Bilateral Nephron Sparing Surgery Research Articles

Bilateral Wilms tumor with anaplasia: A report from the Children's Oncology Group Study AREN0534.

The purpose of this study is to examine the outcomes in children with anaplastic bilateral Wilms tumor (BWT) from study AREN0534 in order to define potential prognostic factors and areas to target in future clinical trials. Demographic and clinical data from AREN0534 study patients with anaplasia (focal anaplasia [FA], or diffuse anaplasia [DA]) were compared. Event-free survival (EFS) and overall survival (OS) were reported using Kaplan-Meier estimation with 95% confidence bands, and differences in outcomes between FA and DA compared using log-rank tests. The impact of margin status was analyzed. Twenty-seven children who enrolled on AREN0534 had evidence of anaplasia (17 DA, 10 FA) in at least one kidney and were included in this analysis. Twenty-six (96%) had BWT. Nineteen percent had anaplastic histology in both kidneys (four of 17 DA, and one of 10 FA). Forty-six percent with BWT had bilateral nephron-sparing surgery (NSS); one child who went off protocol therapy, eventually required bilateral completion nephrectomies. Median follow-up for EFS and OS was 8.6 and 8.7years from enrollment. Four- and 8-year EFS was 53% [95% confidence interval (CI): 34%-83%] for DA; 4-year EFS was 80% [95% CI: 59%-100%], and 8-year EFS 70% [95% CI: 47%-100%] for FA. Three out of 10 children with FA and eight out of 17 children with DA had events. EFS did not differ statistically by margin status (p=.79; HR=0.88). Among the six children who died (five DA, one FA), all experienced prior relapse or progression within 18months. Events in children with DA/FA in the setting of BWT occurred early. Caution should be taken about interpreting the impact of margin status outcomes in the context of contemporary multimodal therapy. Future targeted investigations in children with BWT and DA/FA are needed.

Complex Chromosomal Rearrangement Involving Chromosomes 10 and 11, Accompanied by Two Adjacent 11p14.1p13 and 11p13p12 Deletions, Identified in a Patient with WAGR Syndrome

Three years ago, our patient, at that time a 16-month-old boy, was discovered to have bilateral kidney lesions with a giant tumor in the right kidney. Chemotherapy and bilateral nephron-sparing surgery (NSS) for Wilms tumor with nephroblastomatosis was carried out. The patient also had eye affection, including glaucoma, eye enlargement, megalocornea, severe corneal swelling and opacity, complete aniridia, and nystagmus. The diagnosis of WAGR syndrome was suspected. De novo complex chromosomal rearrangement with balanced translocation t(10,11)(p15;p13) and a pericentric inversion inv(11)(p13q12), accompanied by two adjacent 11p14.1p13 and 11p13p12 deletions, were identified. Deletions are raised through the complex molecular mechanism of two subsequent rearrangements affecting chromosomes 11 and 10. WAGR syndrome diagnosis was clinically and molecularly confirmed, highlighting the necessity of comprehensive genetic testing in patients with congenital aniridia and/or WAGR syndrome.

Surgery in Bilateral Wilms Tumor-A Single-Center Experience.

The treatment of bilateral Wilms tumors (BWT) involves curing the cancer, preserving long-term renal function, and maintaining a good quality of life. Established methods for achieving these goals include preoperative chemotherapy and nephron-sparing surgery (NSS). This study aimed to evaluate the experience of a single institution in treating patients with BWT. We analyzed cases of BWT treated at the Pediatric Oncology Institute-GRAACC-Federal University of São Paulo over a period of 35 years. Bleeding control was performed with manual compression of the renal parenchyma. Thirty-three patients were included in the study. Thirty cases were synchronous tumors. The mean age at diagnosis was 30.4 months (±22 m) and 66.7% were girls. The median follow-up period was 83 months. Neoadjuvant chemotherapy was the primary approach in most patients (87.9%), with a simultaneous upfront surgical approach performed in 84.8%. Most patients underwent bilateral NSS (70.4%). There were no early complications in this series, but 39.4% had clinical complications. The five-year survival rate was 76%. Therefore, it is clear that the surgical approach to BWT plays a crucial role in achieving good outcomes. However, it is difficult to standardize surgical techniques and technology may have the potential to enhance safety.

