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Background: Peritoneal fibrosis is the prevailing complication induced by prolonged exposure to high glucose in patients undergoing peritoneal dialysis. Methods: To elucidate the molecular mechanisms underlying this process, we conducted an integrated analysis of the transcriptome and chromatin accessibility profiles of human peritoneal mesothelial cells (HMrSV5) during high-glucose treatment. Results: Our study identified 2775 differentially expressed genes (DEGs) related to high glucose-triggered pathological changes, including 1164 upregulated and 1611 downregulated genes. Genome-wide DEGs and network analysis revealed enrichment in the epithelial–mesenchymal transition (EMT), inflammatory response, hypoxia, and TGF-beta pathways. The enriched genes included VEGFA, HIF-1α, TGF-β1, EGF, TWIST2, and SNAI2. Using ATAC-seq, we identified 942 hyper (higher ATAC-seq signal in high glucose-treated HMrSV5 cells than in control cells) and 714 hypo (lower ATAC-seq signal in high glucose-treated HMrSV5 cells versus control cells) peaks with differential accessibility in high glucose-treated HMrSV5 cells versus controls. These differentially accessible regions were positively correlated (R = 0.934) with the nearest DEGs. These genes were associated with 566 up- and 398 downregulated genes, including SNAI2, TGF-β1, HIF-1α, FGF2, VEGFA, and VEGFC, which are involved in critical pathways identified by transcriptome analysis. Integrated ATAC-seq and RNA-seq analysis also revealed key transcription factors (TFs), such as HIF-1α, ARNTL, ELF1, SMAD3 and XBP1. Importantly, we demonstrated that HIF-1α is involved in the regulation of several key genes associated with EMT and the TGF-beta pathway. Notably, we predicted and experimentally validated that HIF-1α can exacerbate the expression of TGF-β1 in a high glucose-dependent manner, revealing a novel role of HIF-1α in high glucose-induced pathological changes in human peritoneal mesothelial cells (HPMCs). Conclusions: In summary, our study provides a comprehensive view of the role of transcriptome deregulation and chromosome accessibility alterations in high glucose-induced pathological fibrotic changes in HPMCs. This analysis identified hub genes, signaling pathways, and key transcription factors involved in peritoneal fibrosis and highlighted the novel glucose-dependent regulation of TGF-β1 by HIF-1α. This integrated approach has offered a deeper understanding of the pathogenesis of peritoneal fibrosis and has indicated potential therapeutic targets for intervention. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Granulomatosis with polyangiitis is a systemic inflammatory disease characterized by necrotizing vasculitis that affects small- and medium-sized blood vessels. Granulomatous inflammation affects the lungs, ears, nose, and throat, and commonly affects the kidneys, although the retroperitoneal tissue is rarely affected. Several studies have reported an increased risk of venous thromboembolism. Early diagnosis and treatment are of vital importance due to the rapid progression of the disease. Case presentation: We present the case of a 66-year-old Moroccan man followed for bilateral jugular thrombosis. Cavitary pulmonary nodules and retroperitoneal fibrosis with thrombosis involving several vascular territories were detected on thoracoabdominopelvic computerized tomography scan. Laboratory analyses revealed that the patient was positive for cytoplasmic antineutrophilic antibodies. The diagnosis of granulomatosis with polyangiitis was retained. Treatment with glucocorticoids and immunosuppressive agents resulted in significant clinical and radiological improvement over the following months. Conclusions: We describe the diagnostic steps and the difficulty of managing this patient. Rare manifestations, such as retroperitoneal fibrosis, have been reported in the literature in association with granulomatosis with polyangiitis, and should not delay the diagnosis and treatment of granulomatosis with polyangiitis owing to its severity. [ABSTRACT FROM AUTHOR]

Background: IgG4-related disease (IgG4-RD) includes a group of immune-mediated diseases histologically characterized by lymphoplasmacytic infiltrate with a prevalence of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. Autoimmune pancreatitis, sialadenitis, dacryoadenitis and retroperitoneal fibrosis are the most frequent manifestations. IgG4-related sialadenitis usually affects submandibular glands and is very rare in children. Here we report the case of IgG4-related sialadenitis in a six-year-old patient previously diagnosed as juvenile recurrent parotitis.Case Presentation: A six-year-old patient was referred to our Centre for left parotid swelling of 4 × 3 cm, that was tender, soft in consistency, with overlying red and warm skin. His general condition was good but he was subfebrile; general examination revealed mild enlargement of left cervical lymph nodes. In the last 2 years he had had five episodes of parotitis, diagnosed by another pediatric Center as juvenile recurrent parotitis. On ultrasound examination the left parotid gland appeared enlarged, inhomogeneous, with a colliquative intraparotid lymph node and no evidence of sialolithiasis. Laboratory tests showed an increase of white blood cells and anti-VCA IgM and IgG positivity, with anti-EBNA e anti-EA I negativity. The patient was initially treated with oral antibiotics, but after 10 days the parotid became fluctuating, requiring surgical biopsy and drainage. Postoperative course was regular, with complete remission under oral antibiotic and steroid therapy. Microbiological tests, including cultures for aerobic and anaerobic bacteria, mycobacteria and Bartonella, were negative. Surprisingly, histology showed marked fibrosis and histiocytic and lymphoplasmacellular infiltrate with polyclonal plasma cells mostly expressing IgG4 immunoglobulins. Thus, the diagnosis of IgG4 related chronic sialadenitis in recurrent parotitis and recent EBV infection was made.Conclusions: IgG4-related sialadenitis is very unusual in children. Histology plays a key role in diagnosis, considering that up to 30% of patients have normal serum IgG4 levels, as shown in our case. The lack of previous histological data makes it impossible to attribute our patient's previous episodes of parotitis to IgG4-RD, though it is a very consistent possibility. [ABSTRACT FROM AUTHOR]

