[Show abstract][Hide abstract] ABSTRACT: The authors present two cases of primary sclerosing epithelioid fibrosarcoma (SEF) of the kidney. Both patients had a mass in the upper part of the left kidney without any primary extrarenal neoplastic lesions. Grossly, the tumors were solid masses both measuring 7.5 cm in the greatest diameter. Histologically, one of the lesions exhibited a predominantly lobular growth of round or oval small uniform epithelioid cells in variable cellularity. Circular zones of crowded tumor cells alternating with hypocellular collagenous tissue in a concentric fashion around entrapped native renal tubules were distinctive. The second case was distinctive with significant cytological atypia in the neoplastic cells and prominent reactive proliferations in the trapped renal tubules. Immunohistochemically, vimentin, bcl-2 and MUC4 were diffusely positive in both. They were negative for S-100 protein, CD34, and desmin, whereas CD99 were positive in one lesion. Fluorescence in situ hybridization assay using dual staining probes detected EWSR1-CREB3L1 fusion in each lesion, which is characteristic molecular findings of SEF. One patient presented widespread distant metastases at the time of diagnosis. In the other, no tumor deposits were detected other than primary. Both patients have been alive with 30 and 10 month follow-ups, respectively. These tumors are 6th and 7th cases of primary renal SEF in the literature confirmed by FISH study, which exhibit unique and remarkable histomorphologic features.
[Show abstract][Hide abstract] ABSTRACT: Nonsecretory multiple myeloma (NSMM) is the absence of a detectable monoclonal protein in serum and urine of a multiple myeloma (MM) patient and immunoglobulin light chain (AL) amyloidosis is a significantly rare complication. A case of NSMM with AL amyloidosis and nephrotic range proteinuria is presented. Sharing clinical, therapeutic, and prognostic characteristics with MM, real challenge may be during initial diagnosis of NSMM and assessment of treatment response. In elderly patients with unexplained renal dysfunction, MM should be in the differential diagnosis and the absence of a monoclonal protein should not rule out MM but should remind us of the possibility of NSMM.
[Show abstract][Hide abstract] ABSTRACT: Background IgG4-Related Disease (IgG4-RD) is a recently recognized condition with a wide range of organ involvements.Few data exist on the global perspective of IgG4-RD regarding prevalence and the frequencies of the different clinical presentations.
Objectives This study is aimed to describe the clinical and laboratory characteristics of a series of patients diagnosed with IgG4-RD in Turkey.
Methods A retrospective study was performed. Demographic, clinical characteristics and imaging studies including PET-CT, in addition to serum IgG4 levels were recorded. Totally 48 pathologic specimens of the patients were re-evaluated regarding IgG4-RD morphologic findings and IgG4/total Ig G ratio. Patients were diagnosed according to diagnostic criteria for IgG4-RD.1
Results Totally 27 patients fulfilling the diagnostic criteria were included in the study, 18 of whom (66.7%) were male. Mean age at diagnosis was 55.9±11.8 years.
Retroperitoneal fibrosis is the most common finding. Fourteen (51.8%) of the patients had more than one clinical involvement. Thirty percent of patients had lymphadenopathy. Nine (33.3%) of patients had any additional cardiovascular involvement (thoracic aortitis/inflammatory abdominal aneurisms/coronary periarteritis). The global distribution by specific organ involvement was shown in Table. Three of the patients had normal serum IgG4 levels. Six patients had PET-CT. Patients had a wide range of inflammation assessed by PET-CT (SUV max levels were between 2.2-11.4). Twenty of the patients were pathologically proven IgG4-RD -16 of them had morphologic features and all had a ratio of IgG4/IgG ≥40%.
Conclusions The most usual presentation of IgG4 related disease in our study is middle aged males and as retroperitoneal fibrosis. Cardiovascular involvement, lymphadenopathy, and orbital/periorbital involvements were the other frequent findings. Not also the organ involvement but also inflammation level could be in a very wide range in IgG4-RD. More collaborative studies are required in order to assess the real prevalence of the disease and the frequencies of the different presentations.
