Nephron-sparing surgery for primary mucinous adenocarcinoma of renal pelvis in horseshoe kidney: A case report.

RATIONALE: Mucinous adenocarcinoma of the renal pelvis is extremely rare in malignancy of the renal pelvis, and <100 cases have been reported so far. However, horseshoe kidneys appear to be more prone to this mucinous gland metaplasia. Due to the lack of a specific diagnostic basis, it is difficult to confirm the diagnosis before surgery, which is easy to cause misdiagnosis. PATIENT CONCERNS: We report a 64-year-old male patient who found a cystic mass in the left kidney during physical examination. CT examination showed a horseshoe kidney with a cystic mass 9.5 × 8.0 cm in front of the left kidney, lacking obvious diagnostic features. DIAGNOSIS: It was misdiagnosed as cystic teratoma before the operation, and was diagnosed as mucinous adenocarcinoma of the renal pelvis through pathological examination after the operation. INTERVENTIONS: Resection of the tumor by nephron-sparing surgery and postoperative chemotherapy. OUTCOMES: No tumor recurrence was found at 6 years of follow-up. After 7 years, the patient had multiple metastases in the abdominal wall and peritoneum, and no tumor recurrence was found in the urinary system. The patient received chemotherapy again and survived well. LESSONS: The prognosis of nephron-sparing tumor resection for MRAP is not significantly different from that of radical nephroureterectomy + bladder cuff excision. Because it can reduce the risk that patients can not tolerate follow-up chemotherapy due to abnormal renal function after surgery, which may be more beneficial in patients with kidney abnormalities or chronic disease.

Horseshoe kidney with PLA2R-positive membranous nephropathy.

BACKGROUND: Horseshoe kidney (HSK) is a common congenital defect of the urinary system. The most common complications are urinary tract infection, urinary stones, and hydronephrosis. HSK can be combined with glomerular diseases, but the diagnosis rate of renal biopsy is low due to structural abnormalities. There are only a few reports on HSK with glomerular disease. Here, we have reported a case of PLA2R-positive membranous nephropathy occurring in a patient with HSK. CASE PRESENTATION: After admission to the hospital due to oedema of both the lower extremities, the patient was diagnosed with nephrotic syndrome due to abnormal 24-h urine protein (7540 mg) and blood albumin (25 g/L) levels. Abdominal ultrasonography revealed HSK. The patient's brother had a history of end-stage renal disease due to nephrotic syndrome. Therefore, the patient was diagnosed with PLA2R-positive stage II membranous nephropathy through renal biopsy under abdominal ultrasonography guidance. He was administered adequate prednisone and cyclophosphamide, and after 6 months of treatment, urinary protein excretion levels significantly decreased. CONCLUSION: The risk and difficulty of renal biopsy in patients with HSK are increased due to structural abnormalities; however, renal biopsy can be accomplished through precise positioning with abdominal ultrasonography. In the literature, 20 cases of HSK with glomerular disease have been reported thus far. Because of the small number of cases, estimating the incidence rate of glomerular diseases in HSK is impossible, and the correlation between HSK and renal pathology cannot be stated. Further studies should be conducted and cases should be accumulated to elucidate this phenomenon.

Effects of Eucommia ulmoides extract against renal injury caused by long-term high purine diets in rats.

Diets of overloaded purine-rich foods for a long time are one of the important reasons to cause renal lesions. Eucommia ulmoides is one of the traditional Chinese medicine herbs, which has been used to recover functions of the kidney. However, its mechanism remains unclear. The aim of this study was to explore the effects and protective mechanism of Eucommia ulmoides extract on renal injury caused by long-term high purine diets in rats. SD rats underwent an intragastric adenine (200 mg kg-1 d-1) administration for 9 weeks and were treated for 15 weeks. The results demonstrated that Eucommia ulmoides extract significantly reduced serum Cre and BUN levels in rats. H&E and Masson's trichrome stains showed notable lowering of the infiltration of inflammatory cells, the formation of fibrous tissues and collagen fibers, and improvement in the pathological morphology of kidneys. It also suppressed the protein and mRNA expressions of TGF-ß1 and α-SMA and enhanced E-cadherin expression. Meanwhile, Eucommia ulmoides extract prominently inhibited the mRNA expression of Col I, Col III, Col IV, TIMP-1, and TIMP-2 and promoted expressions of MMP-1, MMP-2 and MMP-9. Through our study, it is the first time to prove that Eucommia ulmoides extract could ameliorate renal interstitial fibrosis and may involve in the regulation of the extracellular matrix (ECM) degradation enzyme (MMPs/TIMPs) system, promotion of the expression of E-cadherin, and suppression of expressions of TGF-ß1 and α-SMA. The results provide a significant implication for the utilization of Eunomia Ulmoides extract as functional foods to enhance renal functions and improve renal injury caused by high purine diets.

