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At least 19 recordsLinked to original sources
PubMed1989

[Nephroblastomas (Wilms' tumors) and special variations of nephroblastomas].

The results of the National Wilms' Tumor Study (NWTS) enabled the subdivision of nephroblastomas into subtypes with "favorable and unfavorable histology". Nephroblastomas with "unfavorable histology" could be discriminated by identifying those tumors not responding to therapeutic regimes proven successful for most cases with "favorable histology". A major disadvantage of the NWTS classification has been the exclusion of cytodifferentiated nephroblastoma variants, which, in contrast to typical nephroblastomas, can be cured by complete nephrectomy with wide excision of perinephric soft tissue. In the current study all types of nephroblastoma and nephroblastoma variants were included to encompass the whole morphological spectrum which these tumors may assume. This unselected material is necessary to define the relation between morphology and prognosis and to compare the treatment results of various clinical trials. Three hundred and four cases of nephroblastoma and related neoplasms on file at the Pediatric Tumor Registry, Kiel, were investigated by conventional light microscopy, electron microscopy, immunohistochemistry and DNA-flow cytometry. Of the "typical" nephroblastomas 50% occurred in the left kidney, 45% in the right kidney, and 5% were bilateral. Five cases were located in extrarenal sites. There were 121 males and 114 females. The peak incidence was noted in the third year of life. Of 135 patients 111 are alive and well, nine are living with disease, and 10 patients have died of disease. The blastemal predominant and stromal predominant types in our study were more frequent than in the NWTS. By contrast, the mixed and epithelial predominant types were more frequent in the NWTS. Patients with nephroblastomas of mixed or blastemal predominant type were older than those with epithelial predominant or stromal predominant type. Electron microscopy showed that nephroblastoma is derived from metanephric blastema. Blastemal cells are capable of differentiating into tubular epithelial cells and stromal cells. Undifferentiated blastemal cells contain exclusively vimentin intermediate filaments, better differentiated blastemal cells vimentin and cytokeratin, and stromal cells exclusively vimentin. Preoperative radio- and/or chemotherapy led to a marked reduction of undifferentiated blastema and poorly differentiated tubules, whereas better differentiated tubules, striated muscle, hyaline cartilage, cells with anaplastic and sarcomatous elements were not affected. Thus, identification of highly malignant nephroblastomas with anaplasia and sarcomatous renal tumors was even possible after preoperative treatment. Congenital mesoblastic nephroma (CMN; n = 17) is a low-grade malignant, cytodifferentiated nephroblastoma which very rarely occurs beyond the fourth month of life and has an excellent prognosis, provided it has been completely resected.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1981

[Genetics of nephroblastoma. I. The heterogeneity of nephroblastoma].

The age of disease onset, sex, birth weight and stature were analysed in 150 children suffering from nephroblastoma. The material was compared with its own control group (92 normal children of the same age) and with the population data from literature. All nephroblastoma patients were divided into 2 groups by the age of disease onset: before 2 years old and after this. The proband birth weight and stature in the first group statistically differed from control and population exponents. Genetic heterogeneity of nephroblastoma is discussed.

Birth Weight↗
PubMed1983

[Genetics of nephroblastoma. II. The incidence of developmental defects and dysplastic and asymmetrical traits in children with nephroblastoma].

The frequency of developmental defects and displasticity characters in children with nephroblastoma was determined in two groups--with early, up to 2 years manifestation of the tumor (ENB), in 40 patients, and with advanced nephroblastoma (ANB), in 59 patients (manifestation of the disease after 2 years). The data were correlated with control groups adequate for age (130 children). The frequency of serious developmental defects was higher in ANB group (20%) than in ENB group (7%). Over a half of developmental defects were hemihypertrophy and doubling of organs. One case of a child with the combination of nephroblastoma and Smith-Lemli-Opitz syndrome was defined. In ANB group an increased frequency of asymmetry in the conjugate organs (foot, hand, middle finger, ears) was found. Its direction is correlated with tumor localization (tumor site). In ENB group no analogous effect was shown. The data obtained present phenotype characteristics of groups with early and late manifestation of the disease which are probably different in their genesis and thus, their identification is necessary for the adequate medicogenetic consultation (examination).

Abnormalities, Multiple↗
PubMed1998

Rarity of surgical complications after postchemotherapy nephrectomy for nephroblastoma. Experience of the International Society of Paediatric Oncology-Trial and Study "SIOP-9". International Society of Paediatric Oncology Nephroblastoma Trial and Study Committee.

