Management Of Bilateral Wilms Tumor Research Articles

Bench surgery with autotransplantation for bilateral Wilms tumor-A feasible technique for renal sinus invasion.

Bilateral Wilms tumor (BWT) with renal sinus invasion requires extremely difficult surgical care. This study presents an alternative strategy for tumor removal while at the same time preserving the renal parenchyma. In total, 9 cases of synchronous BWT were admitted to our hospital between May 2016 to Aug 2020. We retrospectively reviewed the clinical data, surgical technique, and functional and oncological outcomes of these cases. The 9 cases included 3 males and 6 females, with a median age of 12months at surgery (range 7-40). A total of 14 kidney units had renal sinus invasion (77.8%), whereas multifocal neoplasms were observed in 7 units (38.9%). The local stage distribution revealed 1 kidney with stage I, 10 kidneys with stage II, and 7 kidneys with stage III. Nephron-sparing surgery was performed on 15 kidney units (83.3%), among which 13 (72.2%) underwent bench surgery with autotransplantation (BS-AT), whereas 2 (11.1%) were subjected to tumor enucleation in vivo. Urinary leakage was the most prevalent postoperative complication. We observed negative margins. During the mean follow-up of 28.4 months, 2 patients (22.2%) succumbed from sepsis and renal failure, respectively, whereas the other 7 (77.8%) survived without recurrence. Survivors experienced an estimated glomerular filtration rate of 81 ± 15.4 ml/(min × 1.73 m2). The endpoint renal volume of 9 renal units receiving BS-AT significantly increased (P = 0.02). In summary, the surgical management of bilateral Wilms tumor requires meticulous operative approach and technique. Besides, BS-AT provides a viable alternative to nephron-sparing surgery for BWT patients with renal sinus invasion.

Management of intravascular thrombus in cases of bilateral Wilms tumor or horseshoe kidney

PurposeTo describe the oncologic and surgical management of bilateral Wilms tumor or Wilms tumor arising in a horseshoe kidney with intravenous tumor thrombus to help pediatric surgeons negotiate this rare and difficult anatomic circumstance. MethodsA single-institution, retrospective medical record review identified 4 cases of bilateral WT and one case of WT arising in a horseshoe kidney with intravenous tumor thrombus between 2009 and 2021. The presentation, imaging, chemotherapy regimen, intraoperative approach, and surgical and oncologic outcomes were reviewed for each of these patients. ResultsAll patients received a total of 12 weeks of neoadjuvant chemotherapy. In two patients, a staged approach to the bilateral tumors was undertaken with the first side being operated on after six weeks of therapy and the other side undergoing surgery after an additional six weeks of therapy. Of five patients, four underwent nephron-sparing surgery of all tumors and one underwent unilateral radical nephroureterectomy with contralateral nephron-sparing surgery. Tumor thrombectomy was performed in four of five cases; one patient demonstrated a complete response of the intravenous tumor thrombus to neoadjuvant chemotherapy and did not require thrombectomy. Three patients received adjuvant flank radiotherapy. Three patients developed medically managed stage II or III chronic kidney disease and no patient required renal replacement therapy or kidney transplant to date. ConclusionNephron-sparing surgery is feasible and safe to perform in selected cases of bilateral Wilms tumor with intravascular thrombus by utilizing three-drug neoadjuvant chemotherapy, staged approaches to each kidney when appropriate, and detailed preoperative and/or intraoperative mapping of renal venous anatomy. Successful nephron-sparing surgery with tumor thrombectomy is dependent on a branched renal venous system or the presence of accessory renal veins. Level of evidenceLevel 4.

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.

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.

