Urachal yolk sac tumor penetrating the bladder as a diagnostic challenge: a case report and review of the literature

Background Yolk sac tumor (YST) is a germ cell tumor. It is primarily located in the gonads but can also occur extragonadally (extragonadal yolk sac tumor - EGYST), most commonly in the pelvis, retroperitoneum or mediastinum. Only a few YSTs of the urachus have been described. Case report We present a rare case report of a 37-year-old male with episodes of macroscopic hematuria. The histological specimen obtained by transurethral resection showed a solid, and in some parts papillary infiltrative, high-grade tumor with numerous areas of marked nuclear atypia and clear invasion between the detrusor bundles. Glandular pattern has been observed in only minority of the tumor. Immunohistochemistry showed significant positivity for GPC3, SALL4 and cytokeratins AE1/AE3, while KRT7 and GATA3 were negative. We concluded that the biopsy findings were consistent with urothelial carcinoma with infrequent YST differentiation. In definitive surgical specimens we found a malignant epithelial, glandular and cystically arranged tumor of germinal appearance arising from urachus. The surrounding urothelium was free of invasive or in situ tumor changes. We reclassified the tumor as a urachal YST. Conclusion EGYST was suspected because glandular and hepatoid structures were found, but the presence of these structures should be verified by immunohistochemistry.

Prognostic Value of C4d Immunolabelling in Adult Patients With IgA Vasculitis

Background and Objectives: Glomerular C4d deposits are associated the severity and outcomes of IgA nephropathy. Whether this holds true in immunoglobulin A vasculitis (IgAV) is not known. The main objective of the study was to analyze the prognostic value of glomerular C4d immunolabelling on kidney impairment in adults with IgAV.Design, Setting, Participants, Measurements: This retrospective cohort study included 120 adults with IgAV and a kidney biopsy performed between 1995 and 2018 in two French university hospital centers. All paraffin-embedded biopsies were reassessed according to Oxford classification. Immunofluorescence for C4d was performed in all cases. For analysis, patients were grouped according to positivity for C4d in the glomerular area. The main outcome was a composite endpoint of 50% increase in 24 h-proteinuria, or eGFR decrease by 50%, or kidney replacement therapy.Results: The median follow-up was 28.3 months. Twenty-three patients met the composite endpoint, 12 for kidney replacement therapy, 6 for an eGFR decrease >50% and 5 for a >50% increase in proteinuria. At time of biopsy, the median proteinuria was 1.9 g/24 h and the median eGFR 73.5 mL/min/1.73 m2. Among the 102 patients evaluable for C4d, 24 were positive on >30% glomeruli, mainly with a parieto-mesangial pattern. In this group, the initial proteinuria was more frequently nephrotic than in the C4d– group (60% vs. 33%, P = 0.039). Mesangial hypercellularity was more frequent in the C4d+ group (42% vs. 13%; P = 0.006) whereas macroscopic hematuria was more frequent in the C4d– group (18% vs. 0%; P = 0.03). After a median follow-up of 28 months, kidney survival did not differ according to C4d status.Conclusion: In a population of adult IgAV patients, glomerular positivity for C4d was associated with the severity of the kidney disease at presentation, but not with subsequent renal function deterioration.

Nutcracker syndrome - a mini review on current knowledge

Nutcracker syndrome (NCS) seems to be an under-diagnosed condition defined as external compression of the left renal vein and consequent blood outflow impairment. The majority of cases involve the left renal vein entrapment between abdominal aorta and the superior mesenteric artery. The exact epidemiology is unknown due to lack of consensus on diagnostic criteria and often asymptomatic courses. NCS may occur at any age with peak presentation in the second and third decade of life. There is a slight prevalence in females. The most frequent presentations include micro and macroscopic hematuria, orthostatic proteinuria, orthostatic hypotension, flank pain. Patients may develop pelvic congestion syndrome comprising dyspareunia, dysmenorrhea, abdominal pain, pelvic, gluteal, vulvar varicose veins and varicocele in men. Clinical suspicion of NCS based on signs and symptoms, requires imaging confirmation utilising modalities such as doppler ultrasonography, computed tomography and magnetic resonance angiography, intravascular ultrasound and phlebography. Treatment options of NCS range from conservative surveillance to nephrectomy, thus appropriate approach should be based on clinical manifestation and severity of symptoms. Patients presenting with mild to moderate haematuria, and acceptable symptoms should be treated conservatively. In cases of severe symptoms or when conservative management fails, invasive treatment should be considered. Recommended open surgical procedures include left renal vein distal transposition and renal autotransplantation. Endovascular stenting approach seems to be an encouraging solution. Further long term follow-up is required to create objective treatment guidelines.

Tonsillectomy in IgA Nephropathy - Clinical Case

This article presents the clinical case of IgA nephropathy of a patient hospitalized in order to investigate a nephritic syndrome, apparently with acute onset, discovered in the following circumstances: recurrent macroscopic hematuria, decreased urinary volume, the first episode occurring about a month ago in the context of an acute infection of upper airways, remitted under antibiotic therapy, with reappearance every 2 weeks, also accompanied by odynophagia and decreased urinary volume.

Flexible Cystoscopy in the Setting of Macroscopic Hematuria: Do the Findings Justify Its Use?

<b><i>Background:</i></b> Patients hospitalized due to gross hematuria frequently complete evaluation in the outpatient setting. The use of office flexible cystoscopy during hospitalization may lead to prompt diagnosis and treatment but can be limited due to low visualization and artifacts that can hamper diagnostic ability. <b><i>Objective:</i></b> The objective of this study was to assess flexible cystoscopy findings and yield performed in patients hospitalized due to gross hematuria. <b><i>Methods:</i></b> Medical records of patients who underwent flexible cystoscopy while hospitalized during September 2018–December 2019 were reviewed. Cystoscopic findings were categorized into (1) suspicious mass in the bladder or prostate, (2) nonsuspicious changes in the bladder, and (3) nondiagnostic exam. Descriptive statistics were used to report the clinical characteristics of the study cohort and the findings of cystoscopy. Univariate logistic regression analyses were used to identify predictors of malignant findings. <b><i>Results:</i></b> The study cohort consisted of 69 patients (median age of 76 years). Initial cystoscopy findings were suspicious for malignancy in 26/69 patients (38%), nonsuspicious for malignancy in 34/69 patients (49%), and nondiagnostic in 9/69 patients (13%). The median follow-up time was 9 months (range 4–14 months). Twenty patients (29%) were diagnosed with malignancy (sensitivity of 75% and specificity of 78%). The procedure led to either diagnosis or treatment of 39 patients (57%). However, in 30 patients (43%), the initial cystoscopy did not aid in the diagnosis, led to misdiagnoses, or required a follow-up cystoscopy. On univariate analyses, none of the precystoscopy variables were predictive of bladder malignancy. <b><i>Conclusion:</i></b> Flexible cystoscopy in the setting of acute hematuria requiring hospitalization did not lead to diagnosis or treatment in over 40% of cases. In this setting, consideration should be given to performing an upfront cystoscopy under anesthesia.