Disclosure of Interest None declared
Annals of the Rheumatic Diseases 06/2015; 74(Suppl 2):301.2-301. DOI:10.1136/annrheumdis-2015-eular.5444 · 10.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The glomerular tip lesion (GTL) is a distinctive histopathologic lesion which is regarded as a variant of focal and segmental glomerulosclerosis (FSGS). The prognostic significance of GTL among other FSGS variants has been disputed. In order to define the clinical features and outcome of GTL, we retrospectively reviewed the presenting clinical features, laboratory and biopsy findings and surveillance in our cohort of GTL, which consisted of 20 adults with native kidneys (mean age 46 years) with follow-up data ranging from 3 to 137 months. At presentation, mean urine protein, serum albumin and cholesterol levels were 5.17 g/d, 2.6 g/dL and 312.9 mg/dL, respectively, and none had renal insufficiency. Microscopic hematuria was detected in five patients. At biopsy, glomerular segmental lesions consisted of GTL without perihilar or collapsing lesions. GTL was observed in a variable proportion of glomeruli from 2.6% to 100%. Mesangial proliferation was seen in nine cases, at a moderate degree in two and mild in the rest. Three biopsies showed mild, two showed moderate interstitial fibrosis/tubular atrophy. Eleven patients received steroids alone and eight received sequential therapy with steroids and a cytotoxic agent. At a mean follow-up of 40.6 months, 17 patients (85%) achieved complete remission of nephrotic syndrome, 15% had partial remission. Four of 17 suffered from recurrences. No patient progressed to end-stage renal disease. Serum albumin at diagnosis was the only predictor of a recurrence (p = 0.037). Microscopic hematuria correlated with incomplete remission (p = 0.045). Our study demonstrates a clearly favorable prognosis in patients with FSGS-GTL variant.
[Show abstract][Hide abstract] ABSTRACT: Ureteritis cystica (UC) is a benign condition. Although it can often be diagnosed with imaging techniques, we report a case of a child for whom we planned nephrectomy and ureteral augmentation cystoplasty, but abandoned the cystoplasty due to extensive UC in the ureter.
Indian Journal of Urology 10/2014; 30(4):443-4. DOI:10.4103/0970-1591.139592
[Show abstract][Hide abstract] ABSTRACT: Renal carcinoid tumor is an exceedingly rare malignancy. A 57-year-old man with a renal carcinoid tumor discovered after metastasizing to intraocular and bilateral orbital structures is described. The patient presented with a blind painful OS and a right superotemporal subconjunctival mass. Imaging studies revealed a large left intraocular tumor, a mass in the left medial rectus muscle, and right lacrimal gland enlargement. The OS was enucleated, and incisional biopsies were performed from the other 2 lesions. Histopathological studies demonstrated metastatic neuroendocrine tumor with chromogranin and synaptophysin positivity. Systemic work up revealed a right renal mass and multiple hepatic metastatic lesions. Radical nephrectomy was performed, and octreotide, capecitabine, and temozolomide were administered. Removal of the primary tumor and the eye that had no prospect for useful vision and further treatment with octreotide, capecitabine, and temozolomide provided a disease progression-free period of 24 months and allowed the patient to function normally.
Ophthalmic plastic and reconstructive surgery 04/2014; Publish Ahead of Print(4). DOI:10.1097/IOP.0000000000000112 · 0.88 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: A 49-year-old male presented with a painful mass in the left scrotum. An inguinal orchiectomy was performed. Pathological examination revealed a well-differentiated leiomyosarcoma completely located inside the testicular parenchyma. We report this unusual case because primary leiomyosarcoma of the testis proper is extremely rare; our patient being the 19th case recorded thus far in the medical literature. It can lead to significant clinical and diagnostic difficulty due to its wide differential diagnosis and extreme rarity.
Turk Patoloji Dergisi 09/2013; 29(3):227-230. DOI:10.5146/tjpath.2013.01179
[Show abstract][Hide abstract] ABSTRACT: This study was designed to investigate the possible protective effect of lycopene against the renal toxic effects of OTA. Male Sprague-Dawley rats (<200g, n=6) were treated with OTA (0.5mg/kg/day) and/or lycopene (5mg/kg/day) by gavage for 14 days. Histopathological examinations were performed and apoptotic cell death in both cortex and medulla was evaluated by TUNEL assay. Besides, biochemical parameters and activities of renal antioxidant selenoenzymes [glutathione peroxidase 1 (GPx1), thioredoxin reductase (TrxR)], catalase (CAT), superoxide dismutase (SOD); concentrations of total glutathione (GSH), and malondialdehyde (MDA) levels were measured. OTA treatment was found to induce oxidative stress in rat kidney, as evidenced by marked decreases in CAT (35%) activity and GSH levels (44%) as well as increase in SOD activity (22%) vs control group. Furthermore, TUNEL analysis revealed a significant increase in the number of TUNEL-positive cells in cortex (49%) and medulla (75%) in OTA administrated group compared to control (p<0.05). Lycopene supplementation with OTA increased GPx1 activity and GSH levels, and decreased apoptotic cell death in both cortex and medulla vs. control. The results of this study showed that at least one of the mechanisms underlying the renal toxicity of OTA is oxidative stress and apoptosis is the major form of cell death caused by OTA. Besides, our data indicate that the natural antioxidant lycopene might be partially protective against OTA-induced nephrotoxicity and oxidative stress in rat.