Treatment of bilateral Wilms’ tumor in children: how to improve the application of nephron-sparing surgery

To summarize the experience of nephron-sparing surgery (NSS) for bilateral Wilms tumors (BWT) in children. This study included children with BWT admitted to our hospital between January 2008 and June 2022. The details of the treatments and outcomes were analyzed. In all, 70 patients (39 males and 31 females) were enrolled, including 66 patients with synchronous tumors and 4 patients with metachronous tumors. The median age at diagnosis was 13 (3-75) months. Overall, 59 patients received preoperative chemotherapy and 45.8% (54/118) of the 118 sides of WT achieved a partial response (PR). Of the 70 patients, 48 (68.6%) underwent bilateral NSS and 22 (31.4%) underwent unilateral NSS and contralateral total nephrectomy. The proportion of bilateral NSS in the preoperative chemotherapy group was significantly higher than in the non-chemotherapy group (P = 0.031). Additionally, there were 26, 25, 14, and 5 cases of stage I, stage II, stage III, and stage IV, respectively. Among the 70 children, 16 had a recurrence, and 8 died. The 4years EFS and OS were 67.9% and 89.3%, respectively. The long-term survival rates of patients with BWT improved. Hence, preoperative chemotherapy should be administered to enhance the use of NSS in BWT.

Nephron sparing surgery outcomes in Wilms' tumor: is it ready for primetime?

Radical nephrectomy is the gold standard in Wilms tumor (WT) treatment and is combined with adjuvant treatment in early stage disease or performed after neo adjuvant therapy in advanced disease. With the development of novel adjuvant and neoadjuvant therapeutic strategies, there is increasing interest in organ preserving procedures in several adult malignancies. Potential long-term complications of living with a single kidney include hyperfiltration syndrome, hypertension, and fluid retention. If NSS (Nephron Sparing Surgery) were to be adopted for the treatment of WT it would be necessary to preserve the gains in Overall Survival (OS) seen with current treatment protocols. With this in mind we undertook a study of outcomes of NSS performed in the USA using a large population-based registry. We retrospectively queried the SEER Research Plus 18-registries Database 2010-2018 for patients 18years old or younger with Wilms tumor as determined by ICD-O-3 code 8960. Clinical and demographic data was extracted, and statistical analysis was performed in GraphPad PRISM 9 with bivariate analysis and log-rank analysis to determine survival. 1087 patients with Wilms tumor were identified. 73 (6.72%) underwent nephron-sparing surgery. The final cohort consisted of 45 patients (64%) who underwent unilateral NSS and 25 patients (36%) who underwent bilateral NSS. Three were excluded from analysis due to non-renal origin of tumor. Mean age was 3.214years (std dev 3.807). Demographics were similar between the groups, apart from younger age associated with bilateral tumor (p = 0.0441). No differences were found between radiation use (p = 0.4280), chemotherapy use (p = 0.5479), tumor size (p = 0.2186), positive regional lymph nodes (p = 0.707). Log-rank analysis demonstrated that cancer-specific survival was not significantly different between unilateral and bilateral NSS or radical nephrectomy (p = 0.4539). NSS may preserve renal function long-term, an important consideration in the pediatric population. Prospective study is necessary to select the appropriate patient subsets who may benefit from NSS in WT.

Extended Chemotherapy Followed by Staged Bilateral Nephron Sparing Surgery in Advanced Bilateral Wilms Tumor: Individualising Treatment Options for Optimal Outcome

Extended Chemotherapy Followed by Staged Bilateral Nephron Sparing Surgery in Advanced Bilateral Wilms Tumor: Individualising Treatment Options for Optimal Outcome

Bilateral Wilms’ tumour: An international comparison of treatments and outcomes

Introduction:Wilms’ tumour is the most common childhood renal malignancy, with 5–10% of cases presenting bilaterally 1. However, there is currently no consensus between centres on optimal management of bilateral Wilms’ tumours. This is an international multi-centre case series comparing management and outcomes of bilateral Wilms’ tumours between low-income centres (LIC) and high-income centres (HIC).Methods:Patients with bilateral Wilms' tumour were identified from four tertiary referral centres internationally. Data were collected on baseline characteristics, disease status, treatment used and clinical outcomes. Results were compared between individual centres as well as between groups of low-income centres (LIC) and high-income centres (HIC).Results:Data were collected for forty patients. Most patients received preoperative chemotherapy (n = 38, 95%). The most common surgical procedures were bilateral nephron-sparing surgery (n = 10, 25%) and nephrectomy with partial nephrectomy (n = 20, 50%). Ten-year survival after treatment was as follows: LIC's n = 13 (65%); HIC's n = 20 (100%) (p = 0.01).Discussion:Ten-year survival was significantly higher in HIC's. Our results show this may be caused by patient factors such as later presentation with more advanced disease in low-income centres. This comparative case series is the first to report on a large number of cases from multiple international centres, and to compare key outcomes.

