Conservative surgery of the kidney in children

Summary Increasing recognition of bilateral renal involvement in Wilms' tumor (nephroblastoma) has led to a less radical approach to surgical treatment. Experience with bilateral nephrectomy and subsequent dialysis or transplant has been unsatisfactory, and unilateral nephrectomy with contralateral radiotherapy carries the morbidity of radiation in addition to the risk of recurrent disease. Advances in chemotherapy have facilitated preoperative shrinkage of tumors and subsequent local excision of remaining tumor nodules with preservation of functioning renal tissue. We have treated 10 children with bilateral (stage V) Wilms' tumor in this manner. Two-year disease-free survival in this group is 60%, and renal function is satisfactory in all survivors. This experience suggests that nephron-sparing surgery may be possible in the majority of children with Wilms' tumor. Conservative surgery is essential in certain special situations such as solitary kidney or the horse-shoe abnormality.     

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.     

CONSERVATIVE SURGICAL MANAGEMENT OF BILATERAL WILMS TUMOR: RESULTS OF THE UNITED KINGDOM CHILDREN''S CANCER STUDY GROUP

Purpose

Bilateral Wilms tumor presents the clinician with a treatment dilemma. Since 1980 most centers of the United Kingdom Children's Cancer Study Group have used a conservative surgical approach with initial biopsy followed by chemotherapy and delayed surgical resection. We assess the outcome of this treatment approach in terms of survival, and preservation of renal mass and function.

Materials and Methods

We retrospectively analyzed the records of 71 children with bilateral Wilms tumor diagnosed between 1980 to 1995 at 17 United Kingdom Children's Cancer Study Group centers. In 57 patients conservative surgical treatment with initial biopsy was followed by chemotherapy and delayed tumor resection, while 13 underwent initial surgical resection followed by chemotherapy. One patient was excluded from study because the lesion in 1 kidney proved to be a benign cyst. Mean followup was 6 years (range 1 to 15). The percentage of renal tissue involved with tumor and preserved was estimated, and renal function at the last followup was recorded.

Results

Overall survival was 69% with similar survival in the conservatively treated and initial surgical resection groups. At the last followup renal function was normal in 80% of the patients in each group. Mean preserved renal mass was 45 and 35% in the conservatively treated and initial resection groups, respectively, with a trend toward better preservation in those treated conservatively. Bilateral Wilms tumor with an unfavorable histology was associated with a poor prognosis.

Conclusions

Conservative surgical treatment of favorable histology bilateral Wilms tumor may improve the preservation of renal mass and function without impairing patient survival.     

A case of adult Wilms' tumor]

Wilms' tumor is very rarely found in adults and there are no established treatment guidelines for such tumors in adults. A 56-year-old woman was referred to our hospital for further examination of macroscopic hematuria. Computed tomography scan revealed a large right renal mass with enlarged lymph nodes. Angiography showed a hypovascular tumor. She underwent right nephrectomy and resection of lymph node metastasis with a diagnosis of malignant renal tumor. Histopathological examination revealed nephroblastoma with lymph node metastasis. The disease was classified as stage III according to the National Wilms' Tumor Study classification. The patient received adjuvant chemotherapy consisting of ifosfamide, cisplatin, and etoposide. This protocol was selected because of the published poor results with the standard Wilms' tumor chemotherapeutic agents when used in adults. She remained without tumor recurrence as of six months after surgery. Development of better therapeutic approaches to adult Wilms' tumor is awaited.     

Wilms' tumor metastatic to bilateral testes at presentation: case and review of the literature

A 7-year-old male with bilateral palpable testicular masses was found to have metastatic stage IV Wilms' tumors associated with both left renal and lung lesions. The patient was treated successfully with testicular-sparing bilateral partial orchiectomies, radical nephrectomy, chemotherapy, and radiation, with 8 years free of recurrence. This is the only reported case of bilateral testicular tumors from metastatic Wilms' tumor that were present at the initial presentation. A review of the literature of all previous reported cases of metachronous or related testicular metastatic Wilms' tumors is reported.     

