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In this study, we investigated the association between intraductal carcinoma of the prostate (IDCP) along with several histopathological features on prostate biopsy and downgrading of grade group 4 (GG4) prostate cancer (PCa) in patients with the highest grade tumor of GG4 PCa in at least one core. A total of 29 cases had the highest grade tumor of GG4 PCa and radical prostatectomy performed between 2016 and 2021. IDCP was detected in 11 out of 29 cases on biopsy. The cases without IDCP were more likely to be downgraded on prostatectomy than with IDCP, with statistical significance (88.9% vs 36.4%, p = 0.003). The proportions of the highest-grade tumors by length and cores involved, average numbers of PCa-positive cores, and mean patient's age did not differ between cases that were downgraded and not downgraded at prostatectomy. Our results suggest that the absence of IDCP on biopsy could be a predictor of downgrading at prostatectomy for patients with the highest grade tumor of GG4 PCa. [ABSTRACT FROM AUTHOR]

Prostate-specific membrane antigen positron emission tomography (PSMA PET) has been approved by the Food and Drug Administration (FDA) to identify prostate cancer in the setting of biochemical recurrence but can also identify other malignancies. 18F-PSMA PET has not been studied as a potential tool for hepatocellular carcinoma (HCC). We describe the case of a 76-year-old male with a rising prostate-specific antigen (PSA) after definitive prostate cancer treatment and no prior liver pathology who was incidentally found to have HCC on 18F-PSMA PET. [ABSTRACT FROM AUTHOR]

Chronic kidney disease (CKD) has a strong genetic component; however, the underlying pathways are not well understood. Dahl salt-sensitive (SS)/Jr rats spontaneously develop CKD with age and are used to investigate the genetic determinants of CKD. However, there are currently several genetically diverse Dahl SS rats maintained at various institutions and the extent to which some exhibit age-related CKD is unclear. We assessed glomerulosclerosis (GS) and tubulointerstitial fibrosis (TIF) in 3- and 6-mo-old male and female SS/JrHsdMcwi, BN/NHsd/Mcwi [Brown-Norway (BN)], and consomic SS-Chr 1BN/Mcwi (SS.BN1) rats, in which chromosome 1 from the BN rat was introgressed into the genome of the SS/JrHsdMcwi rat. Rats were fed a 0.4% NaCl diet. GS (31 ± 3% vs. 7 ± 1%) and TIF (2.3 ± 0.2 vs. 0.5 ± 0.1) were significantly greater in 6-mo-old compared with 3-mo-old SS/JrHsdMcwi rats, and CKD was exacerbated in males. GS was minimal in 6- and 3-mo-old BN (3.9 ± 0.6% vs. 1.2 ± 0.4%) and SS.BN1 (2.4 ± 0.5% vs. 1.0 ± 0.3%) rats, and neither exhibited TIF. In SS/JrHsdMcwi and SS.BN1 rats, mean arterial blood pressure was significantly greater in 6-mo-old compared with 3-mo-old SS/JrHsdMcwi (162 ± 4 vs. 131 ± 2 mmHg) but not SS.BN1 (115 ± 2 vs. 116 ± 1 mmHg) rats. In 6-mo-old SS/JrHsdMcwi rats, blood pressure was significantly greater in females. RNA-sequencing analysis revealed that inflammatory pathways were upregulated in isolated medullary thick ascending tubules in 7-wk-old SS/JrHsdMcwi rats, before the development of tubule pathology, compared with SS.BN1 rats. In summary, SS/JrHsdMcwi rats exhibit robust age-related progression of medullary thick ascending limb abnormalities, CKD, and hypertension, and gene(s) on chromosome 1 have a major pathogenic role in such changes. [ABSTRACT FROM AUTHOR]