Experimental and toxicologic pathology: official journal of the Gesellschaft fur Toxikologische Pathologie 01/2013; 65(6). DOI:10.1016/j.etp.2012.12.004 · 1.86 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Congenital megacalycosis is a rare renal disease characterized by calyceal dilatation without pelvic or ureteral obstruction. If not accompanied by nephrolithiasis and urinary tract infection, this disease is completely benign and does not cause renal dysfunction. We present a case of congenital megacalycosis that was diagnosed at the age of 41 (oldest case in the literature) after admitting with hematuria and acute renal dysfunction. IgA nephropathy was also diagnosed in this patient. Since renal dysfunction is not likely in these patients, if encountered; renal biopsy should be performed although technically difficult to diagnose the cause of this dysfunction.
[Show abstract][Hide abstract] ABSTRACT: The prognostic value of the type and extent of extracapillary proliferation (ECP) in pauci-immune necrotizing crescentic glomerulonephitis (PIGN) was evaluated in this study. In 141 PIGN cases, all glomeruli with ECP were grouped according to type (cellular, fibrocellular and fibrous) and extent of the lesions in Bowman's space; (segmental, semicircumferential and circumferential, which might be termed full moon-FM). Cases with cellular and fibrous lesions involving ≥50% of glomeruli with ECP were classified as cellular and fibrous groups, respectively, while the remaining cases were classified as fibrocellular. Cases with segmental and circumferential (FM glomerulus) lesions involving ≥50% of glomeruli with ECP were classified as ECPI and ECPIII (FM) groups, respectively, while the rest were classified as ECPII. All the cases were classified according to Berden et al. Significant results were only nearly obtained for the FM group, including the need for dialysis. The Cox regression model revealed a 2.6-fold risk for FM cases regarding dialysis requirement. We propose that the percentage of FM glomeruli should be noted in the pathology report, and cases with more than 50% of FM glomeruli (FM group) should be identified in the group with increased risk of dialysis requirement. Our series also suggests that classification according to Berden et al. is of clinical relevance.
Pathology - Research and Practice 12/2012; 209(2). DOI:10.1016/j.prp.2012.10.012 · 1.40 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Glomerulopathy with fibronectin deposits is a rare hereditary kidney disease characterized by the extensive deposition of fibronectin in glomeruli, particularly in mesangial regions and subendothelial zones. Prognostically, the disease is known as slowly progressive, leading to kidney failure in most cases. We recently diagnosed glomerulopathy with fibronectin deposits in a 24-year-old man in whom proteinuria was detected incidentally. Genetic analysis of the fibronectin 1 (FN1) gene showed heterozygosity for the Y973C mutation. The same mutation was found in his elder brother, who similarly experienced proteinuria. Both patients had normal kidney function but persistent proteinuria after 30 months and 11 years of follow-up, respectively.
American Journal of Kidney Diseases 12/2012; 61(3). DOI:10.1053/j.ajkd.2012.08.050 · 5.90 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Objective:
To determine the incidence of dysplasia as a preneoplastic change and high-risk human papilloma virus (HPV) infection in penile condylomas, which are common HPV-related lesions and considered a risk factor for penile cancer.
Histologic analysis was done of 58 consecutive penile condylomas with tissue diagnosis. An immunohistochemical panel that included stains for p53, Ki-67, and p16INK4a was also used. HPV typing was successfully performed in 43 lesions. Genotyping was accomplished through polymerase chain reaction and flow-through hybridization with an HPV GenoArray Diagnostic Test kit.
Dysplasia was observed in 13 of the 58 condylomas (22%). High-risk HPV DNA was detected in 5 of 10 dysplastic lesions (50%) for which tissue blocks were available for study. High-risk HPV was not detected in the nondysplastic lesions (P<.001). Ki-67≥20% above the basal layer of epithelium and p53-positive immunostaining occurred more frequently in dysplastic lesions than in nondysplastic lesions; however, the difference was not statistically significance. Staining for p16INK4a was not helpful.
Anogenital condylomas in men are usually treated using destructive methods or with medication. We suggest that at least a part of the lesion must be removed and sent for histopathologic examination. If the histologic result shows significant dysplastic alteration, the lesion should be further investigated to determine the subtype of infective virus, because 50% of such lesions are associated with high-risk HPV. When oncogenic pathogens are found, careful patient follow-up for recurrences and counseling for the patient and his sexual partner(s) may be warranted.
