric cases of retroperitoneal fibrosis, with a majority of reported pediatric retroperitoneal fibrosis cases being associated with secondary etiologies. We present an 11-year-old Caucasian female that was diagnosed with idiopathic retroperitoneal fibrosis using magnetic resonance imaging (MRI) to work-up severe bilateral hydronephrosis that was identified with retroperitoneal ultrasound. Given the uncommon nature of this serious condition, we present this case to illustrate the importance for physicians to include retroperitoneal fibrosis in the differential diagnosis of a pediatric patient presenting with obstructive urinary findings and understand the utility of using MRI to diagnosis and monitor this disease.
| S-EPMC6357545 | biostudies-literature
Project description:Retroperitoneal fibrosis (RPF) is an uncommon condition characterized by inflammation and fibrosis in the retroperitoneal space. More than two-thirds of RPF are idiopathic, with the remaining stemed from a variety of secondary causes. It was suggested that IgG4-related RPF is a secondary form of RPF. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-related RPF and IRPF in a large Chinese cohort. We retrospectively reviewed the medical records of 132 RPF patients diagnosed at Peking University People's Hospital between March 2010 and March 2018. Among the 132 patients, the mean age at disease onset was 54.8 years. IgG4-related RPF group showed greater male predominance compared to IRPF group. IgG4-related RPF patients showed a longer interval between symptom onset and diagnosis, and allergic diseases were more common in this group. Sixty-four patients (48.4%) had lower back pain, which was more common in IRPF group than that in IgG4-related RPF patients. In terms of organ involvement, although 42 of 47 patients (89.3%) with IgG4-related RPF had other organ involvement, there were no patients in the IRPF group with other organ involvement. In addition, the serum IgG4 level, elevated eosinophils counts and IgE level were significantly higher in IgG4-related RPF patients. We described the demographic, clinical and laboratory differences between IgG4-related RPF and IRPF patients, indicating their potential differences in pathogenesis, which was of great importance to diagnose and manage the two phenotypes.
| S-EPMC7891782 | biostudies-literature
Project description:IntroductionThe retroperitoneum is the anatomical compartment positioned behind the peritoneal cavity. It is separated into three primary spaces: the anterior pararenal, perirenal, and posterior pararenal spaces. Retroperitoneal cystic mass is a rare surgical problem that is often wrongly identified before surgery.Case presentationWe report a case of a 27-year-old female presenting with abdominal swelling and pain starting from 9 months. An abdominal computed tomography scan showed a right adnexal mass with a high probability of a serous ovarian. The patient was diagnosed with ovarian tumors before surgery, but it was identified with a retroperitoneal cyst during surgery.DiscussionA retroperitoneal cyst's clinical signs and symptoms vary, and the diagnosis can often be challenging. Computed tomography scans are appropriate for assessing retroperitoneal pathology because they produce separate sectional images and couldn't find the correct diagnosis in previous cases.ConclusionThis paper shows the rare case of primary retroperitoneal lesions, which can be hard to diagnose before surgery, even though medical imaging has come a long way.
| S-EPMC9403092 | biostudies-literature
Project description:Retroperitoneal cystic mass is a rare surgical condition that is often misdiagnosed preoperatively. Here, we report a case of a 56-year-old woman who presented with abdominal swelling for a 1-year duration, which was associated with lower abdominal pain for 6 months. Her abdominal radiograph showed a huge radiopaque lesion, and contrast-enhanced computed tomography scan of the abdomen reported it as a left ovarian serous cystadenoma causing local mass effect to the left ureter leading to mild left hydronephrosis. She underwent exploratory laparotomy and noted there was a huge retroperitoneal cystic mass. The histopathological assessment finding was consistent with a benign retroperitoneal cyst. This case report aims to share the rare case of primary retroperitoneal lesions, which can cause a diagnostic challenge preoperatively to all clinicians despite advanced achievement in medical imaging.
