Nephron sparing surgery for unilateral nonsyndromic wilms tumor. The average newly found wilms tumor is many times larger than the kidney in which it started. Patients with hemihypertrophy in addition to their wilms. The treatment of bilateral wilms tumor requires that the tumor be rendered non viable while allowing the adjacent kidney parenchyma to continue normal function. Four to eight percent of all wilms tumors are bilateral. Common sites of metastases are lungs, liver and regional lymph nodes. The adrenal glands were affected by parenchymal hemorrhagic necrosis and the cause of death was ascribed to renal artery hemorrhage figure 1, ac. Oct 01, 1985 bilateral partial nephrectomies is the most conservative of the surgical treatments available for bilateral wilms tumor.
Click on the link to view a sample search on this topic. All but 1 child received preoperative chemotherapy with tumor reduction. Results of the first prospective multiinstitutional. Renal salvage procedures partial nephrectomy and enucleation have been. In this study, the outcome of bwt patients from a single center who were treated by the turkish pediatric oncology group tpog. However, most are discovered before they have spread to other parts of the body. Syndromic causes of wilms tumor occur as a result of alterations to genes such as the wilms tumor 1 wt1 or wilms tumor 2 wt2 genes, and the tumor presents with a group of other signs and symptoms. Max wilms, the german surgeon 18671918 first described this kind of tumor. Synchronous bilateral wilms tumours bwt represent 47% of all wilms tumours wt. Pdf synchronous bilateral wilms tumor paolo indolfi and. Pdf nephron sparing surgery for unilateral nonsyndromic.
For example, among kidneys removed for wilms tumor, more than 40% contained nephrogenic. Mar 18, 2021 with bilateral wilms tumor 6% of cases, surgical exploration, biopsy of both sides, and accurate surgical staging including lymph node biopsy of both sides are performed. Wilms tumor have a mutation in the germline or in tumor tissue. Tumor shows chemotherapy induced changes occupying 40% of the mass. Wt or nrs can occur in both kidneys, termed bilateral disease, found in only 58% of cases. However, these elements may be clinically important but are not yet validated or regu larly used in patient management. Wilms tumor usually arises in one kidney unilateral, but sometimes it can occur in both kidneys bilateral. Due to an increased risk of renal failure in patients with bilateral wilms tumor, these patients receive neoadjuvant therapy with three drug chemotherapy of. Tumor type description wilms tumor includes pediatric patients with wilms and other renal tumors this protocol is not required for accreditation purposes for the following. Some early cancers may have signs and symptoms that can be noticed, but that is not always the case.
Wilms tumor wt is the most common primary renal tumor in children. W rllms tumor is the most frequent renal tumor in childhood. Wilms tumor is bilateral in 4%% of children 4 and may be associated with congenital anomalies such as cryptorchidism 2. Children with bilateral wilms tumor michael ritchey, md department of urology, mayo clinic college of medicine, scottsdale, arizona. The introduction of multimodal therapy has improved the survival rate of bilateral wilms tumors bwt. Historically, the management of bilateral wilms tumor bwt was nonstandardized and suffered from instances of prolonged chemotherapy and inconsistent surgical management which resulted in suboptimal renal and oncologic outcomes. Renal insufficiency is a significant complication of wilms tumor treatment in the 5% with bilateral disease.
Bilateral wilms tumor with neonatal onset and the importance. Wilms tumor in the setting of bilateral nephroblastomatosis. Feb 01, 2021 pubmed is a searchable database of medical literature and lists journal articles that discuss wilms tumor and radial bilateral aplasia. Nephrogenic rests are microscopic foci of primitive blastemal renal elements found in normal kidney tissue in an intralobar or perilobar location and found in normal kidney tissue, in about 30% to 40% of unilateral, and all bilateral wilms tumours, as well as children with. Synchronous bilateral wilms tumor indolfi 20 cancer. Background wilms tumor wt is the most common renal malignant tumor in children. The tumour is associated with undifferentiated embryonic lesions called nephrogenic rests nrs or, when diffuse, nephroblastomatosis. In particular, cases of bilateral wilms tumor, as well as cases of wilms tumor derived from certain genetic syndromes such as denysdrash syndrome, are strongly associated with nephrogenic rests. Several genes are associated with wilms tumor, including cdkn1c, dis3l2, gpc3, cdc73 and wt1.