The aim of the study was to assess rates and types of nephrectomy-related complications in children nephrectomized for nephroblastoma after preoperative chemotherapy. Records of 598 Wilms' tumour patients registered in the International Society of Paediatric Oncology Trial & Study No. 9 (SIOP-9), and pretreated correctly according to the protocol with vincristine + actinomycin D +/- epirubicine or adriamycin prior to nephrectomy, were retrospectively reviewed. Forty-nine patients (8%), who suffered from 54 complications, were identified. Most frequent events were small-bowel occlusions (3.7%) and tumour ruptures (2.8%). Other complications were registered in 2.0% of cases. The low rate of nephrectomy complications and no deaths related to registered ones, are another argument for preoperative chemotherapy in Wilms' tumour patients.

Adolescent↗
PubMed1996

[Resection of nephroblastoma: problems and complications--evaluation of the Nephroblastoma Study SIOP 9/GPOH].

The german SIOP 9/GPOH trial and study registered 486 patients with Wilms' tumor (1/89-3/94). Preoperative chemotherapy was the general approach. The indication for primary surgery was limited (age < 0.5 and > 16 years, uncertainty of diagnosis, emergency). Wilms' tumors were operated in 482 patients (4 died preoperatively) in 78 centres. Surgical and histological reports were analyzed concerning intra- and perioperative problems and complications. A total of 60% of pretreated and 39.8% of untreated tumors had local stage I. Nephroblastomas ruptured intraoperatively in 6.0% after pretreatment versus 11.5% in primary surgery. A tumor thrombus in the inferior vena cava complicated surgery in 3.1% of cases. A total of 14.3% of all histologically positive lymphnodes were correctly biopsied by the surgeon despite their normal macroscopic aspect. The overall rate of intra- and perioperative complications was low.

Adolescent↗
PubMed1995

Paediatric surgical oncology. 5--Nephroblastoma. International Society of Paediatric Oncology (SIOP) Nephroblastoma Trial & Study Committee.

The favourable results from the treatment of Wilms' tumour are an example of the success of multimodal therapy in paediatric oncology. The epidemiology, methods of diagnosis, benefits of pre-operative chemotherapy, basic principles of surgery and post-operative treatment modalities are presented. The approach to the management of Wilms' tumour considered in this paper is mainly that of the International Society of Paediatric Oncology.

Chemotherapy, Adjuvant↗
PubMed1999

Misstaging in nephroblastoma. Causes and consequences. A report of the Sixth Nephroblastoma Trial and Study of the International Society of Paediatric Oncology.

In the Wilms tumour trials and studies of the International Society of Paediatric Oncology (SIOP), the postoperative treatment is based on the extension (stage) and the histological type. Incorrect staging results in under- or overtreatment. The authors studied the causes and consequences of misstaging in SIOP 6. In this study, the final stage was defined by a central panel of pathologists after review of the surgical and histopathological forms and study of representative microscopical sections. In 46 out of 509 trial patients there was a discrepancy between the final stage and the stage determined at the participating centres: 33 patients were understaged of whom 27 survived more than 5 years (18% died) and 13 patients were overstaged of whom 11 survived more than 5 years (15.3% died). All children with tumour extension into the renal pelvis and treated as stage I instead of stage II survived without evidence of disease. Therefore, it was decided to treat these children in the next study as a stage I. In 17 cases the treatment was based on the surgical stage before the pathological stage was known or the treatment was given according to the stage determined by the local pathologist without waiting for the panel review. The numbers are too small to conclude on the consequences of overtreatment on late effects. For all clinicians the main and most important conclusion of this study is: wait for the final pathology report before initiating postoperative therapy.

Adolescent↗
PubMedDate not supplied

[Histology and prognosis of nephroblastoma--with special reference to special variants].