Background: Refractory non-malignant ureterostenosis is challenging to treat. The experience to treat the stenosis primarily cause by retroperitoneal fibrosis with the Resonance and Allium metallic stent is still limited. We aim to evaluate the efficacy and safety of these two stents and provide alternative treatment options.Methods: A retrospective study was conducted for patients with non-malignant ureterostenosis and treated with the Resonance and Allium stents from March 2011 to September 2020 in our department. The efficacy was evaluated by the change of serum creatinine, glomerular filtration rate (GFR), the proportion of GFR of the affected side and hydronephrosis grade. The safety was evaluated by postoperative presence of moderate or severe overactive bladder (OAB), recurrent urinary infection, pain, stent displacement, encrustation and re-obstruction.Results: 33 patients were eligible for the study, including 18 cases treated by the Resonance stents and 15 patients treated by the Allium stents. The patients of two groups had similar age and gender proportion. The cause of ureterostenosis was mainly retroperitoneal fibrosis in both groups but the Resonance group had more idiopathic cases. Follow-up time was significantly longer in the Resonance group than the Allium group (36.2 ± 24.0 vs 9.4 ± 5.0 months, p < 0.001). Both groups showed improvement or maintenance of serum creatinine level, GFR, the GFR proportion of the affected side and hydronephrosis grade after treatment. The Resonance group presented significant higher incidence of moderate or severe OAB, recurrent urinary infection and pain, while the Allium group showed significant more cases of re-obstruction.Conclusion: Both the Resonance and Allium stent can relieve the non-malignant refractory ureterostenosis effectively. The Resonance stent may cause more irritable symptoms while the Allium stent may have a higher rate of re-obstruction. The long term efficacy and safety of the Allium stent in treating non-malignant refractory ureterostenosis requires further study. [ABSTRACT FROM AUTHOR]

Background: Retroperitoneal fibrosis (RPF) is a rare disease with a poor prognosis characterized by systemic inflammation and fibroinflammatory tissue. Idiopathic RPF (IRPF) accounts for approximately two-thirds of RPF cases.Case Presentation: A 56-year-old female patient with abdominal distension was admitted to Changxing Hospital. Laboratory tests revealed mild anemia and elevated CA125, while IgG4 and autoantibodies were within the normal ranges. Computed tomography (CT) revealed a gallbladder-occupying lesion, pancreatic cyst and retroperitoneal mass, which may have contributed to bilateral ureteral compression and hydronephrosis. The initial diagnosis was gallbladder carcinoma with lymph node metastasis. Then, abdominal adhesiolysis, cholecystectomy and partial hepatectomy were performed. Histologically, there were fibrosis and inflammation in the retroperitoneal tissue without any malignant cells in the retroperitoneal or gallbladder tissue. Finally, we confirmed the diagnosis of idiopathic retroperitoneal fibrosis, chronic cholecystitis and pancreatic cyst. The patient recovered well following the CT scan, in which dilatation of the bile duct was reduced, and effusion of the bilateral upper ureter was no longer significant.Conclusion: This atypical case illustrates that RPF can be combined with other biliary tract diseases. The coexistence of other diseases conceals the symptoms of RPF, which increases the difficulty of image identification. A high degree of suspicion is necessary for routine clinical work. As more cases are reported, further advances in the diagnosis and treatment of RPF can be expected. [ABSTRACT FROM AUTHOR]

Background: Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis with slow progression over the years that is particularly difficult to diagnose. Cases: Here we report three cases of ECD without BRAF mutation presenting with a renal mass, hairy kidney appearance, and a rather benign course, for which the diagnosis of ECD was delayed, characterized by multiple investigations and unsuccessful treatments attempts. In two cases the distinction from IgG4-related disease required multiple investigations and reevaluation of the clinical, radiological, histological, and immunological characteristics. Conclusion: A correct diagnosis of ECD may take several years and often requires revisiting previous hypotheses. Reassessment of histological slides and more modern complementary exams such as PET-CT or BRAF and MAPK-ERK mutation analysis can help to confirm the diagnosis of ECD and to select effective therapy. [ABSTRACT FROM AUTHOR]

Introduction: A retroperitoneal abscess caused by duodenal perforation is a relatively rare disease clinically. We report the case of a patient with a local high-density shadow at the head of the retroperitoneal pancreas.Case Presentation: A 28-year-old Chinese man presented with fever and abdominal pain after overeating and heavy drinking. On physical examination, he had mild tenderness in his upper abdomen. Laboratory examination results showed a white blood cell count of 24.06 109/L and a neutrophil absolute value of 18.81 109/L, and a computed tomography scan showed an irregular soft tissue mass with uneven enhancement of the cystic wall in the retroperitoneal space. Gastroscopy showed that there was a fistula in the anterior wall of the duodenal bulb. Endoscopic anastomosis clip system (over-the-scope clip) of the duodenal fistula was performed successfully. After the operation, nasal feeding was provided with a nutrition tube, and empiric anti-infection, acid-inhibiting, and stomach-protecting treatments were administered. Our patient's body temperature gradually returned to normal, and his abdominal pain decreased.Conclusions: A retroperitoneal abscess caused by duodenal perforation can be diagnosed by clinical symptoms and abdominal computed tomography imaging. The choice of treatment should be based on accurate and timely clinical and imaging data. [ABSTRACT FROM AUTHOR]