Synchronous sporadic bilateral multiple chromophobe renal cell carcinoma accompanied by a clear cell carcinoma and a cyst: A case report

BACKGROUNDRenal cell carcinomas are usually unilateral. However, they are bilateral in 2% to 4% of sporadic cases and is considerably more common in familial cases. Synchronous sporadic bilateral multiple chromophobe renal cell carcinoma (CHRCC) with different subtypes is rare.CASE SUMMARYIn this case report, we describe a case of synchronous bilateral CHRCC with two histological variants, accompanied by a clear cell carcinoma and a cyst in a 50-year-old male. The patient underwent retroperitoneal laparoscopic bilateral nephron-sparing surgery and there was no serious postoperative renal dysfunction.CONCLUSIONWe report a rare case of synchronous bilateral CHRCC with two histological variants associated with a clear cell carcinoma and a cyst.

Technical aspects of Nephron-Sparing Surgery (NSS) in children with bilateral centrally located renal tumors

Bilateral Nephron-Sparing Surgery (NSS) is the gold standard as surgical treatment for children suffering from bilateral renal tumors. Whereas this approach is easy to achieve in peripheral tumors, it represents a relevant challenge in lesions that are centrally located. Involvement of the collecting system, localization deep within the renal parenchyma, and proximity to the central renal vessels are often difficult to deal with. A relevant number of patients thus receive unilateral total nephrectomy or undergo incomplete resections. However, surgery can often be performed successfully through experienced surgeons in centers of excellence, even though conditions for resection appear dismal on preoperative imaging. In this article, the authors describe technical aspects of Nephron-Sparing Surgery in complex conditions of bilateral pediatric renal tumors.

Complications Following Nephron-Sparing Surgery for Wilms Tumor

PurposeBecause of the increasing use of nephron-sparing surgery (NSS) in bilateral Wilms tumor, we sought to review the early postoperative complications associated with NSS. MethodsA retrospective review of patients who underwent NSS at our institution from 2000 to 2017 was performed. For comparison, a cohort of patients who underwent radical nephrectomy (RN) was also reviewed. Early (30-day) postoperative complications and oncologic outcomes were assessed. ResultsFifty-five patients underwent either bilateral (46) NSS or unilateral (9) NSS owing to prior resection or congenital solitary kidney. Fifty-four patients who underwent unilateral RN were also evaluated. Twenty NSS patients (36.4%) experienced 21 postoperative complications, including prolonged urine leak (9), infection (8), transient renal insufficiency (1), and intussusception (3). Seven RN patients (13.0%) experienced surgical complications, including infection (4) and intussusception (3). Average intraoperative blood loss was significantly greater in NSS as compared to RN (483.51 ± 337.92 mL and 278.15 mL ± 390.25, respectively, p < 0.001), as was the incidence of positive tumor resection margins (20 [36.4%] and 12 [22.2%], respectively, (p = 0.037). ConclusionsIn our experience, prolonged urine leak, intraoperative blood loss, and positive margins were more frequent in patients undergoing NSS as compared to RN. However, the complications were successfully managed, suggesting that an aggressive approach to NSS in patients with bilateral Wilms tumor is safe and appropriate. Level of evidenceLevel III Type of studyTreatment study.