The treatment consisted ofright nephrectomy and radiation therapy totheright kidney region andtotheleftkidney. Risk factors for metachronous bilateral wilms tumor. Nss has become the standard of care for children with bilateral wilms tumor bwt. Procedure additional excision performed after the definitive resection eg, reexcision of surgical margins cytologic specimens. During a 16year period, 49 children were treated for wilms tumor wt. Wilms tumor early detection, diagnosis, and staging. Synchronous bilateral wilms tumor accounts for 4% to 6% of all wilms tumors. Renal salvage procedures partial nephrectomy and enucleation have been recommended to conserve renal parenchyma. Synchronous bilateral wilms tumor is rare 5 10% of cases but well recognized, conferring a worse prognosis as surgical excision is much more difficult. Wilms tumor and other childhood kidney tumors treatment. Wilms tumor early detection, diagnosis, and staging 1. Recovery from bilateral wilms tumors sage journals. Pdf bilateral wilms tumor metastasis to right spermatic.
The viable tumor consists of blastemal 20%, epithelial 50% and stromal 30% elements, with no evidence of anaplasia. Wilms tumor is the fifth most common pediatric malignancy 7% of all childhood tumors. Wilms tumor, mixed type, intermediate risk tumor, stage iii due to viable and nonviable lymph node metastases comment. Wilms tumor and radial bilateral aplasia genetic and rare. 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. A wilms tumor may grow without being detected until it becomes quite large. Protocol for the examination of specimens from pediatric. During the early part of the series, five patients had nephrectomy on one side and. Bilateral wilms tumor surgical aspects home thieme connect. The management of synchronous bilateral wilms tumor. Wilms tumor and other childhood kidney tumors treatment pdq. We report on a neonate who presented with synchronous bilateral wilms tumors. As far as staging goes, it is described as stage v irrespective of the local staging of each tumor mass.
The age at primary diagnosis was 6 months to 5 years mean 2. Repeat nephronsparing surgery for children with bilateral wilms. Wilms tumour wt is the most common paediatric kidney cancer and affects approximately one in 10 000 children. About 5 to 10% of children with wilms tumor have tumors in both kidneys called bilateral tumors, which may be inherited. Bilateral wilms tumors nephroblastoma radiology case. Its peak incidence table 1 most common age at presentation for solid renal malignancies renal neoplasm age range peak age wilms tumor unilateral form 111 y 3.
The incidence is much lower 34 per 10 000 children in asian countries. Wilms tumor is extremely rare in the neonate, with less than 20 welldocumented cases. Management of bilateral wilms tumor is particularly challenging, considering the chances of recurrence and longterm renal function for. However, a wilms tumor presenting as bilateral and multifocal is very rare. No bilateral lesions in neonates have been described in the literature. We advocate for a broader application of nephronsparing surgery in wilms tumor cases with the goal of preserving renal function without compromising oncologic outcomes. Renal function and outcome following salvage surgery for. Wilms tumor is often first noticed because of abdominal swelling or a mass in the kidney that can be felt upon physical. Outcome and renal function following salvage surgery for bilateral. Pdf synchronous bilateral wilms tumor paolo indolfi. More information on wilms on the nci web site note that you can toggle back and forth between health professional and patient versions. Testicular and paratesticular metastasis due to wt have. Wilms tumor has many causes, which can broadly be categorized as syndromic and nonsyndromic.
The records of 9 children 5 boys, 4 girls diagnosed at vall dhebron hospital with bilateral wilms tumor between 19761995 were analyzed. But the actual but the actual diagnosis of wilms tumor is made when a small piece of the tumor is removed and. Methods the primary endpoint for evaluation was the first appearance of wilms tumor in the remaining kidney. Unilateral nephrectomy was performed in 2 metachronous cases.