Wilms' Tumor with long-delayed recurrence: 25 years after initial treatment

Wilms' tumor is one of the most frequent malignant neoplasms in childhood. Advances in treatment modalities such as the combination of chemoradiation therapy with surgery have enhanced overall survival. However, recurrence of Wilms' tumor is still a problem. In this case, a 28-year-old female had experienced intermittent abdominal pain, and the computed tomography scan showed a huge pelvic mass. The patient had a history of radical nephrectomy for Wilms' tumor with concurrent chemotherapy at the age of three. The pelvic mass was resected in February 2010 and was confirmed to be a recurrent Wilms' tumor. The recurrent tumor showed a classic triphasic Wilms' tumor growth pattern with frequent mitoses and tumor necrosis. Our case is an extraordinary case of a long-delayed recurrent Wilms' tumor after 25 years, which is the longest disease-free interval ever reported. The possible effects of chemotherapy as well as some other mechanisms of this late relapse are discussed.     

Successful treatment of a case of advanced adult Wilms' tumor with pre-surgical chemotherapy, irradiation and radical nephrectomy

A 37-year-old male was referred with a huge renal mass and multiple lung lesions. Abdominal and chest computed tomographic scan revealed a 11×15×17. 5 cm right renal tumor and multiple lung metastases. Neither tumor was enhanced, and no lymph adenopathy was detected. Percutaneous needle core biopsy was performed and the tumors were diagnosed as Wilms' tumor histologically supported by immunohistological positive staining to WT-1 and CD56. Since lung metastases were detected and the renal tumor reached the abdominal aorta on the left side, presurgical systemic chemotherapy (ifosphamide＋ carboplatin＋etoposide ; ICEx 4 courses) was performed. The renal tumor and the lung metastases were markedly decreased in size and subjected to a radical nephrectomy. Histological examination confirmed the diagnosis of Wilms' tumor without anaplastic change categorized as mesenchymal type. As an adjuvant therapy, irradiation to renal bed (40 Gy/20 fr) and bilateral lung (12 Gy/8 fr) were performed. Six months after the end of the therapy, no evidence of recurrence was detected.     

Teratoid Wilms' tumor: case report of a rare variant that can mimic aggressive biology during chemotherapy

Teratoid Wilms' tumor is considered by some as a variant of Wilms' tumor containing at least 50% heterologous differentiated tissue. Fewer than 30 cases have been described. We report a 9-month-old boy with bilateral Wilms' tumors who did not respond to multiagent chemotherapy and underwent right nephrectomy that showed a teratoid Wilms' tumor. The patient continued to survive despite cessation of treatment. The overall predominance of differentiated stromal elements in this subtype of Wilms' tumor might explain the poor response to chemotherapy yet generally favorable outcome. Recognition of this subtype on biopsy might justify earlier surgical intervention that, for bilateral tumors, might allow for greater nephron sparing.     

Preoperative chemotherapy for children with Wilms' tumor.

Five years ago, the use of preoperative chemotherapy for Wilms' tumor was adopted at this institution. Thirty children ranging in age from 5 months to 9 years had histologically confirmed Wilms' tumor (needle biopsy, n = 26; open biopsy, n = 4). Stage was determined by chest and abdominal computed tomography (CT) scan. Bilateral tumors were present in 6 children. All children received actinomycin D and vincristine from 3 weeks to 6 months before surgery. Seven children with bilateral tumors or stage IV disease also received adriamycin. CT-measured tumor masses shrunk in most cases. Subsequently, nephrectomy was performed in 23 patients and partial nephrectomy in 4, 2 of whom had bilateral disease. In 2 patients, residual bilateral well-differentiated epithelial tumor was not surgically resected. One child died before surgery. Reevaluation at delayed total or partial nephrectomy resulted in a downstaging of disease in 12 (41%). Further chemotherapy and radiation was based on the surgical stage. Postoperative chemotherapy (4 months to 2 years) was administered to all patients. The chest and/or abdomen were radiated in 12. Four patients (13%) died, 1 of radiation pneumonitis and 3 of the disease progression (2 with unfavorable histology, 1 of whom had bilateral disease). Two of 4 with unfavorable histology and 4 of 6 with stage IV disease have survived. It is concluded that this preoperative chemotherapy protocol is as effective as the National Wilms' Tumor Study (NWTS) protocol. The treated tumor is smaller, less friable, and easier to remove. Furthermore, because of downstaging, less radiation is necessary for cure.     