Experience in diagnosis and treatment of non-hereditary bilateral synchronous renal carcinoma

Objective To investigate the principles of diagnosis and treatment of non-hereditary bilateral synchronous renal cell carcinoma. Methods This retrospective study analyzed 36 cases of non-hereditary bilateral synchronous renal cell carcinoma in our hospital from January 2008 to December 2016, including 30 males and 6 females. A total of 74 renal tumors were found, in which 34 patients had bilateral single kidney tumor and 2 patients had two tumors in one kidney. The diameter of tumors ranged from 1cm to 11 cm, with an average of (6.8±4.1)cm. The patients that underwent nephron-sparing surgery(NSS) got 4-12 points, with an average of (6.1±3.4) points in R. E.N.A.L. score and 3-13 points, with an average of (6.9±3.7) points in Zhongshan score. The patients are classified into 4 groups according to operation methods. In group A, 16 patients underwent bilateral NSS, which the preoperative creatinine was 63-103 μmol/L with an average of (80.9±11.4)μmol/L. In group B, 7 patients underwent one side of NSS before contralateral radical nephrectomy (RN), which preoperative creatinine was 59-87 μmol/L with an average of (75.7±8.9)μmol/L. In group C, 7 patients underwent one side of RN before contralateral NSS, preoperative creatinine was 57-107 μmol/L, with an average of (77.6±19.2)μmol/L. In group D, 6 patients underwent one side of NSS or RN and spare the contralateral side, 2 of which shifted from NSS to RN after finding tumor invaded pelvis and upper ureter during surgery. Of all the 16 patients with bilateral NSS, 4 patients underwent surgery on the side where tumor had a higher score in the first phase and then the side where tumor had a lower score in the second phase, 11 underwent surgeries in an opposite order. One patient underwent bilateral NSS simultaneously. Group A, B and C are taken into final analysis. Result All the 30 patients underwent surgery successfully. The operation time of NSS ranged from 60 to 110 min with an average of (88.6±23.6)min and RN ranged from 40 to 90 min with an average of (72.3±21.4)min. The warm ischemia time of NSS was 12-40 mins with an average of (29.5±9.7)min. The creatinine of Group A was 62-117 μmol/L with an average of (89.4±15.8)μmol/L and 57-392 μmol/L with an average of (129.6±74.9)μmol/L one month after the first and second surgery respectively. The creatinine of Group B was 64-115 μmol/L with an average of (94±14.4)μmol/L and 93-453 μmol/L with an average of (190.4±117.2)μmol/L one month after the first and second surgery respectively. The creatinine of Group C was 84-113 μmol/L with an average of (90.1±12.1)μmol /L and 88-156 μmol/L with an average of (121.4±24.8)μmol/L one month after the first and second surgery respectively. One patient in Group B and C developed lung metastases. One patient in Group B occurred oliguria after the second stage of surgery, and gradually improved after one week of hemodialysis. The creatine showed no significant difference among Group A, B and C before operation, after the first and second stage(P>0.05). Postoperative hospital stay after the first stage surgery was 3-16 days with an average of (6.7±3.4)d, and 3-16 d with an average of (6.2±3.2)d after the second stage, respectively. Conclusions In principle, bilateral renal tumors should be treated with NSS, which can protect renal functions as much as possible. Among patients who can undergo bilateral NSS, the first-stage surgery should be operated on the simpler and easier side to preserve the kidney of one side as much as possible to lay a good foundation for the second stage surgery. Among patients who undergo one side of RN and the other side of NSS, NSS is recommended for the first stage, and RN for the contralateral second stage after the renal function of the operated side was restored. Key words: Renal carcinoma; Surgery; Nephron-sparing surgery

Bilateral Wilms tumors: Treatment results from a single center.

Aydın B, Akyüz C, Yalçın B, Ekinci S, Oğuz B, Akçören Z, Yıldız F, Varan A, Kurucu N, Büyükpamukçu M, Kutluk T. Bilateral Wilms tumors: Treatment results from a single center. Turk J Pediatr 2019; 61: 44-51. The management of bilateral Wilms tumor (BWT) is challenging, particularly due to its presentation at a younger age, rarity, and difficulty for treatment decisions and surgical evaluation comparing to unilateral WT. In this study, the outcome of BWT patients from a single center who were treated by the Turkish Pediatric Oncology Group (TPOG) Wilms Tumor Regimen were retrospectively reviewed. From 1990 to 2016, 30 patients with synchronous BWT were treated with a preoperative chemotherapy of vincristine and actinomycin-D (VA). Chemotherapy was continued until safe nephron sparing surgery (NSS) could be performed for as long as radiological tumor response continued; otherwise, the chemotherapy was intensified by adding doxorubicin (D) alternating with VA every 6 weeks. The median followup of patients was 59 months (4-297 months). The median duration of preoperative chemotherapy was 81 days and ranged between 14 days and 198 days. Preoperative chemotherapy was modified in seven patients (23%) to the VAD regimen. Twenty-two patients (73%) had a radical nephrectomy on the larger tumor and NSS on the contralateral kidney, and 6 patients (20%) had bilateral NSS. Postoperative tumor stages for stage I, II and III were 60%, 22% and 14%, respectively. The 5-year event free survival (EFS) rates were 100%, 90% and 51% for stages I, II and III (p=0.02), respectively. Unfavorable histology and nephrogenic rests were reported in 20% and 20% of patients, respectively. The 5-year overall survival (OS) and EFS rates were 50% and 25%, respectively, in patients with anaplasia, while the same rates were 96% and 96% in patients with favorable histology tumors (p=0.05 and p < 0.001). The 10-year EFS and OS rates for all patients were 82% and 86%, respectively. Our results are comparable with the literature. VA is effective as initial preoperative treatment of BWT and allows for safe resection.