A reliable diagnosis of amyloidosis is usually based on a tissue biopsy. With increasing options for specific treatments of the different amyloid diseases, an exact and valid diagnosis including determination of the biochemical fibril nature is imperative. Biopsy sites as well as amyloid typing principles vary and this paper describes methods employed at some laboratories specialised in amyloidosis in Europe, Japan and USA. [ABSTRACT FROM AUTHOR]

Background: The amyloidoses are a rare and heterogeneous group of disorders that are characterized by the deposition of abnormally folded proteins in tissues ultimately leading to organ damage. The deposits are mainly extracellular and are recognizable by their affinity for Congo red and their yellow-green birefringence under polarized light. Current classification of amyloid in medical practice is based on the amyloid protein type. To date, 36 proteins have been identified as being amyloidogenic in humans. Summary: in clinical practice, it is critical to distinguish between treatable versus non-treatable amyloidoses. Moreover, amyloidoses with a genetic component must be distinguished from the sporadic types and systemic amyloidoses must be distinguished from the localized forms. Among the systemic amyloidoses, AL continues to be the most common amyloid diagnosis in the developed world; other clinically significant types include AA, ALECT2, and ATTR. The latter is emerging as an underdiagnosed type in both the hereditary and wild-type setting. Other hereditary amyloidoses include AFib, several amyloidoses derived from apolipoproteins, AGel, ALys, etc. In a dialysis setting, systemic amyloid derived from β2 microglobulin (Aβ2M) should be considered, although a very rare hereditary variant has also been reported; several amyloidoses may be typically associated with aging and several iatrogenic types have also emerged. Determination of the amyloid protein type is imperative before specific therapy can be implemented and the current methods are briefly summarized. A brief overview of the target organ involvement by amyloid type is also included. Key Messages: (1) Early diagnosis of amyloidosis continues to pose a significant challenge and requires the participation of many clinical and laboratory specialties. (2) Determination of the protein type is imperative before specific therapy can be implemented. (3) While mass spectrometry has emerged as the preferred method of amyloid typing, careful application of immune methods is still clinically useful but caution and experience, as well as awareness of the limitations of each method, are necessary in their interpretation. (4) While the spectrum of amyloidoses continues to expand, it is critical to distinguish between those that are currently treatable versus those that are untreatable and avoid causing harm by inappropriate treatment. [ABSTRACT FROM AUTHOR]

Unilateral ischemia-reperfusion (UIR) injury leads to progressive renal atrophy and tubulointerstitial fibrosis (TIF) and is commonly used to investigate the pathogenesis of the acute kidney injury-chronic kidney disease transition. Although it is well known that contralateral nephrectomy (CNX), even 2 wk post-UIR injury, can improve recovery, the physiological mechanisms and tubular signaling pathways mediating such improved recovery remain poorly defined. Here, we examined the renal hemodynamic and tubular signaling pathways associated with UIR injury and its reversal by CNX. Male Sprague-Dawley rats underwent left UIR or sham UIR and 2 wk later CNX or sham CNX. Blood pressure, left renal blood flow (RBF), and total glomerular filtration rate were assessed in conscious rats for 3 days before and over 2 wk after CNX or sham CNX. In the presence of a contralateral uninjured kidney, left RBF was lower (P < 0.05) from 2 to 4 wk following UIR (3.6 ± 0.3 mL/min) versus sham UIR (9.6 ± 0.3 mL/min). Without CNX, extensive renal atrophy, TIF, and tubule dedifferentiation, but minimal pimonidazole and hypoxia-inducible factor-1α positivity in tubules, were present at 4 wk post-UIR injury. Conversely, CNX led (P < 0.05) to sustained increases in left RBF (6.2 ± 0.6 mL/min) that preceded the increases in glomerular filtration rate. The CNX-induced improvement in renal function was associated with renal hypertrophy, more redifferentiated tubules, less TIF, and robust pimonidazole and hypoxia-inducible factor-1α staining in UIR injured kidneys. Thus, contrary to expectations, indexes of hypoxia are not observed with the extensive TIF at 4 wk post-UIR injury in the absence of CNX but are rather associated with the improved recovery of renal function and structure following CNX. [ABSTRACT FROM AUTHOR]

