![]() reported that 18F-FDG PET/CT could accurately distinguish active disease from inactive disease (93.9%). assessed the role of MRI in RPF which found that the apparent diffusion coefficient of inactive RPF was higher than that of active RPF or malignant RPF, and diffusion-weighted imaging may aid in the differentiation of inactive RPF malignant neoplasms. CT scan is usually the modality of choice to visualize the location and extent of fibrosis and possible etiology. A differential diagnosis must be made between iRPF and other diseases such as malignancies, infectious diseases, systemic problems, etc. This primarily included ultrasound, CT, MRI, and PET, and in which this disease often manifested as a homogeneous, well-defined plaque enveloping the retroperitoneal organs. Imaging examinations have played an essential role in the diagnosis and follow-up of RPF. Therefore, this case was probably not related to RPF IgG4. Furthermore, IHC of his biopsied fibrous retroperitoneum showed no IgG4-related pathological features. His serum IgE was also at a normal level of 25.8 IU/ml. For this patient, her total IgG was 27.4 g/L, with elevations of IgG1 and IgG2, while IgG3 and IgG4 were normal (Table 1). Liao and colleagues compared the differences between IgG4-RPF and iRPF in a Chinese population that found elevated serum IgE concentration and tissue eosinophilia in the IgG4-RPF subgroup. However, the specificity of serum IgG4 level is limited. Recent studies have proposed that iRPF belongs to IgG4-related diseases (IgG4-RD) which manifests as significantly elevated serum IgG4 and mass-like lesions that could easily be misdiagnosed as tumors. Studies have reported that 10–20% of RPF cases are ANA positive, and a higher frequency of ANA positivity (30–40%) has been observed in iRPF. Laboratory findings of elevated ESR and CRP inflammatory markers and reduced Hb often herald the active state of RPF. Urological manifestations included ureteral obstruction, hypertension secondary to renal artery stenosis, renal failure or insufficiency, and nonfunctioning kidneys are common with ureteral involvement. It is characterized by fibro-inflammatory tissue surrounding the abdominal aorta and compressing the retroperitoneal organs, in particular the ureters. RPF commonly occurs in adults between the ages of 40 and 60, particularly in men (incidents occurring almost two to three times more than in women). While the rest is secondary to certain medications, like inflammatory disorders, malignant diseases, radiation therapy and abdominal surgery, etc. About 70% of RPF cases are idiopathic with no clear etiology. RPF is clinically rare with an age-standardized incidence of 0.1 cases/100,000 people per year. At the time of admission, the patient has a 13-year history of iRPF. This case report describes an iRPF patient who was later diagnosed with endometrial cancer.
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