Synchronous Bilateral Wilms Tumor: Five-Year Single-Center Experience with Assessment of Quality of Life.

Context:Synchronous Bilateral Wilms tumor (sBWT).Aims:This study aimed to assess the outcome of patients with sBWT treated on SIOP protocol.Settings and Design:Retrospective and prospective randomized study.Subjects and Methods:SIOP 93-01 protocol was used to study nine patients of sBWT in a single center and followed up over a period from 2 to 5 years.Statistical Analysis Used:Unpaired t-test and Mann–Whitney U-test were used for analysis.Results:Of nine patients, six were included in the study as three patients lost to follow-up. Among the six patients, there were four girls and two boys with a median age of 2 years. Mean regression in the size of tumor was 87% in four out of six patients. Tumor with unfavorable histology showed 32% response (ratio of favorable: unfavorable histology 2:1). Event-free survival rate was 81.3% and overall survival was 90% over 2–5 years. Recurrence was seen in two patients of whom one had Denys–Drash syndrome. Mean DTPA glomerular filtration rate was 91.4/ml/min/1.73 m2 preoperatively and that of 3 months after completion of treatment was 84/ml/min/1.73 m2. Health-related quality of life (HRQOL) using Pediatric Quality of Life Inventory and Lansky Play Performance Scale revealed significant improvement results of all functioning domains such as physical, social, emotional, and school subscales with P < 0.05 and performance scale (P < 0.04).Conclusions:We suggest SIOP protocol for sBWT and bilateral nephron-sparing surgery in two stages. However, long-term follow-up is required to assess the ultimate renal function outcome. HRQOL is an essential guide in improving the conditions of pediatric cancer survivors.

Bilateral Wilms Tumor: A Surgical Perspective.

Historically, the management of bilateral Wilms tumor (BWT) was non-standardized and suffered from instances of prolonged chemotherapy and inconsistent surgical management which resulted in suboptimal renal and oncologic outcomes. Because of the risk of end-stage renal disease associated with the management of BWT, neoadjuvant chemotherapy and nephron-sparing surgery have been adopted as the guiding management principles. This management strategy balances acceptable oncologic outcomes against the risk of end-stage renal disease. A recent multi-institutional Children’s Oncology Group study (AREN0534) has confirmed the benefits of standardized 3-drug neoadjuvant chemotherapy and the utilization of nephron-sparing surgery in BWT patients; however, less than 50% of patients underwent bilateral nephron-sparing surgery. The coordination of neoadjuvant chemotherapy and the timing and implementation of bilateral nephron-sparing surgery are features of BWT management that require collaboration between oncologists and surgeons. This review discusses the surgical management strategy in the context of BWT disease biology, with an emphasis on timepoints during therapy at which surgical decision making can greatly impact this disease and minimize long-term toxicities.

Clinical features, treatment, and outcomes of bilateral Wilms' tumor: A systematic review and meta-analysis

