Search Results (7)

Background and Clinical Significance: Spontaneous renal hematoma, also known as Wunderlich syndrome (WS), is a rare disease characterized by the acute onset of spontaneous renal hemorrhage into the subcapsular, perirenal, and/or pararenal spaces without a history of prior trauma. WS can be a life-threatening condition due to hemorrhagic shock; consequently, prompt diagnosis and a therapeutic approach are essential for favorable outcomes. Treatment ranges from conservative management to surgical intervention. The most common etiologies are neoplasms and vascular diseases, but WS can also be observed in patients undergoing hemodialysis. In patients with end-stage renal disease (ESRD), especially those on hemodialysis, acquired cystic kidney disease and renal cell carcinoma are among the primary causes of WS. Although less common, WS can develop in dialysis patients even in the absence of traditional (primary) risk factors. In general, patients with chronic kidney disease (CKD) have a paradoxical hemostatic profile, likely explaining their higher tendency to bleed, so WS can occur without existing predisposing factors. The multifactorial pathogenesis in these patients includes functional platelet abnormalities, intimal arterial fibrosis, chronic inflammation, and oxidative stress associated with ESRD. The use of hemodialysis-related antithrombotic medications could serve as another contributing factor increasing the risk of bleeding. Case Presentation: We present a case report of a 62-year-old male on chronic dialysis who developed sudden right-sided lumbar pain and hematuria during dialysis without evidence of prior trauma. Imaging revealed a large subcapsular hematoma of the right kidney. Further investigations did not reveal additional risk factors in this instance; however, his routinely used hemodialysis-related antithrombotic medications were potentially a contributing factor. Despite conservative treatment, his condition worsened, and the hematoma enlarged, requiring emergency nephrectomy. Postoperatively, his condition gradually improved. Conclusions: This case highlights the importance of considering WS in hemodialysis patients, even without the presence of traditional risk factors, as well as including WS in the differential diagnosis of acute abdominal pain. Full article

Renal cell carcinoma (RCC) is the most prevalent solid organ malignancy among kidney transplant recipients, demonstrating substantially higher incidence rates compared to those in the general population. Although RCC is most commonly diagnosed in native kidneys, its development in transplanted kidneys has an infrequent occurrence. The use of immunosuppressive therapies, pre-existing chronic kidney disease and the unique anatomical characteristics of transplanted kidneys represent considerable therapeutic challenges in managing RCC within this patient cohort. Open radical transplantectomy plays a crucial role in curative treatment for localized RCC, whereas nephron-sparing surgery (NSS), in selected cases, can provide similar oncologic benefits while preserving allograft function. Recently, laparoscopic and robotic surgical procedures have demonstrated favorable outcomes as viable alternatives to conventional open surgery. Furthermore, ablative therapies like radiofrequency ablation and cryoablation can be considered therapeutic alternatives for small renal masses, offering the benefit of preserving allograft function, especially in high-risk surgical candidates. Limited data exist regarding the management of metastatic RCC in transplant recipients. Surgery, withdrawal of immunosuppression and systemic adjuvant therapy could be considered. Management of RCC in transplanted kidneys requires a multidisciplinary approach considering patient-specific characteristics, tumor features and the developing landscape of both surgical and non-surgical options. Further research is needed to refine therapeutic strategies in order to achieve optimal oncological outcomes while preserving allograft function. Full article

Background: This study investigated the clinical features, underlying causes, and management of patients with spontaneous perirenal hemorrhage (Wunderlich syndrome; WS). Methods: We retrospectively reviewed the records of patients hospitalized for WS at a single tertiary center between 2011 and 2024. All patients were evaluated for non-traumatic perirenal hemorrhage identified on computed tomography (CT) in the emergency department. Clinical variables, including age, underlying diseases, symptoms, hemodynamic instability, and hospitalization course, were analyzed. Laboratory test results, as well as radiological and pathological findings, were reviewed. Results: The study included 46 events from 38 patients, with a median (IQR) follow-up period of 32 (4–82) months. The most common presenting symptom was flank pain, observed in 44 cases (95.7%). Renal lesions, including visible tumors, were detected in 25 cases (54.3%), while 13 cases (28.3%) exhibited perirenal hematoma without a distinct lesion. Among seven patients with hemodynamic instability (systolic blood pressure < 90 mmHg), one underwent emergency embolization, and four required emergency surgical exploration. Surgical intervention was performed in 13 cases (28.3%), all involving nephrectomy, while radiologic embolization was attempted in seven cases (15.2%), with one patient later requiring delayed nephrectomy. The final diagnosis revealed renal cell carcinoma in eight cases (six patients), angiomyolipoma in 11 cases (six patients), renal cysts in six cases, acquired cystic kidney disease in six cases, sarcoma in three cases, perivascular epithelioid cell tumor in one case, lymphoma in one case, and chronic pyelonephritis in four cases; no specific disease was identified in six cases. During follow-up, six patients died; four of these deaths were directly related to WS or its underlying etiologies. Conclusions: WS is a potentially life-threatening condition, with benign or malignant renal masses being the most common causes. Although the advancement of interventional techniques has led to an increasing number of cases being conservatively managed, the possibility of renal malignancy should always be considered. Full article

