An asymptomatic 75-year-old man who underwent transverse colon cancer surgery two years previously presented with retroperitoneal fibrosis (RPF) around the ventral sacral and right external iliac artery and vein on abdominal computed tomography (CT) during a routine surveillance visit. We assumed cancer recurrence or immunoglobulin G4 (IgG4)-related disease (RD), but although generic tumor markers and IgG4 levels were normal, soluble interleukin 2 receptor (sIL-2R) was elevated at 569 U/mL (reference: 122-496 U/mL). No diagnosis was made at this time, and the patient was followed up. He subsequently developed edema of both lower extremities. Abdominal enhanced CT showed an enlarged RPF without invasion of surrounding organs and with a delayed contrast effect, and positron emission tomography-CT showed fluorodeoxyglucose accumulation in the same area but a lower standardized uptake value (SUV) than at the time of transverse colon cancer diagnosis. Although generic tumor markers and IgG4 levels remained within the reference range, sIL-2R was further elevated to 1100 U/mL. An open biopsy and histopathology showed a high IgG4/IgG-positive cell ratio and infiltration of IgG4-positive plasma cells. The patient was finally diagnosed with IgG4-RD RPF. In cases of RPF after colorectal cancer surgery, the combined findings of elevated sIL-2R, lack of infiltration into surrounding organs, and lower SUV values ​​than at the cancer site could provide useful information to aid the diagnosis of IgG4-RD RPF.

Immunoglobulin G4 (IgG4)-related disease has the potential to impact any part of the body, including the walls of large- and medium-sized blood vessels and the ureters. While histopathologic examination is currently the standard method for identifying organ involvement and diagnosing IgG4-related disease (IgG4-RD), obtaining biopsy or surgical samples from vessel or ureteral walls is challenging. Given that patients may display only mild symptoms, non-invasive imaging plays a vital role in both diagnosing and managing IgG4-related diseases. Multidetector CT scans are valuable in establishing the primary diagnosis, identifying anatomical landmarks and assessing their relationships. Involvement of the genitourinary organs, such as the ureter, bladder, urethra, and male and female reproductive organs in IgG4-RD, is infrequent when compared to kidney involvement. The imaging findings may include the presence of a localised mass within or surrounding the affected organ or a generalised enlargement of the organ. This report includes cross-sectional images of five cases of IgG4-RD involving large- and medium-sized blood vessels (the aorta and superior mesenteric artery) and the ureters.
UNASSIGNED: This case series provides insight into the various imaging appearances of IgG4-related retroperitoneal organ involvement and helps differentiate it radiologically from retroperitoneal fibrosis.

A paraganglioma is a neuroendocrine tumor that may secrete catecholamines and present with symptoms of sympathetic overload such as hypertension and diaphoresis. It is important that paragangliomas are identified, as they must often be treated by surgical excision. IgG4-related retroperitoneal fibrosis (IgG4-RPF) is a systemic inflammatory disease that results in the infiltration of IgG4-positive plasma cells in the retroperitoneum. Such fibrosis may adversely affect nearby organs and tissues. Here, we describe a case of combined paraganglioma and IgG4-RPF in a 47-year-old female patient. This case demonstrates the deleterious effect of these two conditions when they occur simultaneously.

Retroperitoneal fibrosis (RPF) is a rare condition characterized by the presence of fibro-inflammatory tissue surrounding the abdominal aorta and iliac arteries, often leading to the involvement of adjacent organs such as the ureters and inferior vena cava. We present a case report of a 56-year-old Caucasian woman with a complex medical history, including Hodgkin\'s lymphoma treated with chemotherapy and radiotherapy (31 years before), a significant smoking history, and a current presentation of acute kidney injury with oliguria, edema, and hypertension. Initial diagnostic considerations included rapidly progressive glomerulonephritis, supported by clinical and imaging findings. However, the absence of specific autoantibodies and the presence of bilateral calyx-pelvic dilation raised questions regarding alternative diagnoses. Imaging studies, including CT, contrast-enhanced CT, and subsequent MRI, revealed periaortic and paracaval adipose tissue thickening suggestive of periaortitis, leading to a diagnosis of retroperitoneal fibrosis. The multifactorial etiology, including previous radiation therapy, lymphoma history, and significant smoking, posed challenges in establishing a definitive causal link. Despite extensive investigations, including laboratory tests and imaging modalities, no single etiological factor could be conclusively identified. This case underscores the diagnostic complexity of RPF, especially in the presence of multiple potential risk factors, and highlights the importance of considering this condition in the differential diagnosis of patients presenting with renal dysfunction and obstructive uropathy.