Management of bilateral Wilms tumor over three decades: The perspective of a single center

Outcomes of Wilms tumor improved in last 50 years and excellent survival rates can be achieved especially in case of non-metastatic disease and favorable histology. Nevertheless, bilateral cases still stand as a therapeutic challenge. Prognosis of bilateral Wilms tumor (BWT) is not as good as the unilateral tumors of similar stage and histology in terms of survival and renal function. Management of BWT is constantly evolving and still stands as a therapeutic challenge. This study is designed to review and share our experiences on this topic from a surgical standpoint. The records of patients treated in our clinic between 1980 and 2013 according to Turkish Pediatric Group of Oncology protocol were analyzed retrospectively and clinical data, surgical details, pathology results, long term outcomes were analyzed. Thirteen girls and 7 boys with a mean age of 2,5 years were treated. There were 2 patients with Wilms tumor-Aniridia-Growth Retardation complex and one with isolated hemihypertrophy. Metastasis were detected in lungs of 4 patients; liver of 2 and in the cranium of one. All patients except one with the presumptive diagnosis of unilateral Wilms tumor were given preoperative chemotherapy. To sum up; 19 nephroureterectomies, 8 partial nephrectomies and 13 enucleations were performed to 36 kidneys without any major early or late postoperative complications. Pathologic results revealed positive surgical margins in 2 lesions with enucleation and in 2 with partial nephrectomies and anaplasia in 4 patients. Two patients were not operated due to parental disapproval. Two patients had the need of dialysis; one was anephric and the others' renal functions recovered over a year. Seven patients received radiotherapy for pulmonary metastasis, positive surgical margins or local recurrences. Overall, 13 patients survived and 7 died due to metastasis, recurrences, and complication of dialysis and refusal of surgical treatment. Survival among all patients was 65% and 72.2% among operated ones. Of the 7 patients with the partial nephrectomy, 2 died and 5 survived. Among enucleation group, 8 out of 10 survived and 2 died. Survival was slightly higher among enucleation group (80% vs 71.4%). Median time of follow-up for survivors of disease is 5.8 years (min: 6 months and max: 14 years). Outcomes of BWT management have changed dramatically during the last few decades from only survival, to a long life expectancy without the need of renal replacement therapy owing to improvements in treatment options. We argue that positive surgical margins do not necessarily lead to local recurrence. For this reason it may be wiser to favor on more nephron sparing surgery than to achieve negative surgical margins. Adjuvant chemotherapy and radiotherapy may be adequate to prevent local recurrence. Also, survival did not differ significantly between different ways of nephron sparing surgeries, so it may be wiser to choose enucleation over partial nephrectomy which preserves more nephrons. Nephron-sparing surgery should have utmost importance despite the risk of positive margins. On the other hand, there is not enough data to interpret if positive surgical margins have role on distant metastases or not. Presence of metastasis and recurrence seems to be an important determinant of prognosis given the fact that none of the survivors had any metastasis or recurrence. Nephron preservation should be the aim while taking positive surgical margin risk on nephron sparing surgery side relying on postoperative chemotherapy and carefully planned radiotherapy to avoid recurrence. However, there is significant diversity on the management BWT in different centers and a certain validated guideline or protocol to provide the optimal treatment is still lacking.

The management of bilateral Wilms tumor.

Wilms tumor (WT) is the most common malignant renal tumor in childhood. Approximately 5-7% of WT patients present with bilateral disease, either synchronously or metachronously. Bilateral WT usually occurs in younger children and more often in girls. Management of a child with bilateral WT is very challenging. In contrast to unilateral WT, there has not been uniform agreement about the therapeutic strategy in the management of bilateral WT. As surgery is a critical component in the treatment of WT, the aim is to achieve a high cure rate while maintaining adequate long-term renal function in patients with bilateral WT. In the past, radical surgical procedures which lead to the patients on dialysis have been traditionally recommended in these patients. After several multicentre trials, bilateral biopsies followed by pre-operative chemotherapy and then renal salvage surgery have been recommended. The management of bilateral WT has evolved from primary surgical extirpation to kidney-preserving resection after preoperative chemotherapy. Preoperative chemotherapy often results in significant reduction in tumor size, thereby facilitating subsequent renal salvage. The analysis of children with bilateral WT shows that preservation of renal parenchyma is possible following initial preoperative chemotherapy. Only centers with experience in bilateral WT should treat the cases with bilateral WT to provide optimal treatment.