Background: Laser ablation with a neodymium-doped yttrium aluminum garnet (Nd:YAG) laser is a minimally invasive approach which is able to achieve a precise tissue necrosis. The study was aimed to assess the feasibility and efficiency of laser ablation in the treatment of retroperitoneal and hepatic portal unresectable metastatic lymph nodes. Methods: Eight patients including 11 pathologically proven metastatic lymph nodes, 4 in retroperitoneal, 7 in hepatic portal region, were treated by laser ablation. Primary cancers were cholangiocarcinoma (n = 4) and hepatocellular carcinoma (n = 4). Under sonographic guidance, the laser ablation was performed percutaneously. Follow-up contrast computed tomography or magnetic resonance image was performed. Results: The treatments were completed in single process in all the patients. No severe complications occurred. Follow-up contrast computed tomography or magnetic resonance imaging at 1 and 3 months showed partial responses in 11 lymph nodes. The local response rate at the 6 month follow-up was 75.0 %. The overall response rate was 62.5 %. Abdominal pain scores decreased significantly in all patients. Tumor marker levels decreased in six patients. The Child-Pugh grade did not change. Conclusions: The results suggest that sonographically guided laser ablation is technically feasible for the local treatment of unresectable retroperitoneal and hepatic portal lymph nodes from hepatic cancer. Although further study is needed to evaluate its long time efficacy, abdominal pain relief is prominent. [ABSTRACT FROM AUTHOR]

Background: Patients with a primary pure seminoma in the testis who have elevated serum alpha-fetoprotein are rare and should be treated as patients with nonseminomatous germ cell tumors. However, nonpalpable testicular tumors in this condition have never been reported. We describe a case of nonpalpable pure testicular seminoma with elevated serum alpha-fetoprotein presenting retroperitoneal metastasis. Case Presentation: A 29-year-old Asian man was referred to our hospital with right flank pain. Computed tomography showed a mass located between his aorta and inferior vena cava, but a testicular tumor was not detected. His serum levels of lactate dehydrogenase, alpha-fetoprotein, and DUPAN-2 were high. Although no tumor or nodule was palpable in his testis, ultrasonography revealed multiple low echoic lesions in his right testicular parenchyma. He was diagnosed with right testicular cancer with retroperitoneal lymph node metastasis and underwent right high orchiectomy. A pathological examination revealed pure seminoma and no nonseminomatous components were found in the specimen. Three courses of induction systemic chemotherapy (cisplatin, etoposide, and bleomycin) normalized his serum alpha-fetoprotein and DUPAN-2 levels. Three additional courses of chemotherapy (etoposide and bleomycin) were performed, and treatment was completed with laparoscopic retroperitoneal lymph node dissection. Pathology of the dissected specimen showed fibrous and necrotic tissue with no viable cells. He is alive without recurrence 54 months after orchiectomy. Conclusions: We report a case of pure testicular seminoma with elevated serum alpha-fetoprotein and DUPAN-2 presenting retroperitoneal metastasis. We recommend an ultrasound examination of bilateral testes when large retroperitoneal tumors are detected in young men, even if a mass is not palpable in the scrotum. [ABSTRACT FROM AUTHOR]

Background: We presented abdominal liposarcoma cases diagnosed and managed in a tertiary care center and also conducted a literature review on main features of this tumor. Methods: Chart reviews of eight cases were conducted, and clinical, surgical, histopathological, and follow-up data were recorded. Results: Overall, complete surgical resection was performed with adjacent organ resection in 25% of cases, and radiotherapy was not administered. Recurrence was developed in only one case and died after 2 years and 3 months, and other cases are under follow-up without recurrence. Histopatological examinations revealed findings of well-differentiated liposarcoma. Conclusions: According to our surgical experience, the surgical margin positivity may not be a determining factor for the survival of patients with well-differentiated liposarcoma, and in the absence of macroscopic invasion, adjacent organ resection may not be required. Radiotherapy may not be preferred when complete resection of abdominal mass was achieved. [ABSTRACT FROM AUTHOR]

Background: Retroperitoneal lipomas are an extremely rare condition with only 17 cases described in the literature since 1980. They can reach enormous size and cause significant abdominal symptoms. The most important differential diagnosis is the well-differentiated liposarcoma, which preoperatively often may not definitely be ruled out. Case presentation: We present the case of a 73 year-old Caucasian patient with a giant retroperitoneal lipoma of 9 kg measuring 55 cm in diameter. The patient presented with abdominal pain and swelling that had been slowly progressive for the last 15 years. On computerized tomography an immense retroperitoneal tumor was revealed. Intraoperatively, the tumor did not show any signs of infiltrative growth, therefore sole tumor extirpation was performed. Conclusion: Retroperitoneal lipomas are not clearly distinguishable from well-differentiated liposarcomas on imaging and even biopsies may be misleading. Moreover, abdominal symptoms, i.e. pain, obstipation and dysphagia may occur due to mechanical displacement. Therefore, surgical exploration with complete oncological resection is the therapy of choice if malignity cannot be ruled out. [ABSTRACT FROM AUTHOR]