[Show abstract][Hide abstract] ABSTRACT: Objective:
To investigate the effects of bilirubin in a rat model of pyelonephritis.
Experimental pyelonephritis was induced in 32 wistar rats and 4 groups were formed: group 1 (no treatment), group 2 (antibiotic), group 3 (bilirubin), and group 4 (antibiotic + bilirubin). Antibiotic was given on days 3 to 8, and bilirubin was administered between days 0 and 8 of bacterial inoculation. Half of the rats were killed on the 9th day (early period) and histopathological parameters, immunohistochemical renal fibrosis markers, apoptosis, and oxidant/antioxidant system activities were evaluated. The rest of the rats were killed at the 6th week of the study and evaluated for histopathological parameters and renal fibrosis markers.
Inflammatory activity was significantly lower in rats treated with antibiotic + bilirubin vs no treatment group both in the early and late periods. In the late period, inflammatory cell intensity was lower in rats treated with bilirubin vs no treatment and the antibiotic + bilirubin groups. Interstitial fibrosis/tubular atrophy was lower in the antibiotic + bilirubin group vs the no treatment and antibiotic groups, and in bilirubin vs antibiotic group. Tissue inhibitor of metalloproteinase-1 expression was lower in the bilirubin vs antibiotic group. Terminal deoxynucleotidyl transferase mediated 2'-deoxyuridine, 5'-triphosphate nick end labeling(+) cells were significantly lower in bilirubin and antibiotic + bilirubin groups vs no treatment group. Malondialdehyde levels were significantly lower in the antibiotic + bilirubin vs the no treatment group and superoxide dismutase activity was significantly higher in the antibiotic and antibiotic + bilirubin groups vs the no treatment group.
Bilirubin may have protective effects on pyelonephritis-associated inflammation in both early and late periods in addition to fibrosis and apoptosis when applied with antibiotics. When used alone, bilirubin may also prevent inflammation (in the late period) and apoptosis.
[Show abstract][Hide abstract] ABSTRACT: Purpose:
Behçet's disease (BD) is a rare inflammatory disorder with variable articular and systemic manifestations. Amyloidosis is an uncommon complication of BD. The aim of this study is to present clinicopathological characteristics and outcome of 8 patients with renal amyloidosis secondary to BD.
A total of 220 cases of secondary-type renal amyloidosis diagnosed over 26-year period (1981-2007) in a single institution via needle biopsy were reviewed retrospectively, and accompanying BD was found in ten of them. Clinical and follow-up information of the patients was gathered from hospital records and computer-based data system. Two cases were excluded from the study due to another concomitant preamyloidotic condition. Biopsy slides of remaining 8 cases were culled from the archives and re-examined. Extent and pattern of renal amyloid deposition were explored. Amyloid typing was accomplished via immunohistochemistry. Factors acting on outcome and renal survival were explored.
Patients were all male (age range 27-56). Duration of BD before diagnosis of amyloidosis varied from 24 to 192 months, and mean follow-up period was 89 months. Nephrotic syndrome was the most common presentation. Immunohistochemical typing revealed AA-amyloid in all cases. Two cases showed glomerular-dominant, one case vascular-dominant amyloid deposition, and rest were codominant glomerular and vascular, except one that was indeterminate. Patients were treated with steroids and/or different immunosuppressives, three progressed to end-stage kidney failure.
Behçet's disease is one of the rare diseases that lead to AA amyloidosis. Morphology of renal amyloidosis due to BD has diversities in terms of differential amyloid deposition in renal compartments. Patients follow variable clinical courses accordingly.
International Urology and Nephrology 09/2012; 45(3). DOI:10.1007/s11255-012-0268-z · 1.52 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We report a case of a 72-year-old male that underwent two sets of transrectal prostatic needle biopsy (TPNB) within 9 month period. Pathology showed unremarkable benign prostatic tissues in the first group of biopsies while extensive diffuse inflammation with the characteristic features of malakoplakia in the second set. Three cores in the repeat biopsy contained foci of prostatic adenocarcinoma as well. Occurrence of malakoplakia several months after TPNB in our case suggests that microorganisms may have been inoculated to the prostate during the biopsy procedure. We believe that malakoplakia must be added to the list of complications after TPNB.
The Canadian Journal of Urology 02/2012; 19(1):6124-7. · 0.98 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Purpose: This study aims to provide an insight to the developmental changes of the whole refluxing renal unit by investigating the pathological features of the distal end of ureters and their correlation with the scar formation patterns in the kidney.
