Bladder cancer is a urological malignant tumor with high morbidity and mortality. Masses protruding into the bladder cavity is an important feature for clinical diagnosis of bladder cancer. However,patients with encrusted bladder cancer(EBC)do not present with masses protruding into the bladder cavity and thus this malignancy is often misdiagnosed. Four patients were admitted in Peking University People's Hospital from July 2015 to February 2017. All of them were males aged 40 to 77 years(mean:58 years). Patients were mainly manifested as frequent urination,urgency,nocturia,and decreased bladder capacity,with or without difficulty of voiding.Although the bladder walls were markedly thickened,there was no obvious mass on imaging scans. Three patients received urodynamic test,which showed the maximum capacity of the bladder was 41 to 128 ml(mean:91 ml). One patient presented with gross hematuria,two patients presented with microscopic hematuria,and the remaining one patient had no hematuria. No mass was observed by cystoscopy. All of the patients were diagnosed with bladder cancer by repeated biopsy or intraoperative frozen section analysis.
Adult ; Aged ; Biopsy ; Cystoscopy ; Hematuria ; Humans ; Male ; Middle Aged ; Urinary Bladder Neoplasms ; diagnosis ; pathology

Diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI), specifically arising in ileal neobladder, is a rare neoplasm. We present an unusual case of Epstein–Barr virus (EBV)–positive DLBCL-CI arising within neobladder with detailed clinical, histological, and immunophenotypical features in an immunocompetent patient. An 88-year-old male was admitted for gross hematuria. He had undergone radical cystectomy and ileal neobladder 17 years ago for invasive bladder cancer. Computed tomography showed enhancing lesions on dome and posterior wall of neobladder with mucosal thickening and multiple enlarged retroperitoneal lymphadenopathies. Transurethralresection of neobladder lesion revealed the diffuse infiltration of large lymphoid cells which were positive for CD20, CD30, and multiple myeloma oncogen-1 with EBV-encoded small RNAs co-localizing, and diagnosis of EBV-positive DLBCL-CI was made. After multi-agent chemotherapy, the lesion disappeared. We suggest that clinicians should consider the possibility of DLBCL-CI in patients presented with hematuria during follow-up after bladder reconstruction.
Aged, 80 and over ; B-Lymphocytes ; Cystectomy ; Diagnosis ; Drug Therapy ; Follow-Up Studies ; Hematuria ; Humans ; Inflammation ; Lymphocytes ; Lymphoma, B-Cell ; Male ; Multiple Myeloma ; RNA ; Urinary Bladder ; Urinary Bladder Neoplasms

Nutcracker syndrome (NCS) is a syndrome caused by compression of the left renal vein (LRV), between the abdominal aorta and the superior mesenteric artery, resulting in hypertension of the LRV and hematuria. Doppler ultrasonography (US) has been commonly used for the diagnosis of NCS. However, several technical issues, such as Doppler angle and sample volume, need to be considered to obtain satisfactory results. In addition, morphologic changes of the LRV and a jetting phenomenon across the aortomesenteric portion of the LRV on contrast-enhanced computed tomography (CECT) are diagnostic clues of NCS. With proper Doppler US and CECT, NCS can be diagnosed noninvasively.
Aorta, Abdominal ; Diagnosis ; Hematuria ; Hypertension ; Mesenteric Artery, Superior ; Renal Veins ; Tomography, X-Ray Computed ; Ultrasonography, Doppler

Nutcracker syndrome (NCS) refers to left renal vein compression with impaired blood outflow. The etiology of NCS has been attributed to various anatomic anomalies. Posterior NCS is caused by compression of the retroaortic left renal vein between the aorta and spine. The classic symptoms of NCS include left flank pain with gross or microscopic hematuria. The frequency and severity of the syndrome vary from asymptomatic microhematuria to severe pelvic congestion. For this reason, diagnosis of NCS is difficult and often delayed. Here, we report a case of posterior NCS that was incidentally discovered.
Aorta ; Diagnosis ; Estrogens, Conjugated (USP) ; Flank Pain ; Hematuria ; Renal Veins ; Spine