Coexistence of a rare type of ectopic kidney with atypical renal vasculature.

Knowledge of anatomical anomalies is significant for all specialists in clinical practice and may prevent serious complications following medical procedures. This report presents the rare crossed fused renal ectopia (CFRE) with atypical renal vasculature in cadaver of a 68-year-old man. The ectopic kidney was located on right side with four renal veins, three renal arteries, two ureters, where one of them is double. The embryological background, as well as the potential clinical significance of this morphological variation, is discussed. An interventional radiological and surgical procedure should be appropriately implemented to treat anomalies of vessels and CFRE.

Ectopia renal cruzada con fusión. Un diagnóstico fortuito / Crossed renal ectopia with fusion. An incidental diagnosis

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Aggressive angiomyxoma of renal pelvis in a horseshoe-shaped kidney: Rare tumor at an unusual site.

Mesenchymal neoplasm is rare in renal pelvic region. Aggressive angiomyxomas are the neoplasms seen predominantly in female genital region. These tumors are locally aggressive with rare cases presenting with metastasis. Its occurrence in renal pelvis is extremely rare with only five cases reported in the literature. The present case is the sixth case reported in a 15-year-old girl who presented to the surgery department as a case of horseshoe kidney. The mass was excised and a diagnosis of aggressive angiomyxoma was rendered.

A deadly trap for para-aortic lymph node dissection in patients with horseshoe kidney as a complication: a case report.

Horseshoe kidney is a rare congenital renal dysplasia. It is often associated with various anatomical abnormalities, including renal vessel and ureter variability, which increase unpredictable surgical risks. This current report describes the case of a 42-year-old woman diagnosed as having cervical squamous cell carcinoma complicated by horseshoe kidney. She underwent laparoscopic radical hysterectomy, bilateral oophorectomy and lymph node dissection, including dissection of the pelvic, presacral and para-aortic lymph nodes. The surgery was challenging, but no serious complications occurred. Postoperative multi-slice computed tomography angiography confirmed the anatomical variation of the renal location, ureter and renal vessels. To our knowledge, this is the first reported case of cervical carcinoma complicated with horseshoe kidney.

Heparan sulfate in chronic kidney diseases: Exploring the role of 3-O-sulfation.

One of the main feature of chronic kidney disease is the development of renal fibrosis. Heparan Sulfate (HS) is involved in disease development by modifying the function of growth factors and cytokines and creating chemokine gradients. In this context, we aimed to understand the function of HS sulfation in renal fibrosis. Using a mouse model of renal fibrosis, we found that total HS 2-O-sulfation was increased in damaged kidneys, whilst, tubular staining of HS 3-O-sulfation was decreased. The expression of HS modifying enzymes significantly correlated with the development of fibrosis with HS3ST1 demonstrating the strongest correlation. The pro-fibrotic factors TGFß1 and TGFß2/IL1ß significantly downregulated HS3ST1 expression in both renal epithelial cells and renal fibroblasts. To determine the implication of HS3ST1 in growth factor binding and signalling, we generated an in vitro model of renal epithelial cells overexpressing HS3ST1 (HKC8-HS3ST1). Heparin Binding EGF like growth factor (HB-EGF) induced rapid, transient STAT3 phosphorylation in control HKC8 cells. In contrast, a prolonged response was demonstrated in HKC8-HS3ST1 cells. Finally, we showed that both HS 3-O-sulfation and HB-EGF tubular staining were decreased with the development of fibrosis. Taken together, these data suggest that HS 3-O-sulfation is modified in fibrosis and highlight HS3ST1 as an attractive biomarker of fibrosis progression with a potential role in HB-EGF signalling.

Robotic-assisted Laparoscopic Partial Nephrectomy in a Horseshoe Kidney. A Case Report and Review of the Literature.