The role of renal salvage procedures for bilateral Wilms tumor: a 15-year review

PURPOSE: We reviewed our experience with renal salvage procedures in patients with bilateral Wilms tumor to determine the clinical outcome. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.     

Bilateral Wilms' tumor with anaplasia: lessons from the National Wilms' Tumor Study

Purpose

The purpose of this study was to evaluate whether initial diagnostic technique influenced the ability to identify anaplastic histology, to determine the time interval to diagnosis of anaplasia, and to delineate the incidence of discordant pathology in bilateral Wilms' tumor. We hypothesized that delay in diagnosis of anaplasia could affect time to appropriate surgery and intensive multimodality therapy.

Methods

One hundred eight-nine children were enrolled in the fourth National Wilms' Tumor Study with synchronous bilateral tumors, 27 of whom were eventually shown to have anaplastic histology. Initial diagnostic technique, time interval to diagnosis of anaplasia, and the incidence of discordant pathology were determined.

Results

Anaplasia was identified in 0 of 7 tumors by core needle biopsy, 3 of 9 tumors by open wedge biopsy, and in 7 of 9 cases by partial or complete nephrectomy. The mean duration of first chemotherapy regimen (DD or EE) was 20, 39, and 36 weeks, respectively, before anaplasia was identified at second surgery. Discordant pathology between bilateral tumors was identified on final tissue diagnosis in 20 patients. Only 4 patients had anaplastic tumors in both kidneys.

Conclusions

Core needle biopsy did not identify anaplasia in 7 of 7 children. Open biopsy or partial/complete nephrectomy identified anaplasia at initial diagnostic procedure in 10 of 18 children. Twenty of 24 patients at final tissue diagnosis had discordant pathology between the 2 kidneys. Earlier interval incisional biopsy or resection may identify anaplastic histology and limit the duration of chemotherapy targeted to favorable histology for children with bilateral Wilms' tumor and anaplasia.     

Nephron-sparing approach to bilateral Wilms' tumor: in situ or ex vivo surgery and radiation therapy

Bilateral Wilms' tumor patients, who experience local recurrence after maximal multimodality therapy, present a difficult surgical problem. The role of surgery in the management of these patients has changed from ablation to preservation of renal tissue, with bilateral nephrectomy as a last resort. Two children who had recurrent tumors in the remaining kidney underwent nephron-sparing surgery and focal intraoperative radiation therapy. In one case, this nephron-sparing surgery and intraoperative radiation therapy were performed in situ, in the other kidney was removed and reimplanted (ex vivo bench surgery and irradiation). These techniques may allow complete obliteration of gross and microscopic disease, while maximizing residual renal function.     

Bilateral Wilms' tumor

Recent studies indicate a favorable outcome with bilateral Wilms' tumor. From 1971 to 1985, ten children between the ages of 6 months and 5 years were treated for this disease. During the early part of the series, five patients had nephrectomy on one side and partial nephrectomy on the other side, and one patient had bilateral partial nephrectomies at the same operation. More recently four patients initially had a biopsy of both tumors and lymph node sampling followed by chemotherapy. At the second-look procedure two patients had multiple biopsies because there was no obvious tumor. Histologically there was no tumor on the third-look procedure in these two patients. Nine patients had a favorable histology of the Wilms' tumor. One patient had a favorable histology on one side and an unfavorable type of histology on the other side. Eight patients are surviving between 6 months and 13 years. Two died of extensive disease within 16 months of diagnosis. One patient had an unfavorable histology. The good results following partial nephrectomies have led us to attempt to conserve additional tissue, as has been done in the last four patients. Our early results suggest biopsy of the tumor followed by chemotherapy, then a second look, and if necessary, third-look procedures may result in preservation of functioning renal tissue.     

Risks and benefits of percutaneous biopsy and primary chemotherapy in advanced Wilms' tumour.