101 cases of Wilms' tumor (nephroblastoma) were investigated by light microscopy. In 80 cases a diagnosis of triphasic nephroblastoma was made. 21 cases were classified as special variants of Wilms' tumor. These included congenital mesoblastic nephroma (n = 5), fetal rhabdomyomatous nephroblastoma (n = 2), cystic partially differentiated nephroblastoma (n = 3), nephroblastoma with focal or diffuse anaplasia (n = 2), clear cell sarcoma or bone metastasizing renal tumor of childhood (n = 4), rhabdoid tumor (n = 2) and rhabdomyosarcomatous nephroblastoma (n = 3). Based on our own follow-up data and on information from the literature we propose to separate the group of nephroblastomas into three categories of different prognosis: 1. Nephroblastomas of low risk (congenital mesoblastic nephroma, fetal rhabdomyomatous nephroblastoma, cystic partially differentiated nephroblastoma) - in most of these cases simple nephrectomy sufficient as adequate therapy. 2. Nephroblastomas of standard risk (triphasic nephroblastomas) - therapy according to stage of disease. 3. Nephroblastomas of high risk (nephroblastomas with focal or diffuse anaplasia, clear cell sarcoma, rhabdoid tumor, rhabdomyosarcomatous nephroblastoma) - successful therapy has as yet to be developed.

Adolescent↗
PubMed2002

[Fetal rhabomyomatous nephroblastoma. Report of 2 cases and review of the literature].

OBJECTIVE: Fetal rhabdomyomatous nephroblastoma is a particular and very rare histologic variety of nephroblastoma. The aim of this work is to study the principal clinic, therapeutic and evolutive characteristics of the fetal rhabdomyomatous nephroblastoma through two personal cases and a review of the literature. PATIENTS AND METHODS: This is a retrospective study of two observations of fetal rhabdomyomatous nephroblastoma treated in the pediatric surgery departement of Monastir (Tunisia) among 47 cases of nephroblastoma. The diagnosis was confirmed in the two cases by the histologic examination. RESULTS: The two patients were a six and a sixteen months old boy and girl. They were admitted for a voluminous mass occupying the left half-abdomen. The radiologic and biologic explorations load, in the two cases, to the diagnosis of left nephroblastoma. After a first chemotherapy that did not induce a reduction of the tumoral volume, a widened left nephrectomy was performed for the two patients. The histologic examination of the two pieces of nephrectomy concluded to a fetal rhabdomyomatous nephroblastoma with existence in the second case of an extension of the lesions to the renal pelvis and ureter in the form of a pseudo-botryoïde tumor. The tumor was classified stage I in the first case and stage II N0 in the second. The treatment was completed by an adapted post operative chemotherapy according to the SIOP 9 protocol. The two patients are currently in complete remission with an overview of six years and half. CONCLUSION: The fetal rhabdomyomatous nephroblastoma is a special histologic form of nephroblastoma that is characterized by the paucity of pulmonary metastasis, the absence of response to chemotherapy and the possibility of tumoral extension in the renal pelvis and ureter. His prognosis is similar to the classical nephroblastoma.

Female↗
PubMedDate not supplied

Neuroblastoma preoperatively treated as nephroblastoma: does inadequate therapy worsen the prognosis?

BACKGROUND: The current standard in the treatment of nephroblastoma is preoperative chemotherapy based on radiological appearance. After subsequent surgical removal few tumours proved histologically to be neuroblastoma. We asked whether initial chemotherapy according to nephroblastoma trials would change the prognosis for those neuroblastoma patients. RESULTS: Out of 1603 patients registered in the German neuroblastoma trials, 29 patients (1.8 %) have preoperatively been treated according nephroblastoma protocols. Advanced stages (11 stage 3, 12 stage 4) were dominant. Diagnostic work up of those patients revealed elevation of catecholamine metabolites in only 39 % (compared to 80 % of the control patients) and mIBG uptake in only 71 % (compared to 89 % of the control patients). Elevation of NSE was observed in 92 % of patients (control group 72 %). Patients with preoperative nephroblastoma treatment were older than the patients of the control group. Risk factors like MYCN amplification or elevation of LDH were more often detected. The outcome of the patients with preoperative chemotherapy according nephroblastoma trials was worse than that of the control group, but risk group adapted survival analysis revealed no disadvantage. CONCLUSION: The prognosis of children with neuroblastoma tumours, which have been radiologically classified as nephroblastoma, is inferior compared to the prognosis of patients without preoperative nephroblastoma therapy. The difference appears to be associated rather with more unfavourable biology than with the element "preoperative chemotherapy".

Adolescent↗
PubMed1985

Differential renal tumor response to N-ethylnitrosourea and dimethylnitrosamine in the Nb rat: basis for a new rodent model of nephroblastoma.