Approaches to nephron-sparing surgeries (NSS) of renal lesions include partial nephrectomy (PN) and tumor enucleation (TE). Our objective was to examine the pathology of the pseudocapsule and status of the surgical margin in small renal masses treated by NSS and to correlate these findings with the surgical and oncological outcomes. All consecutive renal TE and PN specimens obtained during the period between January 2012 and December 2014, of which clinical follow-up was available, were included in this study. Pathologic features and clinical data were reviewed and analyzed. A total of 117 NSS specimens (59 EN, 58 PN) were reviewed. Clear cell renal cell carcinomas and paraganglioma had the thickest pseudocapsules (0.36 mm), while angiomyolipomas did not form a well-defined pseudocapsule. Other tumors were intermediate in their characteristics. The positive margin rate for TE and PN was 17.2 and 0 %, respectively. Compared to PN, TE involved a significantly shorter procedure time, less blood loss, and fewer post-operative complications. None of the patients from either group was found to have a local recurrence after follow-up imaging. Although positive surgical margins were more frequently seen in TE specimens, local tumor recurrence was comparable to PN. Thus, TE is a reasonable choice for pT1 renal tumors, especially for those without a prominent infiltrative growth pattern. [ABSTRACT FROM AUTHOR]

Mixed epithelial and stromal tumor (MEST) is a biphasic adult renal lesion composed of solid and cystic areas containing spindle cell stroma and epithelium that lines the tubules and cystic spaces. While most MEST lesions are benign, rare cases with malignant morphology and biology have been reported. We present a case of mixed epithelial and stromal tumor of the kidney (MEST) with extension into the inferior vena cava in a young adult male. We discuss the differential diagnosis of MEST in the context of other biphasic cystic renal lesions and the significance of vascular involvement in the setting of an otherwise benign tumor morphology. [ABSTRACT FROM AUTHOR]

Multilocular cystic renal cell carcinoma has been recently excluded from clear cell renal cell carcinoma (CCRCC) category and re-designated as multilocular cystic renal neoplasm of low malignant potential (MCRNLMP) due to its uniformly good outcomes. While strict distinction between MCRNLMP from predominantly cystic CCRCC (pc-CCRCC) is being emphasized, the significance of extensive true cystic component in CCRCC has not been investigated. Herein, we analyzed 57 MCRNLMP, 69 pc-CCRCC, and 46 non-cystic CCRCC. There were no statistically significant differences between the three subtypes in age, gender, and laterality. ISUP grades were 1 (73%) or 2 (27%) for MCRNLMP; for pc-CCRCC were 1 (31%), 2 (60%), and 3 (9%); and for non-cystic CCRCC were 1 (9%), 2 (52%), 3 (26%), and 4 (13%). MCRNLMP were either pT stage 1 (91%) or 2 (9%), pT stages for pc-CCRCC were 1 (92.5%), 2 (1.5%), and 3 (6%) and for non-cystic CCRCC were 1 (58.7%), 2 (6.5%), and 3 (34.8%). None of MCRNLMP patients developed recurrences or metastases, and only 1 contralateral kidney tumor and 1 metastasis developed in pc-CCRCC. In contrast, 19 patients with non-cystic CCRCC developed metastases (5-year PFS 58%, CI 38.3-73.5%), and 1 patient died of disease. Monosomy 3 was common in both MCRNLMP (3/3) and pc-CCRCC (6/7). This large series of MCRNLMP confirms its indolent behavior, shows that pc-CCRCC has significantly better prognosis than non-cystic CCRCC and may define the lower grade spectrum of CCRCC. We recommend that the presence and extent of CCRCC cystic component should be documented in the pathology report. [ABSTRACT FROM AUTHOR]