Background: Isolated retroperitoneal cystic masses are uncommon with an estimated incidence of 1/5750 to 1/250,000. The majority present with size related symptoms, complications, or a mass. Approximately a third of patients are asymptomatic and are diagnosed incidentally. Aetiologies of retroperitoneal cystic masses (RPC) include mesenteric, omental, splenic and enteric duplication cysts. Neoplastic RPCs can be divided into epithelial (mucinous or serous cystadenoma), mesothelial (mesothelioma), germ cell (cystic teratoma) and cystic changes in a solid neoplasm (paraganglioma, neurilemmoma, sarcoma). Case presentation: A 53 year-old man presented to us with abdominal pain related to a large mass in his left upper quadrant with associated anorexia and weight loss. He gave no history of previous trauma and denied having symptoms or a history of pancreatitis. He said he had felt this mass increasing in size over the course of several years. Clinical examination of his abdomen revealed a large firm left sided mass extending to his left upper quadrant. Imaging with computed tomography (CT) and magnetic resonance imaging cholangio-pancreatogram (MRCP) revealed a 13.7 cm × 12.2 cm × 10.9 cm cystic lesion in the retroperitoneum which was separate from the kidney, pancreas, spleen and bowel. At laparotomy, this mass was easily dissected from the surrounding viscera and was excised completely intact. Histopathological assessment found the mass to be a large fibrous pseudocyst with no epithelial lining. Conclusion: We present a rare case of an isolated large retroperitoneal fibrous pseudocyst unrelated to previous pancreatitis which was successfully managed with surgery. [ABSTRACT FROM AUTHOR]

Background The objective of this study was to modify the adrenal gland-sparing strategy based on retroperitoneal laparoscopic radical nephrectomy by reviewing the anatomic relationship between the kidney and the adrenal gland. Methods From June 2010 to October 2012, a total of 68 patients (45 males and 23 females) with localized renal cell carcinoma were treated at our hospital. The study included 35 cases that were right side and 33 cases that were left, and average patient age was 54.06 years. The average tumor size was 4.7 cm. Tumors were classified via the TNM staging system. All patients underwent adrenal gland-sparing surgery based on retroperitoneal laparoscopic radical nephrectomy. Results For each patient, surgery was successful without conversion to open surgery. The average operative time was 56.65 ± 26.60 min, and the mean blood loss was 70.61 ± 60.96 ml. All patients were discharged from the hospital 3 to 8 days after surgery. During surgery, the adrenal gland was slightly lacerated in three cases and the peritoneum showed perforation in six cases. Only one case recurred during the study follow-up. Conclusions Based on retroperitoneal laparoscopy radical nephrectomy, this effective adrenal glandsparing surgery showed direct exposure of tissue and little interference of the upper pole of the kidney. Elevation of the adrenal gland could help with the complete dissection of the adrenal gland from the kidney. The separation of the kidney was rapid, simple and accurate. The probability of adrenal gland damage was reduced. This strategy is recommended for widespread use in T1-2 renal neoplasms. [ABSTRACT FROM AUTHOR]

Introduction Giant cell arteritis is the most common form of large-vessel vasculitides. However, it is probable that extracranial involvement is underdiagnosed in patients with classical giant cell arteritis. In the recent literature most cases of giant cell arteritis have been described in conjunction with aortic aneurysms or dissections. Nonetheless the coexistence of giant cell arteritis and retroperitoneal fibrosis is extremely rare. Here, we describe a case of giant cell arteritis at a very early clinical stage, in a woman with coexistence of retroperitoneal fibrosis. Case presentation We report a case of giant cell arteritis at a very early clinical stage, in a 47-year-old Greek woman with coexistence of retroperitoneal fibrosis who was admitted to our hospital with a history of high-grade fever and mild right periumbilical abdominal pain for the past 30 days. In the context of fever of unknown origin, an abdomen computed tomography was ordered. A temporal artery biopsy was also performed because during hospitalization she complained of a headache. Examination of eosin and hematoxylin slides from biopsy specimens of her temporal artery, showed lesions consisting of predominantly lymphocytes, few plasma cells and occasional polymorphonuclear leucocytes. In addition no giant cells were detected in examining biopsies at multiple levels. This was consistent with giant cell arteritis according to the American college of Rheumatology criteria. An abdomen computed tomography revealed the presence of a retroperitoneal soft-tissue mass located anteriorly to the upper infrarenal aorta at the site of the scintigraphic uptake. The computed tomography and magnetic resonance imaging characteristics of the mass were consistent with retroperitoneal fibrosis, and its morphology suggestive of benignity. Our patient started oral prednisolone and was afebrile from day one. Conclusions In our experience this is the first case of retroperitoneal fibrosis due to giant cell arteritis occurring at the same time. Involvement of the aorta (aortitis) and its branches has been also observed in a subset of patients with giant cell arteritis. In addition, giant cell arteritis has been associated with a markedly increased risk of aortic aneurysm particularly thoracic aortic aneurysm. [ABSTRACT FROM AUTHOR]