Previous studies have indicated that the Nb rat has a higher predisposition to the spontaneous development of nephroblastoma than most other strains of laboratory rat. In order to determine whether this inherent susceptibility could be exploited as a model for Wilms' tumor, Nb rats were treated with various chemicals in regimens known to be associated with renal tumor induction in the young rat. Nb rats were either exposed in utero to various dose levels of N-ethylnitrosourea (ENU) on day 18 +/- 1 of gestation, injected s.c. with one 25 mg/kg dose of dimethylnitrosamine (DMN) as neonates or dosed twice orally with 7,12-dimethylbenz[a]anthracene (DMBA) shortly after ovariectomy. Transplancental ENU at a dose of 60 mg/kg resulted in an average of 50% frequency of unequivocal nephroblastomas in both sexes with no renal mesenchymal tumors. In contrast, DMN administered to neonates produced a similar incidence of renal mesenchymal tumors but no nephroblastomas. DMBA in the ovariectomized female was not a successful strategy for inducing primary renal tumors in the Nb rat (0 nephroblastomas, 1 renal mesenchymal tumor in 16 effective survivors), although the kidney was sometimes the site for metastatic invasion by tumors originating at other locations. The induction of nephroblastomas and renal mesenchymal tumors by ENU and DMN in parallel experiments emphasized the many differences which establish them as distinct and unrelated tumor entities. The relatively high incidence of nephroblastoma in the Nb rat using transplacentally administered ENU appears to represent a suitable basis for developing a rodent model of human nephroblastoma or Wilms' tumor.

9,10-Dimethyl-1,2-benzanthracene↗
PubMed1986

Flow cytometric analysis of nephroblastomas and related neoplasms.

A total of 59 cases of nephroblastoma and related neoplasms were studied by flow cytometry of paraffin-embedded tissue. According to clinical prognosis, cases were subdivided into three groups: Group 1 (low risk) consisted of congenital mesoblastic nephroma (n = 13) and cystic, partially differentiated nephroblastoma (n = 2). Group 2 (intermediate risk) comprised the various subtypes of "typical" nephroblastoma (n = 24) including cases of fetal rhabdomyomatous nephroblastoma (n = 4). In group 3 (high risk) there were cases of anaplastic nephroblastoma (n = 3), clear cell sarcoma of the kidney or "bone metastasizing renal tumor of childhood" (n = 7), and malignant rhabdoid tumor of the kidney (n = 6). The three clinically different groups of tumors also varied in the proportion of cases with aneuploid tumor DNA stemlines, in S-phase fractions, and in proliferation indices (PI = S + G2 + M). Group 1 was generally characterized by a small number of cases with aneuploid tumor DNA stemlines and low values for S-phase fractions and PI, whereas Group 3 showed the largest number of cases with aneuploid tumor DNA stemlines and high values for S-phase fractions and PI. Group 2 was in between. It is concluded that flow cytometry on paraffin-embedded tissue from pediatric tumors may be a useful adjunct in determining prognosis, and that the subdivision of nephroblastomas and related neoplasms into three prognostically different groups is warranted.

Aneuploidy↗
PubMed2003

Clonality and loss of heterozygosity of WT genes are early events in the pathogenesis of nephroblastomas.

Nephrogenic rests (NRs), putative precursor lesions of nephroblastomas (Wilms' tumors), are found in 25% to 40% of kidneys presenting with nephroblastomas. Nephroblastomas are clonal tumors that, according to a genetic multistep model, are thought to arise as subclonal proliferations from NRs by accumulating genetic alterations. Different candidate genes for the pathogenesis of nephroblastomas have been identified, including those at chromosomes 11p13 (WT1 gene), 11p15 (WT2 gene), and 16q (WT3 gene). We investigated clonality and loss of heterozygosity (LOH) at these loci in different subtypes of NR. After microdissection under microscopic control, we analyzed a highly polymorphic locus of the human androgen receptor gene (HUMARA) for nonrandom X-inactivation of genomic DNA using a methylation-sensitive restriction enzyme to investigate clonality. Out of 14 patients, we found that 1 case each of adenomatous and hyperplastic NR and 2 of 7 cases of sclerosing NR were monoclonal. Five patients were noninformative. We assessed LOH at chromosomes 11p13, 11p15, and 16q by analyzing polymorphic gene loci at these regions. One hyperplastic NR and the corresponding tumor showed LOH at 11p13 and 11p15; 1 sclerosing NR and the corresponding tumor exhibited LOH at chromosome 16q. We demonstrate for the first time that sclerosing NRs can exhibit genetic alterations found in nephroblastomas, namely monoclonality and LOH at the WT gene loci. The histological morphology is no different between NRs with these genetic alterations and NRs without them. We conclude that these genetic changes are early events in the multistep genetic pathogenesis of nephroblastomas; however, they do not seem to fully determine a malignant potential of NR.