OBJECTIVE: This study examined the clinical significance of microscopic hematuria and grade of hydronephrosis in ureteral calculi patients visiting the emergency department (ED). METHODS: The EDs of two medical centers were reviewed retrospectively from August 1, 2012 to July 31, 2017. The total ureteral calculi patients were 2,069 and 1,593 patients were analyzed after exclusion. The normal range of microscopic hematuria in both medical centers was 0–5/high power field (HPF) of red blood cell in urinalysis. Therefore, microscopic hematuria more than 5/HPF was defined as positive. The size of the stone, grade of hydronephrosis (normal, mild, moderate, and severe) and location of ureteral calculi in the non-contrast abdomen and pelvis computed tomography (CT) was measured by one person at each medical center using same method. The patients with or without microscopic hematuria and grade of hydronephrosis were then compared. RESULTS: The median size of the ureteral calculi patients without and with microscopic hematuria was 5.4 mm (range, 3.8–9.0 mm) and 4.0 mm (range, 3.0–5.8 mm) (P < 0.001). The patients grouped as normal to mild, and moderate to severe hydronephrosis, without microscopic hematuria was 191 (16.5%) and 91 (20.9%), respectively. On the other hand, microscopic hematuria was 966 (83.5%) and 345 (79.1%) (P=0.042). According to the location of ureteral calculi, with and without microscopic hematuria was 81.9% and 18.1% in the upper ureter, 82.9% and 17.1% in the mid ureter, and 82.6% and 17.4% in the lower ureter, respectively (P=0.935). CONCLUSION: The median size of the stone and grade of hydronephrosis were related to microscopic hematuria but the location of the ureteral calculi was not related. Therefore, in cases without microscopic hematuria in suspected ureteral calculi, clinicians should check the abdomen and pelvis CT for an accurate diagnosis and treatment of ureteral calculi.
Abdomen ; Diagnosis ; Emergencies ; Emergency Service, Hospital ; Erythrocytes ; Hand ; Hematuria ; Humans ; Hydronephrosis ; Methods ; Pelvis ; Reference Values ; Retrospective Studies ; Ureter ; Ureteral Calculi ; Urinalysis

Alport syndrome (ATS) is an inherited glomerular disease caused by mutations in one of the type IV collagen novel chains (α3, α4, and α5). ATS is characterized by persistent microscopic hematuria that starts during infancy, eventually leading to either progressive nephritis or end-stage renal disease. There are 3 known genetic forms of ATS, namely X-linked ATS, autosomal recessive ATS, and autosomal dominant ATS. About 80% of patients with ATS have X-linked ATS, which is caused by mutations in the type IV collagen α5 chain gene, COL4A5. Although an 80% mutation detection rate is observed in men with X-linked ATS, some difficulties do exist in the genetic diagnosis of ATS. Most mutations are point mutations without hotspots in the COL4A3, COL4A4, and COL4A5 genes. Further, there are insufficient data on the detection of COL4A3 and COL4A4 mutations for their comparison between patients with autosomal recessive or dominant ATS. Therefore, diagnosis of ATS in female patients with no apparent family history can be challenging. Therefore, in this study, we used whole-exome sequencing (WES) to identify mutations in type IV collagen in 2 girls with glomerular basement membrane structural changes suspected to be associated with ATS; these patients had no relevant family history. Our results revealed de novo c.4688G>A (p.Arg1563Gln) and c.2714G>A (p.Gly905Asp) mutations in COL4A5. Therefore, we suggest that WES is an effective approach to obtain genetic information in ATS, particularly in female patients without a relevant family history, to detect unexpected DNA variations.
Child ; Collagen Type IV ; Diagnosis ; DNA ; Exome ; Female ; Glomerular Basement Membrane ; Hematuria ; Humans ; Kidney Failure, Chronic ; Korea ; Male ; Nephritis ; Nephritis, Hereditary ; Point Mutation

D-penicillamine has been reported to cause antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presenting as rapidly progressive glomerulonephritis or pulmonary-renal syndrome mostly in adults. We report a pediatric case of D-penicillamine induced ANCA-associated vasculitis that manifests as a pulmonary-renal syndrome with a mild renal manifestation. A 13-year-old girl who has been taking D-penicillamine for five years under the diagnosis of Wilson disease visited the emergency room because of hemoptysis and dyspnea. She had diffuse pulmonary hemorrhage, microscopic hematuria, and proteinuria. Myeloperoxidase ANCA was positive, and a renal biopsy revealed pauci-immune crescentic glomerulonephritis. Under the diagnosis of D-penicillamine-induced ANCA-associated vasculitis, D-penicillamine was switched to trientine, and the patient was treated with plasmapheresis, glucocorticoid, cyclophosphamide, and mycophenolate mofetil. Pulmonary hemorrhage improved rapidly followed by the disappearance of the hematuria and proteinuria five months later.
Adolescent ; Adult ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Child ; Cyclophosphamide ; Diagnosis ; Dyspnea ; Emergency Service, Hospital ; Female ; Glomerulonephritis ; Hematuria ; Hemoptysis ; Hemorrhage ; Hepatolenticular Degeneration ; Humans ; Penicillamine ; Peroxidase ; Plasmapheresis ; Proteinuria ; Trientine ; Vasculitis