Between 1982 and 1988, 36 children with advanced Wilms' tumour underwent percutaneous trucut needle biopsy followed by chemotherapy before definitive surgery. Nephrectomy was performed after a median of 14 weeks of chemotherapy. Substantial reduction in tumour bulk was achieved in 94% of patients. Biopsy morbidity was low and complete concordance between the histological assessment of the tumour in the biopsy specimen and at subsequent nephrectomy was confirmed in 26 of 28 (93%) patients. The overall clinical value of trucut biopsy was 83% (30/36 patients). Survival rates in this high-risk group were comparable to those of children with less advanced disease. Chemotherapy may be the primary treatment of choice for patients with Wilms' tumour. Percutaneous biopsy allows definition of histology in most patients without increasing morbidity.     

Treatment strategy for nodular renal blastema and nephroblastomatosis associated with Wilms' tumor.

Nodular renal blastema and nephroblastomatosis were present in 8 of 118 patients (6.8%) with Wilms' tumor. Five of these 8 patients (63%) had bilateral Wilms' tumors. Two had hemihypertrophy. Preoperative renal angiograms were accurate in detecting these metanephric anomalies. The surgical approach consisted of removal of the most diseased kidney and biopsy for diffuse tumors and wedge resections for localized tumors for the remaining kidney. Postoperatively, radiation was administered when tumor extended outside the kidney. Chemotherapy consisted of vincristine and dactinomycin for 18 mo and adriamycin for 6 mo. This method of management resulted in tumor-free survival of these 8 patients for 1--44 mo (median 24 mo). Nodular renal blastema and nephroblastomatosis may possibly develop into Wilms' tumor. All of these three conditions respond to surgery, chemotherapy, and radiation. When a Wilms' tumor is encountered, it is better to explore and possibly biopsy the opposite kidney. There is a place for second-look laparotomy in this spectrum of congenital anomalies.     

Bench surgery with autotransplantation for bilateral synchronous Wilms' tumor: a report of three cases

The surgical management of bilateral synchronous nephroblastoma remains controversial. The authors describe three cases treated using ex vivo tumor dissection followed by autotransplantation in an attempt to preserve functioning renal tissue. Two children are alive and tumor free with adequate renal function at 30 months and 3 years, respectively. One died from tumor recurrence with metastases 9 months after surgery. This technique is an acceptable alternative to bilateral nephrectomy followed by transplantation.     

Wilms' tumor--single-center experience with renal surgery

OBJECTIVE: To analyze the perioperative complications of renal surgery in a sample of patients with Wilms' tumor (WT), especially with regard to the effects of preoperative chemotherapy. MATERIAL AND METHODS: The case histories of 34 patients (mean age 4 years) who underwent renal surgery for suspicion of WT between 1989 and 2002 were retrospectively analyzed with special regard to intra- or postoperative complications. In total, 32 patients had undergone a radical nephrectomy and two had undergone organ-sparing renal surgery because of bilateral involvement. The median maximal tumor diameter was 9.6 cm. In 10 patients preoperative chemotherapy was completely renounced or had to be stopped early. All other patients were treated according to the protocols of the Société Internationale d'Oncologie Pédiatrique (SIOP)-9 or 93/01 studies. RESULTS: A total of 5/34 patients (14.7%) had perioperative complications. There was one intraoperative tumor rupture in a patient who had undergone an emergency radical nephrectomy before completing preoperative chemotherapy. Furthermore, three patients had to be reoperated on because of small bowel obstruction during the first 12 months after renal surgery. Another patient developed pancreatitis postoperatively due to delayed drainage of pancreatic secretion. These four patients had completed preoperative chemotherapy. All postoperative complications occurred in patients with tumors > 10 cm in diameter or after extended surgery for vascular or extrarenal tumor involvement. CONCLUSIONS: The presented incidence of surgical complications associated with the operative treatment of WT is most probably due to the local extent of the primary tumor leading to more extensive surgical intervention. It remains unclear whether the extent of preoperative chemotherapy influences the complication rate.     