Synchronous bilateral Wilms tumor

The optimal management of bilateral Wilms tumor (BWT) is challenging, and their survival is lower than for unilateral tumors. This report discusses a large series of BWTs treated in Italy in the last 2 decades. This analysis concerns patients with synchronous BWT registered at Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) centers between 1990 and 2011; details on their treatment and outcome are presented and discussed. Ninety BWTs were registered in the AIEOP Wilms tumor database. Preoperative chemotherapy was given for a median 12 weeks before definitive tumor resection was attempted. Forty-eight percent of the patients had preservation of bilateral renal parenchyma. The proportion of bilateral nephron-sparing surgeries was not higher in the 37 patients initially given doxorubicin/vincristine/actinomycin D (32%) than in the 43 children receiving vincristine/actinomycin D alone (58%). The 4-year disease-free survival rate was 66.5% ± 5% and overall survival was 80% ± 5% for the cohort as a whole. The 4-year disease-free survival (overall survival) for 18 children with diffuse anaplasia or postchemotherapy blastemal-type tumors was 51% ± 13% (62% ± 13%), as opposed to 72% ± 3% (88% ± 4%) for 68 children with a favorable histology (log-rank P = .04 [P = .007]). These results provide further evidence that the optimal duration and choice of drugs for preoperative chemotherapy remain an open question. Outcome remained significantly worse for BWT than for unilateral Wilms tumor. To enable the conservative treatment of as many affected kidneys as possible, only centers with experience in BWT should manage such cases.

Renal Function Outcomes in Patients Treated With Nephron Sparing Surgery for Bilateral Wilms Tumor

Management of bilateral Wilms tumor represents a particular challenge in the consideration of long-term renal function for affected patients. Aggressive surgical resection to prevent recurrence must be balanced with the desire to preserve renal function. We evaluated our institutional experience with nephrological outcomes in patients treated with nephron sparing surgery for bilateral Wilms tumor. We identified all patients with synchronous bilateral Wilms tumors presenting to St. Jude Children's Research Hospital between October 1987 and February 2004. We also included patients with Wilms tumor involving a solitary kidney presenting during the same period. A total of 17 patients were identified who underwent nephron sparing surgery, including 16 with bilateral tumors and 1 with tumor in a solitary kidney. Institutional review board approval was obtained to retrospectively review records and analyze outcomes based on long-term renal function, hypertension, proteinuria, need for dialysis and indications for renal transplantation. Eight of the 17 patients initially underwent bilateral nephron sparing surgery and 9 initially underwent a combination of nephrectomy and contralateral nephron sparing surgery. Two patients were eventually rendered anephric following further resections secondary to local recurrence. Before the initiation of therapy all patients had normal baseline creatinine clearance, which was calculated using the Schwartz formula. At a median followup from diagnosis of 72 months (range 15 to 207) 1 patient had renal insufficiency and another 3 had renal failure requiring dialysis. One of the 3 patients on dialysis died of metastatic Wilms and 2 await renal transplantation. None of the remaining patients had evidence of proteinuria. Ten of the 17 patients (58.8%) had hypertension at diagnosis and 9 (52.9%) required antihypertensive medications at the most recent followup. The overall survival rate in this group of patients was 88.2% with no evidence of disease in survivors at the most recent followup. When combined with adjuvant radiation and/or chemotherapy, nephron sparing surgery provides an opportunity to preserve renal function, while maintaining excellent long-term oncological outcomes for patients with bilateral Wilms tumor.