| S-EPMC7642373 | biostudies-literature
Project description:Retroperitoneal fibrosis (RPF) refers to a fibro-inflammatory lesion in the retroperitoneum, often anterolateral to the aorta. Most cases are due to IgG4-related disease (IgG4-RD) or are idiopathic. RPF can lead to severe morbidity. Treatment strategies remain poorly-defined. We evaluated the efficacy and safety of rituximab (RTX) for idiopathic or IgG4-related RPF.We retrospectively reviewed the records of patients who had RPF treated with RTX. Treatment response was determined by assessing changes in both clinical features, including symptoms and laboratory measurements, as well as in the radiographic dimensions of the lesion.Twenty-six patients with IgG4-related (n?=?19) or idiopathic RPF (n?=?7) were identified. Patients without histopathological evidence of IgG4-RD on either retroperitoneal biopsies or sampling of extra-retroperitoneal organs were considered to have idiopathic RPF. Of the 26 patients, 19 (73%) received RTX without additional glucocorticoids. All 19 patients who presented with pain reported symptomatic improvement following RTX. Among 25 patients with follow-up imaging, 22 (88%) had radiologic improvement. Among 10 patients with ureteral stents and/or percutaneous nephrostomy tubes, 4 (40%) underwent successful stent or tube removal. Responses to treatment were similar among those treated with RTX monotherapy and those treated with RTX and glucocorticoids. RTX was generally well tolerated, but 3 (12%) patients experienced severe infections.In this study, RTX for RPF led to resolution of symptoms in all patients and radiographic improvement in the majority. Prospective studies of RTX for RPF are indicated.
| S-EPMC6211888 | biostudies-other
Project description:Bronchogenic cysts are a congenital primitive foregut-derived developmental malformation, generally occurring in the posterior mediastinum. Their development in the retroperitoneum is extremely rare. Imaging techniques, such as multidetector computed tomography (MDCT), are typically effective in the detection of these lesions. Here, we describe three cases of a retroperitoneal cyst presenting as a para-adrenal mass. Only one boy presented with abdominal pain, and the other two showed no clinical symptoms. Endocrinological evaluation of all three cases was performed, and no adrenal hormone secretion was detected. All three cases were misdiagnosed preoperatively. Each patient underwent surgery, and one symptomatic patient became asymptomatic after surgery. Pathologic examination confirmed all three masses as bronchogenic cysts. The three cases showed some similar MDCT imaging features, including a complete adrenal structure, a cystic or solid mass in the adrenal region, and no obvious enhancement. Therefore, bronchogenic cysts should be considered in the differential diagnosis of retroperitoneal masses, even though accurate preoperative diagnosis remains difficult. A contrast-enhanced MDCT scan may be useful for differentiating hyper-attenuated cysts from other soft tissue masses.
| S-EPMC8785309 | biostudies-literature
Project description:BackgroundWe previously identified a MUC5B gene promoter-variant that is a risk allele for sporadic and familial Idiopathic Pulmonary Fibrosis/Usual Interstitial Pneumonia (IPF/UIP). This allele was strongly associated with increased MUC5B gene expression in lung tissue from unaffected subjects. Despite the strong association of this airway epithelial marker with disease, little is known of mucin expressing structures or of airway involvement in IPF/UIP.MethodsImmunofluorescence was used to subtype mucus cells according to MUC5B and MUC5AC expression and to identify ciliated, basal, and alveolar type II (ATII) cells in tissue sections from control and IPF/UIP subjects. Staining patterns were quantified for distal airways (Control and IPF/UIP) and in honeycomb cysts (HC).ResultsMUC5B-expressing cells (EC) were detected in the majority of control distal airways. MUC5AC-EC were identified in half of these airways and only in airways that contained MUC5B-EC. The frequency of MUC5B+ and MUC5AC+ distal airways was increased in IPF/UIP subjects. MUC5B-EC were the dominant mucus cell type in the HC epithelium. The distal airway epithelium from control and IPF/UIP subjects and HC was populated by basal and ciliated cells. Most honeycombing regions were distinct from ATII hyperplasic regions. ATII cells were undetectable in the overwhelming majority of HC.ConclusionsThe distal airway contains a pseudostratified mucocilary epithelium that is defined by basal epithelial cells and mucus cells that express MUC5B predominantly. These data suggest that the HC is derived from the distal airway.