Laparoscopic nephroureterectomy for Wilms' tumor: oncologic considerations

Wilms' tumor is the most common malignant renal tumor of childhood. Surgical resection is an important aspect of therapy and is traditionally performed through an open transabdominal approach. The advent of advanced laparoscopic techniques for benign renal lesions has led to interest in applying a minimally invasive approach to Wilms' tumors in children. We describe a 2-year-old girl who presented with a right renal mass measuring 18 × 13 cm with peritoneal seeding and pulmonary metastatic disease. Wilms' tumor was confirmed on open biopsy. After neoadjuvant chemotherapy, a laparoscopic resection of the tumor with right radical nephroureterectomy, retroperitoneal lymph node dissection, and resection of peritoneal metastases was achieved using one 12-mm and four 5-mm ports. The specimens were removed in an endoscopic retrieval bag through a small Pfannenstiel incision by extending the 12-mm port site. The patient had an uncomplicated recovery and was discharged on the fifth post-operative day. She remains disease free at 19 months postoperatively. Minimally invasive techniques may be considered for resection of Wilms' tumor provided oncologic principles are carefully followed.     

Bilateral Wilms' tumors: changing concepts in management

Bilaterality is uncommon in Wilms' tumor, being present in 4% to 8% of the cases. We report the combined experience of two children's hospitals in one city over a 20-year period. We encountered nine cases of synchronous bilateral nephroblastoma (National Wilms' Tumor Study 3, stage V). Age at diagnosis ranged from 9 to 41 months (mean 23 months). There were five girls and four boys. Associated findings include nephroblastomatosis in three cases (33%), one of which also had a familial history; undescended testis in two cases; and minor anomalies in two other cases. Surgical treatment consisted of unilateral nephrectomy with contralateral partial nephrectomy or tumorectomy in six cases, nephrectomy with contralateral biopsy only in two cases, and the other patient had bilateral biopsies initially, followed at a later date by partial nephrectomy on one side. All patients received chemotherapy; actinomycin D (AMD) only was used in the oldest case, vincristine and AMD in five cases, to which was added cyclophosphamide in one case and adriamycin in two. Seven patients received radiation therapy. Seven out of the nine patients survived more than 2 years (77%); five are well, off chemotherapy, with no evidence of disease from 4 to 11 years after diagnosis. Two patients suffered from chronic renal failure and one died from complications after renal transplantation more than 19 years after diagnosis. The two patients who died from their disease presented with more advanced tumor. Therefore, the agressiveness of multimodal therapy can be tailored according to stage and histology, and effective chemotherapy allows maximal preservation of renal parenchyma in patients with stage I and II tumors.     

Risikofaktoren intra- und postoperativer Komplikationen in der Chirurgie des Wilms-Tumors

BACKGROUND: Wilms' tumor is the most common renal tumor in childhood. Preoperative treatment is still under discussion. The aim of this study was to determine, using our own patient collective, the risk factors for and type of intraoperative complications which can occur. In addition, the influence of the surgical procedure and tumor size on the complications and survival rate was analyzed. METHODS AND MATERIALS: A total of 66 patients with Wilms' tumor were retrospectively analyzed. Evaluation included histology, size of the primary tumor as well as neoadjuvant and adjuvant chemotherapy. The total survival rate over periods of 5 and 10 years postoperatively were analysed using Kaplan-Meier survival probabilities. RESULTS: All patients underwent radical nephrectomy: 63 using the transperitoneal and three the lumbar approach. The tumors had a mean size of 9.8 cm (range 2.5-20.0). Twenty patients (30.3%) received neoadjuvant chemotherapy for tumor reduction, while 46 patients underwent surgery without preoperative chemotherapy. Complications occurred in eight patients (15.2%). In two, a the tumor ruptured under surgery, four patients developed an ileus and two suffered cardiac arrest. One patient had postoperative hypertonia and another an incisional hernia. All complications occurred with a tumor size >5 cm or in the patient group without neoadjuvant chemotherapy. The 10 year survival rate was 89.4%. CONCLUSIONS: The risk of complications is associated with the local size of the primary tumor. Through tumor reduction, neoadjuvant chemotherapy influences the expression of the such complications. Transperitoneal tumor nephrectomy is the method of choice in surgery for Wilms' tumors.