Retroperitoneal fibrosis (RPF), also referred to as Ormond\'s disease, is a rare fibroinflammatory condition characterized by abnormal fibrous tissue deposition in the retroperitoneal space, which traditionally presents with ureteral obstruction. Nonetheless, our case report showcases an exceptional instance involving a 70-year-old female patient who presented with symptoms suggestive of colonic obstruction, an unusual presentation that is not commonly associated with RPF. Although RPF has established associations with autoimmune conditions such as immunoglobulin G4-related disease and systemic lupus erythematosus, its connection to colonic obstruction remains undocumented in the medical literature. Our patient is a 70-year-old female who presented with constipation, anemia, and fecal occult blood. Her past medical history included a hysterectomy due to fibroids, right breast lumpectomy, type 2 diabetes mellitus, subclinical hyperthyroidism, hypertension, and obesity. Upon physical examination, the patient\'s abdomen appeared protuberant but was non-tender to palpation. Bowel sounds were normal, and there was no distension. Notably, there was no tenderness in the right or left costovertebral angles, nor was there any guarding. Workup with colonoscopy could not be completed due to the inability to pass a colonoscope beyond the rectosigmoid junction. Further workup with barium enema confirmed an apple core lesion seen in the rectosigmoid concerning for a neoplastic or inflammatory process. Finally, a computed tomography scan of the abdomen and pelvis showed a 7.1 cm right pelvic mass attached to the bladder and cecum, moderate right hydroureteronephrosis, and a 5.2 cm left adnexal mass with soft tissue changes narrowing the sigmoid colon. The next step was to take the patient for an exploratory laparotomy. During exploratory laparotomy, extensive adhesions and desmoplastic reactions were observed in the pelvic region, involving the sigmoid colon, bladder, cecum, and appendix. Two firm masses were identified in the retroperitoneum, one located in the left lower quadrant (LLQ) adherent to the posterior wall of the sigmoid colon and one in the right lower quadrant (RLQ) adherent to the posterior wall of the cecum. Three specimens were sent to pathology for further examination: a portion of the sigmoid colon, a resection from the RLQ mass, and a resection from the LLQ mass. Pathology reported dense fibrotic masses with abscess-like formation, reactive in nature and of unclear etiology, and negative for malignancy. They were negative for fibromatosis (β-catenin negative), and IgG4+/IgG+ was approximately 5%. Interestingly, the LLQ mass also contained remnants of the fallopian tube and ovary and benign cystic changes. This case report presents a unique and atypical presentation of RPF, deviating from the conventional presentation of ureteral obstruction. The patient\'s initial symptoms suggested colonic obstruction, a clinical scenario rarely linked to RPF. This case underscores the significance of considering diverse clinical presentations when diagnosing RPF, thereby expanding our comprehension of the condition\'s clinical spectrum and ultimately refining patient care and management.

A prompt response to glucocorticoids is a clinical hallmark of IgG4-related disease. However, manifestations characterised by prominent tissue fibrosis on histological examination can be less responsive to glucocorticoid therapy than other types of IgG4-related disease. These manifestations include retroperitoneal fibrosis, fibrosing mediastinitis, Riedel thyroiditis, orbital pseudotumor, and hypertrophic pachymeningitis, among others. To explain this discrepancy, a preliminary distinction into proliferative and fibrotic phenotypes of IgG4-related disease has been proposed on the basis of clinical presentation, pathological features, and response to immunosuppressive therapy. Implications of this classification for patient management remain an important area of investigation. In this Series paper, we aim to dissect the pathophysiology of tissue fibrosis in IgG4-related disease and discuss how clinicians should approach the management of fibrotic manifestations of IgG4-related disease based on the most recent diagnostic and therapeutic developments.