Horseshoe kidney is a rare renal fusion anomaly, and because of limited mobilization of the kidney and its multiple arterial blood supplies, minimally invasive surgery for renal tumors can be challenging. We describe a case of a right-side oncocytoma in a horseshoe kidney managed robotically and review the literature of robotic-assisted laparoscopic surgical resection of kidney tumors in renal fusion anomalies. Robotic-assisted laparoscopic partial nephrectomy in a horseshoe kidney is feasible. Fusion-related limited mobility during the procedure, as well as an extremely variable blood supply, require meticulous planning. Multi-phase computed tomography and interactive 3D anatomical models are helpful tools to prepare for surgery.

Organ-Preserving Surgical Treatment of a Horseshoe Kidney Occupied by a Large Renal Cell Carcinoma with Extensive Venous Invasion: A Case Report.

The horseshoe kidney is one of the most common congenital disorders affecting the urogenital system. Following a fusion of the lower kidney poles, which in turn lead to the formation of an isthmus, this anatomical variation is accompanied by other characteristic properties like an incomplete ascension, ventral rotation of the pelvices as well as atypical vascular supply. Even though renal carcinoids and Wilms tumors are more common in horseshoe kidneys, the incidence of renal cell carcinomas seems to be unaffected. Here we report the case of a locally advanced renal cell carcinoma with extensive venous invasion occurring in a horseshoe kidney and its complex surgical management. The whole primary tumor as well as a majority of venous tumor thrombi could be removed by a combination of 2/3 nephrectomy and cavotomy with thrombectomy. During 1 year of follow-up, the patient neither suffered from a tumor relapse, nor did he require renal replacement therapy. Thus, we conclude that even in cases of RCC where advanced disease is associated with complex anatomical situations, organ-preserving surgical treatment should be pursued to achieve excellent functional and oncological results.

Evaluation of the accuracy of renal depth estimation formulas in horseshoe kidney.

Estimation formulas are usually used to calculate renal depth when glomerular filtration rate (GFR) is measured by the Gates method. Horseshoe kidney (HSK) anatomical structure is different from the normal form of the kidney. The existing formulas are based on the normal form. It is unknown whether the existing formulas are valid in HSK patients. This study was performed to estimate the accuracy of the existing 6 renal depth estimation formulas in HSK.Renal depth and total thickness (T, cm) of the body at the level of the kidneys were measured by CT in 94 HSK patients. Their sex, age, height (H, cm), and weight (W, kg) were recorded. The existing 6 estimation formulas were used to obtain estimated renal depth. Correlation coefficients, Bland-Altman analysis, and paired t test were performed between estimated and the CT measured renal depth.Estimated renal depths were all lower than the CT measured renal depths and there was significant difference between estimated and CT measured renal depth. The CT measured renal depth and estimated renal depth derived from Ma GY formula correlated best (right: r = 0.80, P < .01; left: r = 0.77, P < .01). The renal depth derived from Tonnesen formula was significantly lower than the CT measured renal depth. The agreement between the estimated renal depth derived from Tonnesen formula and the CT measured renal depth was the worst, with the mean difference of (right: -3.11 ±â€Š1.13 cm; left: -2.79 ±â€Š1.07 cm). The agreement between the estimated renal depth derived from Li Q formula and Ma GY formula and the CT measured renal depth was the best, with the mean difference of right: -1.68 ±â€Š1.09 cm; left: -1.32 ±â€Š1.06 cm and right: -1.59 ±â€Š1.01 cm; left: -1.59 ±â€Š0.99 cm, respectively. But the greatest error of the difference between Li Q formula and Ma GY formula estimated depth and the CT measured depth was up to -4.83 cm, and the estimated deviation is unacceptable.All the existing formulas do not fully apply to HSK. To provide reliable and accurate estimates of renal depth, we should develop a new formula to estimate the renal depth in HSK patients.

Minimal change disease in horseshoe kidney.

The horseshoe kidney is a frequent urological birth defect. The most frequent complications are urinary tract infections, stones and hydronephrosis. The occurrence of glomerular disease in horseshoe kidney is rare. Therefore, we report the first case of minimal change disease occurring in a patient with horseshoe kidney in literature. A 22-year-old Caucasian man without personal or family medical history admitted to the pneumology department for a pulmonary artery embolism. In presence of a generalized oedema, a biological assessment was performed yielding intense nephrotic syndrome with urine protein excretion 22g/day. The abdominal ultrasound revealed a horseshoe kidney. Hence a scanno-guided kidney biopsy was taken yielding minimal change disease. High dose steroids were started, then gradually tapered with good response. Horseshoe kidney is the most common renal fusion anomaly, with a prevalence of 0.25% among the general population. The occurrence of glomerular nephropathy in horseshoe kidney has been reported in few cases. We report the first case of minimal change disease occurring in a patient with horseshoe kidney in literature. The mechanism of the association between the horseshoe kidney and these renal pathologies could not be explained in the previous reports. There is no literature data indicating a high rate of glomerulonephritis in horseshoe kidneys. The co-incidence of two renal diseases in this patient can be only a coincidence. The question that arises is whether this glomerulopathy is associated or not with this anatomical abnormality. Further studies are needed to answer this question.