Introduction Follicular dendritic cells are non-phagocytic, non-lymphoid cells of the immune system that are necessary for antigen presentation and the regulation of reactions in the germinal centers of the lymph nodes. Follicular dendritic cell sarcoma is an unusual cancer, particularly in the intra-abdominal region. In the present report we describe an unusual case of retroperitoneal follicular dendritic cell sarcoma that emphasizes the difficulty of diagnosing and treating this tumor. Retroperitoneal follicular dendritic cell sarcoma has only been rarely reported in the literature to date. Case presentation A 46-year-old Chinese woman of Han ethnicity presented with chronic right lower quadrant abdominal pain over the preceding 4 weeks. The tumor was resected and submitted to histopathological examination. The case was verified as retroperitoneal follicular dendritic cell sarcoma by microscopic examination and immunohistochemical analysis. After diagnosis, she received postoperative radiotherapy and chemotherapy. She has survived 3 years postoperatively, although she has a pulmonary metastasis. Conclusions Retroperitoneal follicular dendritic cell sarcoma may demonstrate aggressive potential. This study indicated that postoperative adjuvant radiotherapy and chemotherapy could extend the survival of a patient with retroperitoneal follicular dendritic cell sarcoma. [ABSTRACT FROM AUTHOR]

Introduction: This case report illustrates the relatively rare occurrence of a urinoma masquerading as an abscess in a patient with refractory recurrent non-Hodgkin's lymphoma. Although urinomas have been reported after urinary instrumentation, our case was confounded by a concurrent pleural effusion, an immunocompromised host, and clinical appearance and subsequent incision and drainage that was consistent with an abscess. Although not the first case reported in the literature, the constellation of findings in our patient demonstrates the importance of including urinoma in the differential diagnosis of patients with flank mass.Case Presentation: Here we report a case of a retroperitoneal fluid collection found in an immunocompromised 65-year-old Caucasian woman. She had recurrent non-Hodgkin's lymphoma and was neutropenic when the collection was found. Drainage of the fluid demonstrated infection, but the case was complicated by increasing fluid production and the development of an ipsilateral pleural effusion. Despite pleural drainage, the abscess output continued to be high. A computed tomography scan demonstrated fluid collection around the renal calices suggestive of rupture; analysis of the fluid was suggestive of a urinoma. The etiology, pathogenesis, and treatment of urinomas in such patients are discussed.Conclusions: Urinomas can present in patients who have had urinary tract instrumentation or trauma, but can occur in other hosts. Patients with hematologic malignancy can develop malignant or sympathetic pleural effusions and are at risk for skin and soft tissue infections. Clinicians caring for immunocompromised patients, particularly hematologists, oncologists, and transplant clinicians, should be aware of this potential complication because rapid identification and attempt at correction are important to optimize outcome. [ABSTRACT FROM AUTHOR]

Background: Extra-adrenal paraganglioma of the retroperitoneum with metastasis to the vertebra is very rare. To our knowledge this is the first report of this kind of disease in the literature. Case presentation: Here, we present an oroginal case of paraganglioma of the retroperitoneum with metastasis to the abdominal vertebra in a 42-year-old female patient who was successfully treated by complete removal of the tumor and its metastasis. The patient was followed up for four years and remained disease-free. Conclusion: Our case demonstrated the need to consider paraganglioma of the retroperitoneum in the differential diagnosis of retroperitoneal mass, metastatic tumors to the vertebra, and the importance of radical surgery for a successful management of the disease. Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/ vs/1956611954880197 [ABSTRACT FROM AUTHOR]

Composite pheochromocytoma/paraganglioma is a rare tumor with elements of pheochromocytoma/ paraganglioma and neurogenic tumor. Most were located in the adrenal glands, and extra-adrenal composite pheochromocytoma is extremely rare. Only 4 cases in the retroperitoneum have been described in the online database PUBMED. Here, we report a case of retroperitoneal extra-adrenal composite pheochromocytoma and review the related literature. Virtual slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/ vs/1700539911908679. [ABSTRACT FROM AUTHOR]

Introduction: Similar to pheochromocytomas, paragangliomas can secrete catecholamines, although they are usually non-functional and clinical presentation is non-specific. We present a case of accidental, intra-operatively diagnosed neuroendocrine-active sympathetic paraganglioma, which was suspected and confirmed during elective retroperitoneal tumor removal.Case Presentation: A 25-year-old Caucasian Croatian man, American Society of Anesthesiologists status 1, underwent elective surgery for retroperitoneal tumor removal. The tumor had been discovered by chance during a routine examination and was suspected to be a sarcoma. Our patient had no history of previous medical conditions nor did he have symptoms characteristic of a neuroendocrine secreting tumor. The results of ultrasound and magnetic resonance imaging studies showed a large, well demarcated retroperitoneal tumor mass in his upper abdomen localized between the aorta and vena cava, measuring approximately 9×6×4.5cm. In the operating room an epidural catheter was inserted at the T7 to T8 level prior to induction of general anesthesia. Epidural analgesia was maintained by an infusion pump with local anesthetic and opiate mixture. During the surgical excision of the tumor, hemodynamic changes occurred, with hypertension (205/110mmHg) and tachycardia (up to 120 beats/minute). In spite of the fact that the surgical field of work did not include adrenal glands whose direct manipulation could explain this occurrence, there was a high degree of suspicion for the presence of a neurosecreting tumor. His clinical symptoms were relieved after administration of urapidil, esmolol and magnesium sulfate. After tumor excision, our patient developed severe hypotension. Hemodynamic stability was reinstated with aggressive volume replacement, with crystalloids and colloids, vasopressors and hydrocortisone. His post-operative course was unremarkable and on the eighth post-operative day our patient was discharged from hospital, with no consequences or symptoms on follow-up two years after surgery.Conclusions: Our patient's case emphasizes the need to consider the presence of extra-adrenal paragangliomas in the differential diagnosis of retroperitoneal tumors, despite their rare occurrence. In our patient's case, invasive hemodynamic monitoring during combined general anesthesia and epidural analgesia and early recognition of catechol-induced symptoms raised suspicion of the existence of a paraganglioma, and this led to an adequate therapeutic approach and favorable outcome of the surgery. Pre-operative recognition of paragangliomas could lead to better pre-operative preparation, but even high clinical suspicion in undiagnosed forms during surgery and the availability of rapid and short-acting vasodilatators, α-blockers and β-blockers might favor good outcome. [ABSTRACT FROM AUTHOR]