Most wilms tumors are found before they have spread metastasized to other organs. Sometimes more than one wilms tumor is present in the affected kidney. Maximal preservation of renal function in patients with. S original contribution 03603016802016708f02mvo bilateral wilms tumor arnold w. Rickham37 reported a case ofbilateral wilms tumor inwhich a left nephrectomy was done along with a partial nephrectomy ofthe right kidney. Although nephrogenic rests are a predisposing condition for the development of wilms tumor, not all kidneys with nephrogenic rests develop wilms tumor. Wilms tumor, 2009 gejala klinis nephroblastoma wilms tumor. Bilateral wilms tumors treated according to the japan. Hristozova pediatric clinic of oncohematology, university hospital st marina, varna, bulgaria introduction although wilms tumour wt is one of the most common solid malignancies in children, bilateral disease is rare and is seen in only 4 to 7 % of all patients. The incidence of bilateral involvement it is generally estimated to be 5% to 10%. Wilms tumor and other childhood kidney tumors acco.
Achiev ing curative resection of such tumors by partial nephrectomy or tumor enucleation while. Wilms tumor is a form of kidney cancer that primarily develops in children. In children with bilateral wilms tumor, the therapy should aim at maximal preservation of renal paren chyma and function. The differential diagnosis of wilms tumor include malignant round cell tumors such as neuroblastoma, nonhodgkins lymphoma, rhabdomyosarcoma and other pe. It is the commonest childhood renal tumour and the third commonest paediatric malignancy. The result is a mass of rapidly dividing, primitive, small cells, called wilms tumor. Kaplanmeier analysis rates of overall survival for metachronous bilateral wilms tumor were 49. Wilms tumors often become quite large before they are noticed. The median age for multicentric, unilateral cases was intermediate between the bilateral and unicentric medians. Hamilton te, ritchey ml, haase gm, argani p, peterson sm, anderson jr, green dm, shamberger rc ann surg 2011 may.
The pathogenesis of wilms tumor is complex and multifactorial. It is an embryonal tumour derived from the metanephros. A report from the childrens oncology group ann surg. Nonsyndromic wilms tumor is not associated with other symptoms or pathologies. Wilms tumor wilms tumor nephroblastoma accounts for 87% of pediatric renal masses and occurs in approximately 1. Wilms can affect one kidney, called unilateral, or both kidneys, called bilateral. Synchronous bilateral wilms tumours bwt represent 47% of all wilms tumours wt and present at a younger age tha. Recent studies indicate a favorable outcome with bilateral wilms tumor. Wilms tumor nephroblastoma is an embryonal renal cancer comprised of a combination of blastemal, epithelial and stromal cells and is the most common renal malignancy in childhood. Bilateral wilms tumors treated according to the japan wilms.
Background the introduction of multimodal therapy has improved the survival rate of bilateral wilms tumors bwt. From 1971 to 1985, ten children between the ages of 6 months and 5 years were treated for this disease. Results of the first prospective multiinstitutional treatment study in children with bilateral wilms tumor aren0534. Nearly all cases of wilms tumor are diagnosed before the age of 10, with twothirds being found before age 5. The cumulative risk of contralateral disease as a function of time since initial. About 5% to 10% of children with wilms tumors have bilateral disease tumors in both kidneys. Most nephroblastomas are on one side of the body only and are found on both sides in less than 5% of cases, although people with denysdrash.
The constellation of wagr syndrome occurs in association with an interstitial deletion on chromosome 11 del11p prevalence is about 0. Renal sparing surgery for children with bilateral wilms tumor. Wilms tumor is the most common solid abdominal tumor of childhood. Diagnosis of wilms tumor can be obtained by fetal ultrasonography. It shows association with certain congenital anomalies and it has an increased occurrence of familial cases. Emphasis is now on neoadjuvant chemotherapy followed by surgery for residual masses. Wilms tumour oncogenesis is a multistep process and thought to develop via a premalignant stage. Tucker op, mcgill cw, pokorny wj, fernbach dj, harberg fj. Two loci on chromosome 11 have been implicated in the genesis of a minority of wilms tu. Protocol for the examination of resection specimens from. Bilateral wilms tumor with neonatal onset and the importance of prenatal diagnosis.
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