A second reported malignancy in a patient with Morquio syndrome

Morquio syndrome is a rare lysosomal storage disease that affects multiple organ systems. However, it is rarely associated with malignancy. We present the case of a 30-year old man with Morquio syndrome associated with gastric adenocarcinoma. This case also demonstrates two other findings that have not been previously described in patients with Morquio syndrome - malrotation of brainstem and cerebellum, without clinical neurologic deficit, and persistence of fetal lobulation in the kidneys.

Clear cell sarcoma of kidney involving a horseshoe kidney and harboring EGFR internal tandem duplication.

Clear cell sarcoma of kidney (CCSK) is a rare renal malignancy, previously unreported in horseshoe kidney (HSK). B-cell lymphoma 6 corepressor (BCOR) gene internal tandem duplication (ITD) was identified as a recurrent somatic alteration in approximately 85% of CCSKs. This and the YWHAE-NUTM2B/E fusion, the second most common recurrent molecular alteration in CCSK (10%), are considered to be mutually exclusive. However, there is a subset of CCSKs that do not harbor either the BCOR-ITD or YWHAE-NUTM2 translocation and lack known molecular alterations. Herein, we report the first case of CCSK arising in HSK and harboring epidermal growth factor receptor ITD.

Partial nephrectomy in horseshoe kidney: Primary carcinoid tumor.

Primary neuroendocrine carcinoma of the kidney is a rarely observed clinical condition because neuroendocrine cells are not found in kidney parenchyma. It's not clinically and radiologically possible to distinguish from other kidney tumors. Incidence with horseshoe kidney anomaly, it should be considered as a definitive diagnosis for the patients with this condition. In this case report, we reported about a carcinoid tumor in horseshoe kidney in a 37-year-old woman.

Left Renal Vein Compression and Horseshoe Kidney: An Extraordinary Association.

OBJECTIVE: Our aim was to report the importance of an awareness of renal artery variations if surgical procedures are indicated in this region. Presentation and Intervention: A 41-year-old female patient was admitted to our hospital for preoperative staging of the rectum carcinoma. A computed tomography (CT) scan clearly revealed the presence of normally positioned small right and left renal arteries, and a large aberrant renal artery originating from the distal abdominal aorta in a case with a horseshoe kidney (HSK). A multidetector CT angiography was performed using a 256-detector row CT scanner to obtain an image of vascular structures and associated pathologies. CONCLUSION: In this patient with HSK, the left renal vein compression was unusual and the CT examination provided a good delineation of vascular and urinary tract anomalies.

The horseshoe kidney: Surgical anatomy and embryology.

Horseshoe kidneys are a common, yet enigmatic, renal malformation. This review critically appraised the literature surrounding the embryology, etiology and clinical anatomy of horseshoe kidneys. The systematic literature search produced 104 articles, and 56 primary and further secondary references. There were several etiological theories regarding horseshoe kidneys. The established view was that during ascent, the kidneys come into close apposition as they pass through an arterial fork. Another possible mechanism related to lateral flexion of the trunk or rotation of the caudal embryo; the association of asymmetrical horseshoe kidneys with a number of vertebral conditions supported this hypothesis. More recent animal models implicated the notochord and sonic hedgehog signaling. Furthermore, it has been suggested that the isthmus may be the result of ectopic mesenchymal tissue. Surgical anatomy of the horseshoe kidney is complex, due to variability in location, orientation and blood supply. Both arterial and venous anatomy is highly variable. This raised the question of whether anomalous blood supply is the cause or result of abnormal renal position. In the majority of cases, the isthmus contained functional renal parenchyma. In over 90% of cases, fusion between the kidneys occurred at the lower pole. Despite commonly being quoted as 'held back by the inferior mesenteric artery' at L3, in reality the isthmus was only found immediately inferior to this in 40% of cases.