BACKGROUND: Lateral approaches for lumbar interbody fusion (LIF) allow for access to the lumbar spine and disc space by passing through a retroperitoneal corridor either pre- or trans-psoas. A contraindication for this approach is the presence of retroperitoneal scarring that may occur from prior surgical intervention in the retroperitoneal space or from inflammatory conditions with fibrotic changes and pose challenges for the mobilization and visualization needed in this approach. However, there is a paucity of evidence on the prevalence of surgical complications following lateral fusion surgery in patients with a history of abdominal surgery.
OBJECTIVE: The primary aim of this study is to describe the association between surgical complications following lateral interbody fusion surgery and prior abdominal surgical.
METHODS: Retrospective study.
METHODS: Patients over the age of 18 who underwent lateral lumbar interbody fusion at a large, tertiary care center between 2011 and 2019 were included in the study.
METHODS: The primary outcome included medical, surgical, and thigh-related complications either in the intraoperative or 90-day postoperative periods. Additional outcome metrics included readmission rates, length of stay, and operative duration.
METHODS: The electronic health records of 250 patients were reviewed for demographic information, surgical data, complications, and readmission following surgery. The association of patient and surgical factors to complication rate was analyzed using multivariable logistic regression. Statistical analysis was performed using R statistical software (R, Vienna, Austria).
RESULTS: Of 250 lateral interbody fusion patients, 62.8% had a prior abdominal surgery and 13.8% had a history of colonic disease. The most common perioperative complication was transient thigh or groin pain/sensory changes (n=62, 24.8%). A multivariable logistic regression considering prior abdominal surgery, age, BMI, history of colonic disease, multilevel surgery, and the approach relative to psoas found no significant association between surgical complication rates and colonic disease (OR 0.40, 95% CI 0.02-2.22) or a history of prior abdominal surgeries (OR 0.56, 95% CI 0.20-1.55). Further, the invasiveness of prior abdominal surgeries showed no association with overall spine complication rate, lateral-specific complications, or readmission rates (p>.05).
CONCLUSIONS: Though retroperitoneal scarring is an important consideration for lateral approaches to the lumbar spine, this study found no association between lateral lumbar approach complication rates and prior abdominal surgery. Further study is needed to determine the impact of inflammatory colonic disease on lateral approach spine surgery.

Retroperitoneal fibrosis, a rare and often idiopathic condition, poses significant diagnostic challenges. While most cases are considered idiopathic or immune-mediated, a small but important proportion are associated with malignant neoplasms, with implications for prognosis and management. The present study describes the case of a 69-year-old man who presented to the emergency department of the Virgen de las Nieves University Hospital (Granada, Spain), with a 2-week history of epigastric pain, vomiting and altered bowel habits. Laboratory investigations revealed previously undiagnosed renal insufficiency. An abdominal computed tomography (CT) scan showed extensive diffuse retroperitoneal infiltration extending from the periduodenal region to the pubic bone, resulting in gastric dilatation and hydronephrosis. A CT-guided retroperitoneal biopsy was performed and pathology confirmed the presence of urothelial carcinoma. This diagnosis led to the initiation of a chemotherapy regimen consisting of carboplatin and gemcitabine specifically designed for urothelial carcinoma. A follow-up 18F-FDG PET scan performed 6 months later showed a partial functional response. This case illustrates a rare presentation of urothelial carcinoma masked by extensive retroperitoneal fibrosis, and highlights the importance of accurate diagnosis in reducing tumor burden and improving the clinical status of patients.

BACKGROUND: In 2018, diagnostic criteria were introduced for IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis (PA/RPF). This study assessed the existing criteria and formulated an improved version.Methods and Results: Between August 2022 and January 2023, we retrospectively analyzed 110 Japanese patients diagnosed with IgG4-related disease (IgG4-RD) involving cardiovascular and/or retroperitoneal manifestations, along with 73 non-IgG4-RD patients (\"mimickers\") identified by experts. Patients were stratified into derivation (n=88) and validation (n=95) groups. Classification as IgG4-RD or non-IgG4-RD was based on the 2018 diagnostic criteria and various revised versions. Sensitivity and specificity were calculated using experts\' diagnosis as the gold standard for the diagnosis of true IgG4-RD and mimickers. In the derivation group, the 2018 criteria showed 58.5% sensitivity and 100% specificity. The revised version, incorporating \"radiologic findings of pericarditis\", \"eosinophilic infiltration or lymphoid follicles\", and \"probable diagnosis of extra-PA/-RPF lesions\", improved sensitivity to 69.8% while maintaining 100% specificity. In the validation group, the original and revised criteria had sensitivities of 68.4% and 77.2%, respectively, and specificities of 97.4% and 94.7%, respectively.
CONCLUSIONS: Proposed 2023 revised IgG4-related cardiovascular/retroperitoneal disease criteria show significantly enhanced sensitivity while preserving high specificity, achieved through the inclusion of new items in radiologic, pathological, and extra-cardiovascular/retroperitoneal organ categories.

UNASSIGNED: A 50-year-old woman presented a dry syndrome, joint pain, inflammatory syndrome, polyclonal hypergammaglobulinemia, and tubulointerstitial nephritis. Imaging studies (including FDG PET/CT) revealed infrarenal retroperitoneal fibrosis with periaortitis and hypermetabolic osteosclerotic lesions. Bone scintigraphy demonstrated intense uptake in the femoral, tibial, and radial regions, suggestive of non-Langerhans histiocytosis, specifically Erdheim-Chester disease. A bone biopsy confirmed the presence of IgG4-positive plasma cells but no histiocytes. The patient received corticosteroid therapy followed by rituximab, resulting in a complete response. This case suggests an atypical manifestation of bone lesions in IgG4-related disease, emphasizing the diagnostic challenge between IgG4-related disease and Erdheim-Chester disease.