BACKGROUND/AIMS: Because of rarity, role of chemotherapy of bladder adenocarcinoma are still unidentified. Therefore, we performed a retrospective analysis of the clinical features and chemotherapy outcomes of bladder adenocarcinoma. METHODS: Eligible patients for this retrospective analysis were initially diagnosed with bladder adenocarcinoma and presented with a clinically no other primary site of origin. The collected data included age, gender, performance status, stage, hemoglobin, albumin, initial date of diagnosis, treatment modality utilized, response to treatment, presence of relapse, last status of patient, and last date of follow-up. RESULTS: We retrospectively reviewed 29 patients, who were treated with chemotherapy for bladder adenocarcinoma at 10 Korean medical institutions from 2004 to 2014. The median age of patients was 58 years (range, 17 to 78) and 51.7% of the patients were female. Urachal adenocarcinoma was identified in 15 patients. Of 27 symptomatic patients, 22 experienced gross hematuria. Twelve patients were treated with 5-f luorouracil based chemotherapy, five were gemcitabine based, three were taxane and others. Thirteen of them achieved complete response (10.3%) or partial response (34.5%). Median progression-free survival (PFS) and overall survival (OS) for all patients were 10.6 months (95% confidence interval [CI], 9.5 to 11.6) and 24.5 months (95% CI, 1.2 to 47.8), respectively. The cases of urachal adenocarcinoma exhibited worse tendency in PFS and OS (p = 0.024 and p = 0.046, respectively). CONCLUSIONS: Even though bladder adenocarcinoma had been observed moderate effectiveness to chemotherapy, bladder adenocarcinoma is a highly aggressive form of bladder cancer. PFS and OS were short especially in urachal carcinoma.
Adenocarcinoma* ; Diagnosis ; Disease-Free Survival ; Drug Therapy* ; Female ; Follow-Up Studies ; Gynecology* ; Hematuria ; Humans ; Recurrence ; Retrospective Studies* ; Urinary Bladder Neoplasms ; Urinary Bladder*

C4 glomerulopathy is a recently introduced entity that presents with bright C4d staining and minimal or absent immunoglobulin and C3 staining. We report a case of a 62-year-old man with C4 glomerulonephritis (GN) and uveitis. He presented to the nephrology department with proteinuria and hematuria. The patient also had intermediate uveitis along with proteinuria and hematuria. A kidney biopsy that was performed in light of continuing proteinuria and hematuria showed a focal proliferative, focal sclerotic glomerulopathy pattern on light microscopy, absent staining for immunoglobulin or C3 by immunofluorescence microscopy, with bright staining for C4d on immunohistochemistry, and electron-dense deposits on electron microscopy. Consequently, C4 GN was suggested as the pathologic diagnosis. Although laser microdissection and mass spectrometry for glomerular deposit and pathologic evaluation of the retinal tissue were not performed, this is the first report of C4 GN in Korea and the first case of coexisting C4 GN and uveitis in the English literature.
Biopsy ; Diagnosis ; Glomerulonephritis* ; Hematuria ; Humans ; Immunoglobulins ; Immunohistochemistry ; Kidney ; Korea ; Mass Spectrometry ; Microdissection ; Microscopy ; Microscopy, Electron ; Microscopy, Fluorescence ; Middle Aged ; Nephrology ; Proteinuria ; Retinaldehyde ; Uveitis ; Uveitis, Intermediate*

Primary or metastatic malignant melanoma can mimic benign blue nevus in rare cases, making the diagnosis challenging. Herein, we report an exceptionally rare case of blue nevus-like melanoma and its blue nevus-like metastasis which was detected by catheterized urine cytology. The patient presented with blue-colored papuloplaques on his temple which were diagnosed as blue nevus-like melanoma on punch biopsies. While he was admitted for administration of chemotherapy, hematuria was detected. Catheterized urine cytology revealed singly scattered oval to spindle-shaped pigmented cells with a moderate degree of variation in shape and size. Many of them had small nuclei with indiscernible to inconspicuous nucleoli while only a few cells showed nuclear enlargement and nuclear hyperchromasia, which could be diagnostic pitfalls. Most of the cells on the smear were positive for HMB45 immunostaining, which confirmed the diagnosis of metastatic blue nevus-like melanoma. To the best of our knowledge, the present case is the first report describing cytomorphologic findings of blue nevus-like metastasis of melanoma in the urine specimen.
Biopsy ; Catheters* ; Diagnosis ; Drug Therapy ; Hematuria ; Humans ; Melanoma* ; Neoplasm Metastasis ; Nevus, Blue