Adenoviruses are emerging as important viral pathogens in hematopoietic stem cell and solid organ transplant recipients, impacting morbidity, graft survival, and even mortality. The risk seems to be highest in allogeneic hematopoietic stem cell transplant recipients as well as heart, lung, and small bowel transplant recipients. Most of the adenovirus diseases develop in the first 6 months after transplantation, particularly in pediatric patients. Among abdominal organ recipients, small bowel grafts are most frequently affected, presumably due to the presence of a virus reservoir in the mucosa-associated lymphoid tissue. Management of these infections may be difficult and includes the reduction of immunosuppression, whenever possible, combined with antiviral therapy, if necessary. Therefore, an awareness of the pathology associated with such infections is important in order to allow early detection and specific treatment. We reviewed six transplant recipients (small bowel, kidney, and heart) with adenovirus graft involvement from two institutions. We sought to compare the diagnostic morphology and the clinical and laboratory findings. The histopathologic features of an adenovirus infection of the renal graft and one native kidney in a heart transplant recipient included a vaguely granulomatous mixed inflammatory infiltrate associated with rare cells showing a cytopathic effect (smudgy nuclei). A lymphocytic infiltrate, simulating T cell rejection, with admixture of eosinophils was also seen. In the small bowel grafts, there was a focal mixed inflammatory infiltrate with associated necrosis in addition to cytopathic effects. In the heart, allograft adenovirus infection was silent with no evidence of inflammatory changes. Immunohistochemical stain for adenovirus was positive in all grafts and in one native kidney. All patients were subsequently cleared of adenovirus infection, as evidenced by follow-up biopsies, with no loss of the grafts. Adenovirus infection can involve allografts as well as native organs in solid organ transplant recipients. Infection is associated with variable necrosis and acute inflammation, in addition to a rejection-like infiltrate. Hematuria in non-renal solid organ transplant recipients may be associated with adenovirus nephritis and clinically silent graft involvement. Prompt diagnosis (aided by immunohistochemistry (IHC) and serology), with specific treatment, can prevent graft loss. [ABSTRACT FROM AUTHOR]

Patients with renal cell cancer (RCC) develop metastatic spread in approximately 33% of cases. The clinical management of patients with metastatic RCC is complicated by the lack of significant efficacy from available therapies. Common sites of metastases include the lung, liver, bone, brain, and adrenal gland, with case reports detailing the capacity of RCC to appear almost anywhere in the body. More than one organ system is often involved in the metastatic process. Metastases may be found at diagnosis or at some interval after nephrectomy. Approximately 20% to 50% of patients will eventually develop metastatic disease after nephrectomy. A shorter interval between nephrectomy and the development of metastases is associated with a poorer prognosis. Patients with metastatic RCC face a dismal prognosis, with a median survival time of only 6 to 12 months and a 2-year survival rate of 10% to 20%. Recent advances in biologic response modifier therapy have brought new hope to a small percentage of patients who respond to this therapy and rekindled interest in cytoreductive nephrectomy as an integral part of the management of these patients. [ABSTRACT FROM AUTHOR]

The amyloidoses are a 'group' of disorders, all of which are associated with deposits that display similar staining and ultrastructural features and are toxic to tissues. Many proteins--currently 31 protein types and many more variants--have been shown to undergo such transformations. Among the various currently known amyloidoses, there are marked differences with regard to their pathogenesis and incidence, while the associated clinical picture is frequently overlapping. However, the therapies that are currently available are amyloid-type specific. The diagnosis of amyloidosis thus involves two steps: (i) a generic diagnosis, followed by (ii) an amyloid type-specific diagnosis or 'amyloid typing'. Immunofluorescence in frozen sections or immunohistochemistry (IHC) in paraffin sections has traditionally been used in the typing of amyloid. However, IHC of amyloid differs significantly from IHC in other areas of surgical pathology; both caution and experience are necessary for its interpretation. The rationale for the application of proteomic methods to amyloid typing lies in the relative abundance of amyloid proteins in tissue where, frequently, it is the 'dominant' protein. Proteomic techniques include the following steps: sample preparation, protein extraction and digestion into peptide fragments, followed by their subsequent separation and measurement by mass spectrometry (MS) and protein identification by informatics. The advantages aswell as the limitations of both methods--immunohistochemistry and MS-based proteomics--are discussed. The current recommendations for the application of proteomics in renal amyloidosis are summarized. [ABSTRACT FROM AUTHOR]