Background: Operative management of all gunshot's traumas carries a high rate of unwarranted interventions that are known to cause serious complications. Selective nonoperative management is thus being increasingly practiced which has reduced these avoidable interventions. Physical examination and computed tomography scans are most sensitive in assessing need of laparotomy. Assessment of internal injuries on the basis of an estimated bullet trajectory is often practiced but has seldom been studied. We report a case of conservative management of a thoraco abdominal gun shot patient where an estimated bullet trajectory was indicative of serious injuries. To the best of our knowledge this is the first report of a thoraco abdominal gunshot that, despite of a protracted trajectory, had no sequelae and was thus managed nonoperatively. Case presentation: A 30 year old male patient having height of 180 cm and weight of 70 kg (Body Mass Index 21.6) presented with complaint of a penetrating injury at left side of upper torso. The patient had no symptoms or obvious bleeding and was vitally stable. On examination a 1 cm × 1 cm entry wound at the left 3rd intercostal space in the mid clavicular line was identified. The chest and abdomen were otherwise unremarkable on examination. The chest radiograph displayed clear lung fields. The abdominal radiographs displayed a bullet in the upper left quadrant of the abdomen lateral to the spine. The bullets estimated trajectory from 3rd intercostal space and its lodgment in the abdomen lateral to the spine indicated severe visceral injury. The computed tomography scan showed that the bullet was lodged postero-medially to the left kidney. All thoracic, intra peritoneal and retroperitoneal visceral structures were identified to be normal. The patient remained clinically and vitally stable, hence was managed nonoperatively being discharged after 48 h of observation. Conclusion: From this case we conclude that decision for managing gun shot patients should be based on objective clinical and diagnostic findings. We recommend further investigation of the predictability of estimated trajectory for visceral injuries and consequent operative intervention as we found it to be misleading in this case. [ABSTRACT FROM AUTHOR]

Background: Malignant transformation describes the phenomenon in which a somatic component of a germ cell teratoma undergoes malignant differentiation. A variety of different types of sarcoma and carcinoma, all non-germ cell, have been described as a result of malignant transformation. Case presentation: A 33-year-old man presented with a left testicular mass and elevated tumour markers. Staging investigations revealed retroperitoneal lymphadenopathy with obstruction of the left ureter and distant metastases. Histopathology from the left radical orchiectomy showed a mixed germ cell tumour (Stage III, poor prognosis). The ureter was stented and four cycles of cisplatin, etoposide and bleomycin chemotherapy administered. After initial remission, the patient recurred four years later with a large retroperitoneal mass involving the renal vessels and the left ureter. Left retroperitoneal lymph node dissection with en-bloc resection of the left kidney was performed. Histopathology revealed a germ cell tumour metastasis consisting mainly of mature teratoma. Additionally, within the teratoma a papillary renal cell carcinoma was found. The diagnosis was supported by immunohistochemistry showing positivity for AMACR, CD10 and focal expression of RCC and CK7. There was no radiological or histopathological evidence of a primary renal cell cancer. Conclusions: To the best of our knowledge, malignant transformation into a papillary renal cell carcinoma has not been reported in a testicular germ cell tumour metastasis following platinum-based chemotherapy. This histological diagnosis might have implications for potential future therapies. In the case of disease recurrence, renal cell cancer as origin of the recurrent tumour has to be excluded because renal cell carcinoma metastases would not respond well to the classical germ cell tumour chemotherapy regimens. [ABSTRACT FROM AUTHOR]

The benign cystic mesothelioma of the peritoneum is a rare lesion and is known for local recurrence. This is first case report of a rapidly developing massive abdominal tumor with histological finding of benign cystic mesothelioma (BCM). We describe a BCM arising in the retroperitoneal tis[sue on the right side, lifting ascending colon and cecum to the left side of abdomen. Patient was an active 58-year-old man who noticed a rapid abdominal swelling within a two month time period with a weight gain of 40 pounds. Patient had no risk factors including occupational (asbestos, cadmium), family history, social (alcohol, smoking) or history of trauma. We will discuss the clinical, radiologic, intra-operative, immunohistochemical, pathologic findings, and imaging six months after surgery. Patient has no recurrence and no weight gain on follow up visits and imaging. [ABSTRACT FROM AUTHOR]