BackgroundWilms' tumor(WT) is the most common malignant renal tumor of childhood. Despite the good prognosis of WT, bilateral Wilms' tumor (BWT) still has a poor outcome. We systematically reviewed the literature on BWT, aiming to define its clinical features, treatment, and outcomes. MethodsPubMed, OVID EMbase, Web of Science, and Cochrane Library were systematically searched for studies published from 1980 to 2017. Case series and comparative studies reported clinical data of BWT patients were included. ResultsA total of 32 studies comprising 1457 patients were retained for primary outcome. Hemihypertrophy, cryptorchidism, and Beckwith–Wiedemann syndrome(BWS) are the most common congenital anomalies and syndrome. 86% of patients had favorable histology (FH). Patients with local stage I or II accounted for 64%, and 12.6% had metastasis at diagnosis. Bilateral nephron-sparing surgery (NSS) was achieved in 33.8%. Recurrence and renal failure occurred in 20% and 8%. The overall survival (OS) was 73%. In comparative studies, OS of patients undergoing bilateral NSS was similar to that of other operation types. ConclusionPrognosis of BWT has been improved but is significantly poorer than WT. Bilateral NSS was recommended by most centers to preserve more renal volume. However, finding a balance between retaining renal function and avoiding recurrence remains a question. Type of studySystematic review. Level of evidenceLevel IV.

Retrospective analysis to determine outcomes of patients with bilateral Wilms tumor undergoing nephron sparing surgery: 15-year tertiary single-institution experience.

To describe our clinical experience with nephron sparing surgery (NSS) for bilateral Wilms tumor and evaluate the outcomes of patients treated at one of the largest pediatric medical centers in China. Medical records of children with bilateral Wilms tumor undergoing NSS in the Children's Hospital of Chongqing Medical University during a 15-year period were retrospectively analyzed. Data collected were composed of age at surgery, tumor response, tumor rupture during resection, final pathologic margins, use of radiation therapy, pathology reports, renal function, and patient survival. A total of 18 eligible patients (10 males, 8 females) with bilateral Wilms tumor at a mean age of 2.28 ± 1.12years were identified. The administration of preoperative chemotherapy did not result in universally successful outcomes. All children underwent successfully unilateral or bilateral NSS, of which one had positive pathologic margins and five received radiation therapy postoperatively. The rates of tumor rupture and positive lymph nodes involvement were 11.1 and 19.4%, respectively. The pathological study showed favorable histology and unfavorable histology in 32 and 4 kidneys, respectively. The 4-year event-free survival and overall survival rates were 68.18 and 85.56%. In univariable analysis, tumor histology (p = 0.0028) and disease stage (p = 0.0303) appeared significantly associated with overall survival. After a median follow-up period of 41.5months (range 10-89), three of the surviving patients were diagnosed with hypertension and one had renal insufficiency. Our experience suggests that NSS has become a feasible and effective option with good oncologic outcomes. Further research, ideally in a multicenter randomized manner, is warranted to better assess the role of NSS in this challenging clinical scenario.

Outcome and renal function following salvage surgery for bilateral Wilms tumor

Objective: The aim of this study was to determine the surgical outcomes and renal function following salvage surgery for bilateral Wilms tumor (BWT). Summary background data The challenge for the surgeon treating BWT lies in striking a fine balance between renal preservation and oncological clearance.Methods: This is a retrospective review of medical records in a tertiary care hospital in India. Nine children with BWT who presented between 2005 and 2015 were reviewed and followed up through telephone. Survival rates were calculated using the Kaplan–Meier method. A P value of less than 0.05 was considered statistically significant.Results: Seven (78%) of nine children were boys and two (22%) were syndromic. Six (67%) children presented at less than 1 year of age. Eight (89%) children presented with an abdominal mass. There were no metastases at presentation. All children underwent trucut biopsy and neoadjuvant chemotherapy. Six children underwent surgery: four underwent bilateral nephron sparing surgery (NSS) and two underwent unilateral nephrectomy with contralateral NSS. Tumor recurred in two children. The mean follow-up was 38 months (range: 5–108 months). Creatinine clearance (CrCl) improved postoperatively in all children. Postoperative hypertension was transient and resolved with improvement in CrCl.Conclusion: Children with BWT in the Indian subcontinent may be younger than those in the rest of the world. NSS yields good outcomes even for recurrences. Postoperative hypertension is transient in the majority of patients and correlated with improvement in CrCl. Prognosis is related to operability and syndromic association.Keywords: bilateral Wilms, creatinine clearance, hypertension, nephron sparing surgery

21 - Gain-of-Function SAMD9L Mutations Cause a Syndrome of Cytopenia, Immunodeficiency, Myelodysplastic Syndrome and Neurological Symptoms