Down syndrome (DS), the most common survivable autosomal aneuploidy, is associated with a high prevalence of congenital anomalies of the kidney and urinary tract (CAKUT), significantly increasing the risk of chronic kidney disease (CKD). This review examines the diversity of CAKUT phenotypes reported in individuals with DS, focusing on anomalies affecting the kidney, ureter, bladder, and urethra. According to available literature, hydronephrosis is the most common renal anomaly, often secondary to other CAKUT phenotypes, followed by renal hypoplasia and glomerulocystic disease. Furthermore, obstructive uropathies are also frequent but usually lack detailed characterization in the literature. Key features of CAKUT in DS, including reduced kidney size, renal cystic diseases, acquired glomerulopathies, reduced nephron number, and immature glomeruli heighten the risk of CKD. Also, early detection of lower urinary tract dysfunction (LUTD) is critical to prevent progressive upper urinary tract damage and CKD. Despite the prevalence of CAKUT in DS, reported between 0.22% and 21.16%, there is a lack of standardized diagnostic criteria, consistent terminology, and extended follow-up studies. Systematic screening from infancy, including regular renal monitoring via urinalysis and ultrasound, plays a critical role in the timely diagnosis and intervention of CAKUT. To further enhance diagnostic accuracy and develop effective therapeutic strategies, increased awareness and focused research into the genetic factors underlying these anomalies are essential. Moreover, a multidisciplinary approach is indispensable for managing CAKUT and its associated complications, ultimately ensuring better long-term outcomes and an improved quality of life for individuals with DS. Full article

The progressive formation of single or multiple cysts accompanies several renal diseases. Specifically, (i) genetic forms, such as adult dominant polycystic kidney disease (ADPKD), and (ii) acquired cystic kidney disease (ACKD) are probably the most frequent forms of cystic diseases. Adult dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by multiple kidney cysts and systemic alterations. The genes responsible for the condition are known, and a large amount of literature focuses on the molecular description of the mechanism. The present manuscript shows that a multiscale approach that considers supramolecular physical phenomena captures the characteristics of both ADPKD and acquired cystic kidney disease (ACKD) from the pathogenetic and therapeutical point of view, potentially suggesting future treatments. We first review the hypothesis of cystogenesis in ADPKD and then focus on ACKD, showing that they share essential pathogenetic features, which can be explained by a localized obstruction of a tubule and/or an alteration of the tubular wall tension. The consequent tubular aneurysms (cysts) follow Laplace’s law. Reviewing the public databases, we show that ADPKD genes are widely expressed in various organs, and these proteins interact with the extracellular matrix, thus potentially modifying wall tension. At the kidney and liver level, the authors suggest that altered cell polarity/secretion/proliferation produce tubular regions of high resistance to the urine/bile flow. The increased intratubular pressure upstream increases the difference between the inside (Pi) and the outside (Pe) of the tubules (∆P) and is counterbalanced by lower wall tension by a factor depending on the radius. The latter is a function of tubule length. In adult dominant polycystic kidney disease (ADPKD), a minimal reduction in the wall tension may lead to a dilatation in the tubular segments along the nephron over the years. The initial increase in the tubule radius would then facilitate the progressive expansion of the cysts. In this regard, tubular cell proliferation may be, at least partially, a consequence of the progressive cysts’ expansion. This theory is discussed in view of other diseases with reduced wall tension and with cysts and the therapeutic effects of vaptans, somatostatin, SGLT2 inhibitors, and potentially other therapeutic targets. Full article

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary cystic kidney disease, with patients often having a positive family history that is characterized by a similar phenotype. However, in atypical cases, particularly those in which family history is unclear, a differential diagnosis between ADPKD and other cystic kidney diseases is important. When diagnosing ADPKD, cystic kidney diseases that can easily be excluded using clinical information include: multiple simple renal cysts, acquired cystic kidney disease (ACKD), multilocular renal cyst/multilocular cystic nephroma/polycystic nephroma, multicystic kidney/multicystic dysplastic kidney (MCDK), and unilateral renal cystic disease (URCD). However, there are other cystic kidney diseases that usually require genetic testing, or another means of supplementing clinical information to enable a differential diagnosis of ADPKD. These include autosomal recessive polycystic kidney disease (ARPKD), autosomal dominant tubulointerstitial kidney disease (ADTKD), nephronophthisis (NPH), oral-facial-digital (OFD) syndrome type 1, and neoplastic cystic kidney disease, such as tuberous sclerosis (TSC) and Von Hippel-Lindau (VHL) syndrome. To help physicians evaluate cystic kidney diseases, this article provides a review of cystic kidney diseases for which a differential diagnosis is required for ADPKD. Full article

Renal cell carcinoma (RCC) occurring in the setting of end-stage renal disease (ESRD) shows unique clinicopathological characteristics. The two most frequent types of ESRD-associated RCC are acquired cystic kidney disease-associated renal cell carcinoma (ACKD-RCC) and clear-cell papillary renal cell carcinoma (ccpRCC). Other types of RCC also occur in ESRD, albeit with different frequencies from the non-ESRD general population. The histological features of RCC do not vary in the setting of ESRD vs. non-ESRD, yet other findings, such as multifocality and multiple tumor types, are more frequent in ESRD. Studies have generated novel and important knowledge of the etiology, epidemiology, diagnosis, treatment, immunophenotype, and molecular characteristics of ESRD-associated RCC. Knowledge of these data is important for both pathologists and other physicians who may encounter ESRD patients with RCC. This review presents a comprehensive summary and update of the literature on RCC in ESRD, with a focus on the two most frequent types, ACKD-RCC and ccpRCC. Full article