The diet-induced obesity (DIO) model is frequently used to examine the pathogenesis of obesity-related pathologies; however, only minimal glomerulosclerosis (GS) has been reported after 3 mo. We investigated if GS develops over longer periods of DIO and examined the potential role of hemodynamic mechanisms in its pathogenesis. Eight-week-old male obesity-prone (OP) and obesity-resistant (OR) rats (Charles River) were administered a moderately high-fat diet for 5 mo. Radiotelemetrically measured blood pressure, proteinuria, and GS were assessed. OP (n = 10) rats developed modest hypertension (142 ± 3 vs. 128 ± 2 mmHg, P < 0.05) and substantial levels of proteinuria (63 ± 12 vs. 12 ± 1 mg/day, P < 0.05) and GS (7.7 ± 1.4% vs. 0.4 ± 0.2%) compared with OR rats (n = 8). Potential hemodynamic mechanisms of renal injury were assessed in additional groups of OP and OR rats fed a moderately high-fat diet for 3 mo. Kidney weight (4.3 ± 0.2 vs. 4.3 ± 0.1 g), glomerular filtration rate (3.3 ± 0.3 vs. 3.1 ± 0.1 ml/min), and glomerular volume (1.9 ± 0.1 vs. 2.0 ± 0.1 μm³ - 10-6) were similar between OP (n = 6) and OR (n = 9) rats. Renal blood flow autoregulation was preserved in both OP (n = 7) and OR (n = 7) rats. In contrast, Nω-nitro-L-arginine methyl ester (L-NAME) administration in conscious, chronically instrumented OP (n = 11) rats resulted in 15% and 39% increases in blood pressure and renal vascular resistance, respectively, and a 16% decrease in renal blood flow. Minimal effects of L-NAME were seen in OR (n = 9) rats. In summary, DIO-associated GS is preceded by an increased hemodynamic sensitivity to L-NAME but not renal hypertrophy or hyperfiltration. [ABSTRACT FROM AUTHOR]

Small renal tumors are usually enwrapped in a pseudocapsule with well-confined borders, a feature that facilitates the performance of nephron-sparing surgeries (NSS). Our study was designed to evaluate the histologic features of the pseudocapsule of small renal tumors. One hundred seventy-eight renal tumors (≤4 cm), which were surgically removed by total nephrectomy, partial nephrectomy, or enucleation procedures during 2002-2013, were re-examined microscopically. Special attention was paid to the completeness and thickness of the pseudocapsule as well as the extra-pseudocapsular extension (EPE); components of the pseudocapsule and the intra-pseudocapsular vasculature (size/number) were evaluated. The data were analyzed according to the histological tumor types, Fuhrman grades, and sizes. Student's t test and chi-square tests were used for statistical analysis. Among 178 renal tumors, clear cell renal carcinomas (RCC) showed the thickest pseudocapsule (average 0.23 mm), while oncocytoma showed the thinnest (average thickness of 0.09 mm). Chromophobe RCC had the highest rate of EPE and the highest percentage of tumors with larger (≥0.2 mm) intra-pseudocapsular arteries. The EPE rate was also related to the nuclear grade ( p = 0.001). Muscular differentiation, reticulin, and collagen components were present in the fibrous stroma of the pseudocapsule. Our study suggests that clear cell RCC has the thickest pseudocapsule while oncocytoma has a poorly developed pseudocapsule, but shows the least infiltrative pattern. In small RCC (≤4.0 cm), the EPE rate is related to tumor grade but not to tumor size. Larger arterioles (≥0.2 mm) are encountered infrequently within the tumor pseudocapsule, with the highest percentage being found in chromophobe RCC and the lowest in papillary RCC. [ABSTRACT FROM AUTHOR]