Because of the increasing use of nephron-sparing surgery (NSS) in bilateral Wilms tumor, we sought to review the early postoperative complications associated with NSS.A retrospective review of patients who underwent NSS at our institution from 2000 to 2017 was performed. For comparison, a cohort of patients who underwent radical nephrectomy (RN) was also reviewed. Early (30-day) postoperative complications and oncologic outcomes were assessed.Fifty-five patients underwent either bilateral (46) NSS or unilateral (9) NSS owing to prior resection or congenital solitary kidney. Fifty-four patients who underwent unilateral RN were also evaluated. Twenty NSS patients (36.4%) experienced 21 postoperative complications, including prolonged urine leak (9), infection (8), transient renal insufficiency (1), and intussusception (3). Seven RN patients (13.0%) experienced surgical complications, including infection (4) and intussusception (3). Average intraoperative blood loss was significantly greater in NSS as compared to RN (483.51 ± 337.92 mL and 278.15 mL ± 390.25, respectively, p < 0.001), as was the incidence of positive tumor resection margins (20 [36.4%] and 12 [22.2%], respectively, (p = 0.037).In our experience, prolonged urine leak, intraoperative blood loss, and positive margins were more frequent in patients undergoing NSS as compared to RN. However, the complications were successfully managed, suggesting that an aggressive approach to NSS in patients with bilateral Wilms tumor is safe and appropriate.Level IIITreatment study.

Is Nephron Sparing Surgery Justified in Wilms Tumor With Beckwith-Wiedemann Syndrome or Isolated Hemihypertrophy?

Patients with Beckwith-Wiedemann syndrome (BWS) or isolated hemihypertrophy (HH) treated for a Wilms tumor (WT) carry an increased risk of developing metachronous lesion. There are no guidelines on precise indications for nephron sparing surgery (NSS) in unilateral WT (UWT). The objective of this retrospective study was to delineate the indications of NSS in patients with BWS/HH treated for WT and to evaluate their outcome. All cases of BWS/HH treated for a WT according to SIOP protocols from 1980 to 2013 were reviewed. Patients were divided into two groups (G): isolated UWT (G1) and bilateral lesions (G2) with two subgroups: bilateral tumors suspected of malignancy (G2a), and unilateral tumor suspected of malignancy with contralateral nephroblastomatosis (G2b). Forty-six patients were included (34 G1, three G2a, and nine G2b). Nine NSS and 25 total nephrectomies (TN) were performed in G1, two bilateral NSS and one NSS with contralateral TN in G2a, and eight NSS and one TN in G2b. The 3-year event-free survival was 92.3% (95% CI [77.9-97.5%]). One death occurred after a local relapse following a TN for a stage III stromal WT (G1) and another after a combined local and distant relapse following a NSS for a stage I diffuse anaplastic WT (G2b). There were two metachronous WT (4%), 3 years after a TN (G1) and 12 years after a NSS (G2b). NSS is recommended in bilateral WT and may be an option in selected UWT patients with BWS/HH because it was not associated with an increased risk of local relapse.

Surgical strategy in treating synchronous sporadic bilateral renal cell carcinoma

Objective To evaluate the impact of different surgical approach on postoperative renal function in patients with synchronous sporadic bilateral renal cell carcinoma (SSBRCC). Methods The medical records of 28 patients with SSBRCC were reviewed. Of all these cases, 21 patients were male and 7 were female, and the mean age was 51 (25-63) years. None of these cases had family history of renal carcinoma. The treatment and other clinical data were analyzed retrospectively. Results All of the 28 patients underwent bilateral surgeries. Single procedures were performed on 16 patients, of which, one underwent bilateral radical nephrectomy (RN), 8 bilateral nephron-sparing surgery (NSS) and 7 unilateral NSS combined with contralateral RN. Staged procedures were performed on 12 patients, of which bilateral NSS was on 5 patients (NSS-NSS group), NSS followed by RN (NSS-RN group) on 3 patients and RN followed by NSS (RN-NSS group) on 4 patients. In term of postoperative renal function preservation for SSBRCC patients, the standard NSS was better than RN. Between different staged procedures, NSS-NSS was the preferred choice, and the NSS-RN was superior to the RN-NSS. Conclusions In term of preservation of postoperative renal function, the staged procedures were recommended, and NSS was preferred in the first stage. In deciding the second stage surgical approaches, the surgeons should take the renal function preservation and the tumor size into consideration. Key words: Carcinoma, renal cell; Bilateral surgery; Strategy; Renal function