Background: To assess the influence of sentinel lymph nodes (SNs) SPECT/CT and 18F-choline (18F-FCH) PET/CT in radiotherapy (RT) treatment planning for prostate cancer patients with a high-risk for lymph node (LN) involvement. Methods: Twenty high-risk prostate cancer patients underwent a pelvic SPECT acquisition following a transrectal ultrasound guided injection of 99mTc-Nanocoll into the prostate. In all patients but one an 18F-FCH PET/CT for RT treatment planning was performed. SPECT studies were coregistered with the respective abdominal CTs. Pelvic SNs localized on SPECT/CT and LN metastases detected by 18F-FCH PET/CT were compared to standard pelvic clinical target volumes (CTV). Results: A total of 104 pelvic SNs were identified on SPECT/CT (mean 5.2 SNs/patient; range 1-10). Twenty-seven SNs were located outside the standard pelvic CTV, 17 in the proximal common iliac and retroperitoneal regions above S1, 9 in the pararectal fat and 1 in the inguinal region. SPECT/CT succeeded to optimize the definition of the CTV and treatment plans in 6/20 patients due to the presence of pararectal SNs located outside the standard treatment volume. 18F-FCH PET/CT identified abnormal tracer uptake in the iliac LN region in 2/19 patients. These abnormal LNs were negative on SPECT/CT suggesting a potential blockade of lymphatic drainage by metastatic LNs with a high tumour burden. Conclusions: Multimodality imaging which combines SPECT/CT prostate lymphoscintigraphy and 18F-FCH PET/CT identified SNs outside standard pelvic CTVs or highly suspicious pelvic LNs in 40% of high-risk prostate cancer patients, highlighting the potential impact of this approach in RT treatment planning. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the feasibility of pre-operative radiotherapy (54 Gy) with Helical Tomotherapy (HT) followed by surgery. Methods and materials: Ten patients with non-metastatic resectable retroperitoneal liposarcomas were treated by pre-operative tomotherapy (54 Gy) and surgery. Clinical and biological toxicities were evaluated on the CTCAEV3.0 scale. For nine patients, delivered tomotherapy plans were compared with retrospectively-planned dynamic intensity-modulated radiotherapy (IMRT) dosimetric studies. Results: No immediate or late Grade>2 toxicities were observed after radiotherapy. Post-operatively, one patient died and three patients experienced Grade 3 toxicity (two digestive and one metabolic). These toxicities disappeared and only two patients presented persistent Grade 1 paresthesia. R0 resection was obtained for four patients, R1 for four, and R2 resection for two. With a median follow-up of 26 months, no local or metastatic relapse was observed. Dosimetric comparisons between HT and retrospectively-planned IMRT demonstrate adequate target volume coverage for both techniques. Gastrointestinal sparing is higher with HT with a D200cc reduced by 5 Gy. Integral dose (ID) was increased in HT. Conclusions: High dose pre-operative radiotherapy (54 Gy) for retroperitoneal liposarcoma is feasible and mostly well tolerated. Cumulative toxicity and tolerance depend mainly on patient's general status. Image-guided radiation therapy (IGRT) is essential, irrespective of the IMRT technique used. Furthermore, HT offers the possibility of sparing selected areas in such complex volumes [ABSTRACT FROM AUTHOR]

Unlabelled: Introduction: Surgical treatment for lumbar burst fractures is complex and typically involves either a retroperitoneal corpectomy and/or a posterior pedicle screw fixation. We describe the case of a patient with a lumbar burst fracture who was cured via a less invasive approach that has not been previously reported as standalone treatment.Case Presentation: This 25-year-old Caucasian man presented with excruciating axial low back pain exacerbated by any attempt to elevate the head of the bed after a motor vehicle accident. Computed tomography demonstrated a burst L4 fracture without spinal canal compromise. The patient underwent a bilateral vertebroplasty with an injectable polymer that mimics cortical bone. Postoperatively, the patient was progressively mobilized in a thoracolumbar spinal orthosis brace without any recurrence of pain. Postoperative computed tomography showed no loss of height in the L4 vertebral body. At one-year postoperatively, the patient was symptom free and the computed tomography scan showed good fracture healing.Conclusion: Retroperitoneal corpectomy and/or posterior multi-segment fixation for lumbar burst fractures without neural compression in young patients are associated with loss of mobility and potential future adjacent level disease. Our limited vertebroplasty intervention with close postoperative clinical monitoring has not been previously described as standalone treatment, and it offers the advantages of less operative morbidity and maintenance of lumbar mobility in selected patients. [ABSTRACT FROM AUTHOR]

The article presents the findings of a research conducted on Retroperitoneoscopic Laparo-endoscopic Single-site Radical Nephrectomy (RLESS-RN). It mentions that the retroperitoneal approach (RLESS) was followed by using the Alexis wound retractor as a single access with conventional laparoscopic instruments. It concludes that RLESS technique of radical nephrectomy is a safe and feasible procedure for management of localized renal cancer. It highlights the advantage of LESS, when compared with conventional laparoscopic procedure.