| S-EPMC3603941 | biostudies-literature
Project description:ObjectiveIn order to determine whether the immune balance of T helper 17(Th17)/regulatory T(Treg) is related to the pathogenesis of idiopathic retroperitoneal fibrosis (IRPF), we analyzed the differences in peripheral blood lymphocytes, CD4+T cell subsets and cytokines between patients with IRPF and healthy people to clarify the CD4+T cell subsets, especially Treg cell subsets, and the role of cytokines in the pathogenesis of IRPF.MethodsThis study included 22 patients with IRPF, 36 patients with IgG4-related diseases (IgG4-RD) without retroperitoneal fibrosis (RPF), and 28 healthy controls. The absolute numbers and percentage of peripheral blood lymphocyte subsets and CD4+T cell subsets in each group were detected by flow cytometry, and the serum cytokine level was detected by flow cytometric bead array (CBA).ResultsCompared with the healthy group, the absolute value of B cells in peripheral blood of IRPF patients was significantly decreased, and T, natural killer (NK), CD4+ and CD8+ were not significantly abnormal. The absolute numbers of Th2 cells were lower than healthy group(p=0.043). In particular, the absolute numbers of Treg cells were significantly lower than healthy group(p<0.001), while the absolute numbers of Th17 cells increased(p=0.682). Th17/Treg was significantly higher than healthy group (p< 0.001). Cytokine analysis showed that the level of interleukin (IL)-4 in IRPF patients was higher than healthy group(p=0.011), IL-6, IL-10, IL-17, TNF-α and IFN-γ were significantly higher than healthy group (all p<0.001). Receiver operating characteristic (ROC) curves showed that IL-10 and TNF-α could distinguish bilateral ureteral dilatation in IRPF patients, with areas under the ROC curve (AUCs) of 0.813 (95% CI:0.607-1.000, p=0.026) and 0.950 (95% CI:0.856-1.000, p=0.001), respectively. IL-6 could distinguish bilateral ureteral obstruction, with an AUC of 0.861 (95% CI: 0.682-1.000, p=0.015).ConclusionsOur study showed that IRPF patients had reduced Treg cells and indeed had Th17/Treg imbalance, which may be related to the pathogenesis of the disease. The levels of IL-6, IL-10 and TNF-α appear to be associated with the progression of IRPF.
| S-EPMC9744965 | biostudies-literature
Project description:SummaryA 44-year-old athletic man presented in 2009 with severe low back pain. Dual-energy x-ray absorptiometry revealed severe osteoporosis; serum testosterone was 189 ng/dL while serum estradiol (E2) measured by liquid chromatography/mass spectrometry was 8 pg/mL. DNA was extracted and sequenced from a blood sample from the patient since his maternal first cousin also had low bone mass and both patients were screened for aromatase dysfunction by PCR analysis for the CYP19A1 gene, which encodes aromatase. No known pathologic mutations were observed in the coding exons, but novel single nucleotide polymorphisms were detected both in the proband and in his cousin. Treatment with topical testosterone started in August 2010. Over the next 8 years, testosterone dosage was varied and switched from topical gel to injections and maintained on depo-injections of testosterone at about 60 mg once per week. Re-examination in March 2012 included a brain MRI to exclude pituitary lesions; hyperparathyroidism was ruled out (normal serum parathyroid hormone, calcium, and calcium to phosphorous ratio) and celiac disease was excluded (negative transglutaminase antibodies). Follow-up in October 2018 showed improved bone mineral density of the lumbar spine by 29% and of the left femoral hip by 15% compared to baseline measurements. This reveals the importance of measuring serum E2 for making the correct diagnosis, as well as for monitoring a therapeutic effect. Herein, we propose treatment of male osteoporosis where serum E2 levels are below about 20 pg/mL with testosterone to reverse osteoporosis.Learning pointsEstrogen deficiency in the diagnosis of male idiopathic osteoporosis. Importance of serum estradiol in male osteoporosis. Role of polymorphisms in aromatase gene on bone health. Reversal of osteoporosis. Tailored testosterone treatment for bone health.
| S-EPMC10241241 | biostudies-literature
Project description:Video 1Endoscopic full thickness resection with retroperitoneal dissection for duodenal myogenic cyst with adjustable traction from an independently controlled snare.
| S-EPMC9832242 | biostudies-literature