ANG II is thought to increase the susceptibility to hypertension-induced renal disease (HIRD) via blood pressure (BP)-dependent and BP-independent pathways; however, the quantitative relationships between BP and HIRD have not been examined in ANG II-infused hypertensive rats. We compared the relationship between radiotelemetrically measured BP and HIRD in Sprague-Dawley rats (Harlan) chronically administered ANG II (300-500 ng.kg-1min-1, n = 19) for 4 wk versus another commonly employed pharmacological model of hypertension induced by the chronic administration of N"-nitro-L-arginine methyl ester (L-NAME, 50 mg.kg-1min-1, n = 23). Despite the significantly higher average systolic BP associated with ANG II (191.1 ± 3.2 mmHg) versus L-NAME (179.9 ± 2.5 mmHg) administration, the level of HIRD was very modest in the ANG II versus L-NAME model as evidenced by significantly less glomerular injury (6.6 ± 1.3% vs. 11.3 ± 1.5%, respectively), tubulointerstitial injury (0.3 ± 0.1 vs. 0.7 ± 0.1 injury score, respectively), proteinuria (66.3 ± 10.0 vs. 117.5 ± 10.1 mg/day, respectively), and serum creatinine levels (0.5 ± 0.04 vs. 0.9 ± 0.07 mg/dl, respectively). Given that HIRD severity is expected to be a function of renal microvascular BP transmission, BP-renal blood flow (RBF) relationships were examined in additional conscious rats administered ANG II (n = 7) or L - N A M E (n = 8). Greater renal vasoconstriction was observed during ANG II versus L-NAME administration (41% vs. 23% decrease in RBF from baseline). Moreover, administration of ANG II, but not L-NAME, led to a unique BP-RBF pattern in which the most substantial decreases in RBF were observed during spontaneous increases in BP. We conclude that the hemodynamic effects of ANG II may mediate the strikingly low susceptibility to HIRD in the ANG II-infused model of hypertension in rats. [ABSTRACT FROM AUTHOR]

Mixed epithelial and stromal tumor (MEST) is a distinctive adult biphasic neoplasm of the kidney characterized by the presence of solid and cystic areas composed of spindled stroma and epithelium lining tubules and cystic spaces respectively. Most MESTs are benign although sarcomatous transformation has rarely been reported. It has not been clearly established whether the epithelial component represents entrapped tubules or constitutes a true neoplastic component. We report an unusual case of a biphasic tumor of the kidney with a benign stroma and a focal component of papillary carcinoma arising in one of the cysts and discuss its pathogenesis. [ABSTRACT FROM PUBLISHER]