THE ROLE OF RENAL SALVAGE PROCEDURES FOR BILATERAL WILMS TUMOR: A 15-YEAR REVIEW

We reviewed our experience with renal salvage procedures in patients with bilateral Wilms tumor to determine the clinical outcome. From 1982 to 1997, 23 children with bilateral Wilms tumor were treated with partial nephrectomy at our institution, including 7 who were also treated with brachytherapy. Medical history, use and response to chemotherapy and brachytherapy, operative records, renal function, pathological results, survival, and techniques for partial and repeat nephrectomy and brachytherapy were reviewed. We treated 8 boys and 15 girls, of whom 21 who presented with synchronous bilateral Wilms tumor underwent primary chemotherapy followed by secondary partial nephrectomy. A total of 44 partial nephrectomies were performed and brachytherapy was done in 7 patients. Ten children have normal renal function and no disease, 10 are dead and 2 have metastatic disease. Anaplasia was the most significant factor associated with an unfavorable outcome (p = 0.003). Of the patients who were cured 60% had a positive response to initial chemotherapy compared with only 25% who had an unfavorable outcome (p = 0.09). No significant differences were noted with respect to gender, age at presentation, highest local tumor stage at presentation or initial nephrectomy. No patient treated with brachytherapy had local recurrence. Preoperative chemotherapy followed by nephron sparing surgery is indicated in patients with bilateral Wilms tumor, while in those with diffuse anaplasia nephron sparing surgery is contraindicated. Brachytherapy should be considered for treating local disease involving chemoresistant tumors.

A technique for the use of afterloading 137Cs brachytherapy in renal-sparing irradiation of bilateral wilms tumor

To describe a renal sparing brachytherapy technique for treating patients with bilateral Wilms tumor who have limited residual tumor measuring 2 cm or less after initial chemotherapy. A technique for using brachytherapy in the radiotherapeutic management of bilateral Wilms tumor is described. Three patients with bilateral Wilms tumor were treated at our institutions. All three patients had initial nephrectomy of the contralateral kidney followed by chemotherapy. Local excision of residual tumor in the remaining kidney was done in all three cases. A 137Cs isotopic source was placed in the tumor bed at the time of the second surgery using a simple afterloading applicator. The techniques of applicator placement, localization, and brachytherapy dosimetry are described. The minimum tumor dose varied from 16 to 25 Gy. All three patients are alive and well at 28, 48, and 66 months after the procedure. There were no serious operative or postoperative sequelae. This simple brachytherapy technique was effective in selected cases of bilateral Wilms tumor where a renal-sparing radiotherapy approach was needed. This technique is most applicable when there is residual intrarenal tumor after partial nephrectomy, when the tumor is unifocal, and when the tumor bed is less than 2 cm diameter.

The Current Management of Bilateral Wilms Tumor

The good prognosis of synchronous bilateral Wilms tumor seems inappropriate for the magnitude of the disease process. Our experience with 11 cases demonstrates the unusual tumor response to chemotherapy and limited preservative surgery. Although 2 patients died 2 with metastatic disease have responded to chemotherapy and are alive at 5 and 8 years after treatment. In addition, 1 patient has survived with biopsy only and no definitive surgery. Although all surgical options have been used, ranging from biopsy alone to bench surgery with autotransplantation to bilateral nephrectomy, our experience demonstrates the effectiveness of a conservative approach with initial biopsy, chemotherapy and subsequent partial nephrectomy if needed. Our survival data and the histological examination of the tumors after chemotherapy suggest a strong relationship of bilateral Wilms tumor to the nodular renal blastema-nephroblastomatosis complex, and a mechanism to explain the excellent tumor response to chemotherapy.

Bilateral Wilms Tumors

The current management of bilateral Wilms tumor is reviewed and the high incidence of concurrent anomalies with bilateral Wilms tumor is demonstrated in 4 of 5 children. Standardization of management has not been established. Of the 5 patients 3 survived 30 months to 11 years without recurrent tumor. Two had unilateral nephrectomy and partial nephrectomy, while 1 had bilateral nephrectomy and allotransplant. All survivors have had adjunctive chemotherapy and radiotherapy.