Background: Distinguishing an atypical lipomatous tumor/well-differentiated liposarcoma from a benign lipomatous tumor on morphology alone can be difficult and there is an established role for MDM2 fluorescent in situ hybridization studies in making this differential diagnosis. There is no literature on the role for MDM2 fluorescent in situ hybridization studies in distinguishing between a well-differentiated liposarcoma with extreme fibrosis and a fibrosing inflammatory pseudotumor.Case Presentation: We report the case of a 76-year-old Australian woman initially diagnosed by an excision biopsy with a retroperitoneal fibrosing inflammatory pseudotumor. She was then diagnosed 5 years later with a pleomorphic undifferentiated sarcoma. Upon review of the original resection specimen, we were able to show that the tumor demonstrated MDM2 amplification. MDM2 amplification was also present in some adjacent bland adipose tissue, and also in the tumor recurrence as a pleomorphic undifferentiated sarcoma.Conclusion: Taken together, our findings provide strong evidence that the original tumor was a misdiagnosed well-differentiated liposarcoma with extreme fibrosis, and the pleomorphic undifferentiated sarcoma represented a recurrence of the same tumor with dedifferentiation. [ABSTRACT FROM AUTHOR]

Background: Primary retroperitoneal serous adenocarcinoma (PRSA) is an extremely uncommon malignancy exclusively reported in females. Due to the rarity of the disease, it is difficult to establish a standardized treatment.Case Presentation: We describe a unique case of PRSA in a 71-year-old male who presented with right-sided lower back pain and numbness. Magnetic resonance imaging identified a mass invading the adjacent psoas muscle and twelfth rib. Tissue biopsy confirmed poorly differentiated PRSA. Patient was initially treated with neoadjuvant carboplatin and paclitaxel chemotherapy regimen. This resulted in complete radiological resolution of the tumor. However, 12 weeks later, rapid recurrence was noted on follow-up CT scan. The patient was then treated with external radiotherapy with concurrent nivolumab, an anti-PD-1 antibody. The patient displayed a positive response to treatment with reduction in primary tumor and metastases and had a sustained disease control.Conclusion: Treatment with radiotherapy in combination with anti-PD-1 antibody could be an effective modality of management for PRSA. [ABSTRACT FROM AUTHOR]

Background: Nephrogenic diabetes insipidus (DI) secondary to a urinary tract obstruction is a rare condition. Herein, we report a case of partial nephrogenic DI due to obstructive uropathy in a patient with Castleman's disease.Case Presentation: A 78-year-old man underwent computed tomography (CT) at his local hospital because of persistent edema of the leg and polyuria (both lasting approximately 2 months); retroperitoneal fibrosis was detected on the CT scan. An abdominal CT scan showed bilateral hydronephrosis, and a surgical biopsy of the para-aortic lymph node revealed Castleman's disease. To resolve the hydronephrosis, a double J stent was inserted; however, his polyuria continued. As his serum osmolality (311 mOsm/kg) was greater than 300 mOsm/kg, and his serum sodium level was 149 mEq/L, a water deprivation test was not performed. On a vasopressin challenge test, his urine was not sufficiently concentrated to the expected range, indicating partial nephrogenic DI. He was treated with hydrochlorothiazide (25 mg/day), and his urine output gradually decreased to within the normal range. The patient recovered uneventfully and underwent treatment for Castleman's disease.Conclusion: To the best of our knowledge, this is the first case of partial nephrogenic DI due to obstructive uropathy associated with Castleman's disease. [ABSTRACT FROM AUTHOR]

Background: Retroperitoneal desmoid-type fibromatosis (DF) is an uncommon mesenchymal neoplasm presenting as a firm mass with locally aggressive features. It usually manifests as a well-circumscribed or ill-defined, solid mass on cross-sectional imaging. Cystic changes of DF have been described in the literature in association with prolonged medical treatment or abscess formation. However, spontaneous cystic change is rarely reported. Case presentation: Here we report the case of a 46-year-old patient with a DF mimicked a large cystic tumor in the retroperitoneum. Ultrasonography and computed tomography were performed in order to search for localizations and characteristics of the cystic tumor. Radiological findings showed an oval cystic mass with a relatively thick wall, measuring 18.3 × 12.3 × 21.5 cm in the left upper abdomen. Laparoscopic spleen-preserving distal pancreatectomy was performed and histopathological examination by immunohistochemical study enabled us to diagnose a DF invading the pancreatic parenchyma. The patient remained asymptomatic during an 8-month follow up period. Conclusions: We report an extremely rare case of retroperitoneal DF with spontaneous cystic change. DF can manifest as a mainly cystic mass with a thick wall, as in our case, which makes the correct diagnosis difficult. DF should be included in the preoperative differential diagnosis of a cystic retroperitoneal mass, regardless of its rarity. [ABSTRACT FROM AUTHOR]

Background: Aspergillosis in patients with impaired immunity usually presents with invasive pulmonary infection and dissemination to a variety of organs via hematogenous spread. Aspergilloma in the retroperitoneal cavity is a rare disease with only a few cases reported in the literature. To the best of our knowledge, the present case of a retroperitoneal aspergilloma with no surgical history is only the second report in the literature.Case Presentation: A 65 year-old man, who had been receiving immunosuppressive treatment for rheumatoid arthritis with vasculitis for 9 years, was referred to the Urology Department with a retroperitoneal mass. This was confirmed by computed tomography performed during treatment for pulmonary aspergilloma. Because it was not possible to rule out malignant disease (e.g., liposarcoma), surgical exploration was performed. Pathological examination revealed aspergillus hyphae with fat necrosis, and retroperitoneal aspergilloma was diagnosed and appropriately treated. The tumor did not recur subsequently.Conclusion: Our present case emphasizes that pharmacological treatments for aspergilloma in the retroperitoneal cavity have poor drug transitivity, so the relative effectiveness of pharmacological response is not useful for differentiating retroperitoneal aspergilloma from malignant disease. [ABSTRACT FROM AUTHOR]