The purposes of this study are to evaluate the adequacy of pathologic lymph node (LN) staging in radical cystectomy specimens from patients with urothelial carcinoma of the bladder and to analyze the frequency of LN metastases among different anatomic regions. All radical cystectomies performed for primary urothelial bladder cancer over a 5-year period (January 2007-September 2012) at a single institution were reviewed. Particular attention was paid to the total number of LNs examined, the number and location of LNs with metastases (positive LNs), and the presence or absence of extranodal tumor extension and/or lymphovascular invasion in the cystectomy specimen. Results and data were analyzed with Origin 6.0 and Microsoft Office Excel 2007 software. A total of 248 radical cystectomies with 8,432 LNs were reviewed. A total of 60 (24 %) cases, with 274 positive LNs out of the 1,982 total (13.8 %), were identified with a male to female ratio of 6.5:1 (52 male, 8 female patients). The average number of LNs examined in each case was 33.0 ± 20.9 (range 5-112). The average number of positive LNs identified in each case was 4.5 ± 4.8 (range 1-26). Among all of the LNs, the hypogastric/obturator (internal iliac) LNs were the most commonly submitted (35.2 %) and also yielded the highest number of positive LNs (46.0 %). On average, for cases staged pN1 and pN2, there was one positive LN per 17.8 and 8.9 LNs examined from the primary drainage LNs, respectively. For pN3 cases, one out of 4.4 secondary drainage LNs was found to be positive. Similarly, one out of 4.0 distant LNs was found to be positive in cases with pM1 staging. Our study suggests that, on average, 23 LNs (including 18 primary drainage LNs and five secondary drainage LNs) should be submitted for optimal pN staging. For adequate pM1 staging, an average of four distal LNs should be evaluated. In total, an average of 27 LNs (23 for pN staging and 4 for pM staging) should be examined in radical cystectomy specimens. We also propose to stratify the number of positive LNs according to the drainage area. [ABSTRACT FROM AUTHOR]

Gouty panniculitis is a rare manifestation of gout. Clinically, it is characterized by indurated subcutaneous nodules in nonjoint areas. Pathologically, typical characteristic gouty tophi can be seen in subcutaneous tissue. It is postulated that gouty panniculitis develops as a consequence of uric acid accumulation in the body and localized inflammatory changes in subcutaneous tissue. We report a case of a 46-year-old man with 20-year history of gout, who developed multiple subcutaneous nodules over the abdomen and right groin/thigh area over a 2-year period. After a recent episode of congestive heart failure and acute renal failure, the nodules increased in size and the overlying skin became erythematous and ulcerated. Pathologic examination demonstrated typical tophi in the dermis and subcutaneous tissue. A review of the literature yielded fifteen similar cases that had been previously reported. We conclude that gouty panniculitis may be a manifestation of undertreated gout and may be exacerbated by the deterioration of other systemic functions. [ABSTRACT FROM AUTHOR]

Angiomyolipomas, composed of thick-walled blood vessels, smooth muscle, and adipose tissue, belong to the perivascular epithelioid cell neoplasms (PEComas), a family of tumors believed to be derived from perivascular epithelioid cells which co-express smooth muscle and melanocytic markers. Although most angiomyolipomas are benign, a subset of PEComas has metastatic potential. The pathologic and clinical spectrum of these tumors continues to evolve. We sought to evaluate a subset of renal angiomyolipomas with a minimal amount of fat. We studied 48 renal angiomyolipomas in 41 patients (33 females and 8 males). Based on the amount of adipose tissue, the lesions were categorized as fat-poor, fat-average, and fat-rich lesions (<25, 25-75, and >75 % of fat, respectively). Stains for smooth muscle actin, calponin, HMB-45, melanocyte-associated antigen PNL2, estrogen, and progesterone receptor were examined. Four patients (all females) had more than one lesion, four had coexistent uterine leiomyomata, two had coexistent renomedullary interstitial tumor, and males had only single lesions. Except for one woman, all lesions were sporadic. Twenty-nine were fat-poor (60 %) lesions; 8, fat-average (17 %) lesions; and 11, fat-rich (23 %) lesions. The fat content did not correlate with tumor size: the largest fat-poor and smallest fat-rich lesions were >6 and <2 cm, respectively. All lesions stained with smooth muscle actin and HMB-45; 41 % of tumors were positive for estrogen receptor (11 females and 1 male). No patient had metastases (follow-up 2-11 years). In our series, fat content in angiomyolipoma was not associated with tumor size. Fat-poor angiomyolipomas affected predominantly women and were morphologically and radiologically distinct as mimickers of malignancy. Whether they are biologically different from conventional tumors requires further studies. [ABSTRACT FROM AUTHOR]