Methods: The study includes 83 children (49 male, 34 female), who underwent ureteroneocystostomy (UNC) operations. Distal end of ureters ( approximately 3-4 mm) were resected during the reimplantation operation and 110 ureter specimens from 83 patients were available for pathological examination. The presentation age, clinical presentation pattern, reflux grade, scar patterns on DMSA were obtained from records and possible correlations with histopathological findings were investigated. Scar patterns were defined as presence of no scar (NS) , diffuse scar (DS) or focal cortical scars (FS) . During histopathological investigations we have looked at general structural changes, inflammatory changes (inflammation intensity grouped as G1,2 and 3), extracellular matrix content, smooth muscle content, ureteric diameter and wall thickness.
Results: Histologic examinations revealed that of the 110 ureterorenal units, 35 had no scars, (33%), 30 had diffuse scar (27%) and 45 had focal scars (40%). When scar rates were investigated for grade of reflux as low grade ( G1-2) vs high grade ( G 3, 4, 5) the scar rates was found as 39,5%, 90,3% respectively. The inflammation intensity at the highest level (grade 3) was observed in 66%, 28,9%, 36,4% of the kidneys with diffuse scar, focal and no scar respectively (p<0.03). The pathologic features according to different scar patterns are listed in the table.
Distal end pathological features
Ureteral wall thickness
↑↑↑, Grade 3
↑, Grade 1
↑, Grade 1
Conclusion: The findings in this study provide some evidence to improve our understanding of congenital scar formation and reflux resolution. This investigation shows that there are significant differences of histologic structure of the distal end of the ureters when they are classified as ureters of the kidneys with diffuse scar, focal scar and no scar. There is more intense inflammation, more collagen deposition, less smooth muscle content and increased ureteric wall thickness in the ureters of the kidneys with diffuse scar when compared to others with less or no scar. Presence of diffuse scar formation in the kidney is also reflected within whole renal unit and this is probably due to a developmental pathology rather than being an acquired one.
2011 American Academy of Pediatrics National Conference and Exhibition; 10/2011
[Show abstract][Hide abstract] ABSTRACT: Idiopathic nephrotic syndrome (INS) is a genetically heterogeneous group of disorders characterized by proteinuria, hypoalbuminemia, and edema. Because it typically results in end-stage kidney disease, the steroid-resistant subtype (SRNS) of INS is especially important when it occurs in children. The present study included 29 affected and 22 normal individuals from 17 SRNS families; genome-wide analysis was performed with Affymetrix 250K SNP arrays followed by homozygosity mapping. A large homozygous stretch on chromosomal region 12p12 was identified in one consanguineous family with two affected siblings. Direct sequencing of protein tyrosine phosphatase receptor type O (PTPRO; also known as glomerular epithelial protein-1 [GLEPP1]) showed homozygous c.2627+1G>T donor splice-site mutation. This mutation causes skipping of the evolutionarily conserved exon 16 (p.Glu854_Trp876del) at the RNA level. Immunohistochemistry with GLEPP1 antibody showed a similar staining pattern in the podocytes of the diseased and control kidney tissues. We used a highly polymorphic intragenic DNA marker-D12S1303-to search for homozygosity in 120 Turkish and 13 non-Turkish individuals in the PodoNet registry. This analysis yielded 17 candidate families, and a distinct homozygous c.2745+1G>A donor splice-site mutation in PTPRO was further identified via DNA sequencing in a second Turkish family. This mutation causes skipping of exon 19, and this introduces a premature stop codon at the very beginning of exon 20 (p.Asn888Lysfs*3) and causes degradation of mRNA via nonsense-mediated decay. Immunohistochemical analysis showed complete absence of immunoreactive PTPRO. Ultrastructural alterations, such as diffuse foot process fusion and extensive microvillus transformation of podocytes, were observed via electron microscopy in both families. The present study introduces mutations in PTPRO as another cause of autosomal-recessive nephrotic syndrome.
The American Journal of Human Genetics 06/2011; 89(1):139-47. DOI:10.1016/j.ajhg.2011.05.026 · 10.93 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Drug-induced interstitial nephritis is one of the causes of graft dysfunction in renal transplant recipients. Although commonly implicated as a cause of drug-induced interstitial nephritis in the general population, proton pump inhibitor-induced interstitial nephritis has not yet been reported in renal transplant recipients. Trimethoprim-sulfamethoxazole is responsible for most cases of interstitial nephritis in this population. Here, we describe the first case of proton pump inhibitor-related interstitial nephritis in a renal transplant recipient.
International Urology and Nephrology 05/2011; 44(6). DOI:10.1007/s11255-011-9972-3 · 1.52 Impact Factor