Purpose: The cardiac and renal protective effects of phosphodiesterase-5 (PDE-5) inhibitors against ischemia-reperfusion injury have recently been demonstrated in animal studies. We evaluated the effect of pretreatment with the PDE-5 inhibitor zaprinast on warm renal ischemia in a rat model. Methods: Female Sprague-Dawley rats underwent concomitant right nephrectomy and left renal hilar occlusion for 30 minutes. Twelve animals were equally divided into three groups: Group 1 received no pharmacologic pretreatment, group 2 was pretreated with zaprinast 10 mg/kg, and group 3 was pretreated with zaprinast 20 mg/kg. Zaprinast was dissolved in 25% dimethyl sulfoxide and given as a single intraperitoneal injection 30 minutes before surgery. Serum blood urea nitrogen (BUN) and creatinine levels, histopathology, and TUNEL staining for apoptosis were assessed 24 hours postoperatively. Results: The mean creatinine level for groups 1, 2, and 3 was 0.73 mg/dL, 0.55 mg/dL, and 0.38 mg/dL, respectively. These values were not statistically different ( P=0.099). The mean BUN levels of 35.8 mg/dL for group 1, 27.3 mg/dL for group 2, and 23.3 mg/dL for group 3 were also statistically similar ( P=0.278). There were no objective differences in histopathologic evaluation or TUNEL staining between the groups. Conclusion: This study did not demonstrate a beneficial effect of zaprinast pretreatment on renal parameters after warm ischemic injury. [ABSTRACT FROM AUTHOR]

Primary benign vascular lesions of the kidney are uncommonly encountered in routine surgical pathology practice. They can, however, mimic malignancy or be an incidental finding adjacent to a malignancy. Fifteen specimens harboring 16 primary benign renal lymphatic/vascular lesions were identified from our files from 1999 to 2011 and subjected to a detailed pathologic evaluation and clinicopathologic correlation. Clinical and demographic data were available for all the 15 cases. There were ten males and five female patients with age range of 33-74 years (mean 54 years). Lesions ranged from 0.5 cm to 40 cm (average, 6.6 cm). There were six arteriovenous malformations (AVMs), four hemangiomas, three anastomosing hemangiomas, two lymphangiomas, and one solid intravascular papillary endothelial hyperplasia (IPEH). Five AVMs were located in the kidney parenchyma and one in the pelviureteric system. Additional associated lesions ranged from renal stones to renal cell carcinoma in two cases (one lymphangioma and one AVM). One AVM was associated with a capillary hemangioma in the vicinity, and another with a history of renal cell carcinoma in the contralateral kidney. Capillary hemangiomas and lymphangiomas were noninfiltrative and lacked cytological atypia and mitotic activity. Except for a renal pelvic AVM, all other renal AVMs radiologically mimicked malignancy. The patients had undergone partial or radical nephrectomies except for the renal pelvic AVM which was laparoscopically excised. To the best of our knowledge, none of the cases had any syndromic/systemic associations. Benign vascular lesions of the kidney are rarely seen in routine surgical pathology practice, partly because a vast majority of them are medically treated by embolization. However, lesions mimicking renal malignancy are subjected to surgery. They may exist as isolated lesions or coexist with malignant lesions either in the ipsilateral or the contralateral kidney. [ABSTRACT FROM AUTHOR]

Fibrinogen A a-chain (AFib) and apolipoprotein AI (AApoAI) amyloidosis due to variants in the AFib and ApoAI genes are the most common types of hereditary amyloidosis in Europe and the United States. Liver is the exclusive source of the aberrant amyloidogenic protein in AFib and responsible for supplying approximately half of the circulating variant ApoAI. Nephrotic syndrome and renal impairment due to renal amyloidosis are common disease manifestations; however, recent research provides evidence to support a more diverse and systemic disease phenotype, which in turn has implications in the management of the hereditary amyloidoses with solid organ transplantation and, in particular, liver transplantation. [ABSTRACT FROM AUTHOR]