Child, Preschool↗
PubMed1982

Histological and ultrastructural studies of nephroblastoma in rats induced transplacentally by ethylnitrosourea.

Histologic and ultrastructural features of nephroblastomas in Sprague-Dawley rats induced by a single transplacental injection of ethylnitrosourea (ENU) on the 15-16th day of gestation are described and their significance is discussed with respect to the histogenesis of the tumors. Many renal tumors were observed, in addition to predominant occurrence of tumors of the nervous system. Forty-two of 142 offsprings survived over 100 days developed renal tumors, of which 37 rats had nephroblastomas, three rats had cortical adenomas, and one rat had a cystadenoma. The nephroblastomas were found in 35 rats unilaterally and in two rats bilaterally. Most of these tumors had various amounts of hyalinized stroma. In the kidneys with nephroblastomas, isolated, small foci of renal blastema were also occasionally noted. Electron microscopy revealed that nephroblastomas consisted of two types of cells, namely, epithelial and mesenchymal cells. The epithelial cells showed cellular differentiation of varying degree; they are freely lying in the stroma, forming well differentiated tubules or immature tubule-like structures and solid cell nests. Most of the undifferentiated cells observed around the tubular structures and cell nests are demonstrated to have epithelial features. These observations seem to support the concept that nephroblastoma is originated from metanephric blastema.

Animals↗
PubMed1996

Demonstration of renin gene expression in nephroblastoma by in situ hybridization.

Most patients with nephroblastoma have high levels of plasma renin and some are hypertensive. Blood pressure falls after removal of the affected kidney, suggesting that nephroblastoma is associated with renin production either by the tumour or by the kidney. In this study, direct evidence was sought of renin gene expression in nephroblastoma using in situ hybridization. Digoxigenin-labelled riboprobes and an immunoperoxidase technique were used to detect cells containing renin mRNA: this showed renin gene expression in 9 out of 12 cases. There were positive cells within metanephric blastema and in occasional neoplastic glomeruloid structures, confirming that in seven cases nephroblastoma tumour cells expressed the renin gene. However, renin gene expression was also demonstrated in perivascular cells of uncertain lineage in seven cases; in five cases there was evidence of renin gene expression in both tumour cells and perivascular cells. The latter finding raises the possibility that some of the cells expressing the renin gene could be stromal cells. It is concluded that nephroblastomas contain cells that express the renin gene and that some are tumour cells, while other perivascular cells may be stromal cells.

Child↗
PubMed1990

Renin gene expression in nephroblastoma.

Most cases of nephroblastoma have high plasma levels of prorenin which is biologically inactive. Plasma prorenin levels fall to normal following nephrectomy. In order to ascertain whether renin synthesis occurs in nephroblastomas we decided to search for renin-specific mRNA using a cDNA probe and Northern blot analyses on total RNA purified from snap-frozen human tumour tissue obtained at nephrectomy. We demonstrated renin-specific mRNA in 5/11 (45 per cent) nephroblastomas. It was 1.6 Kb in length, similar to the mRNA detected in normal kidney tissue and in kidneys with renal artery stenosis. In one of the cases of nephroblastoma, in which we could detect no normal renin mRNA at 1.6 Kb, the cDNA probe hybridized with a higher molecular weight mRNA 3 Kb in length. We conclude that some nephroblastomas synthesize renin.

Gene Expression↗
PubMed1998

Adhesive activity of gicerin, a cell-adhesion molecule, in kidneys and nephroblastomas of chickens.

Gicerin, a cell-adhesion molecule belonging to the immunoglobulin superfamily, has both homophilic and heterophilic binding activities to neurite outgrowth factor, an extracellular matrix molecule in the laminin family. Gicerin is thought to play a role in the normal development of chicken kidney, because it is expressed abundantly in the embryonic organ and only slightly in the mature organ. In this study, we have examined the adhesive activity of gicerin in the kidney to characterize its function in organogenesis. We have also examined the function of gicerin in chicken nephroblastomas ("embryonic nephromas"), which show various structures resembling those in embryonic kidneys. Immunohistochemically, the expression patterns of gicerin and neurite outgrowth factor in nephroblastomas are similar to those of embryonic kidneys. Cell-aggregation assays have shown that primary culture cells from both embryonic kidneys and nephroblastomas have strong aggregation activities, and that each aggregation is partially inhibited by gicerin antibody. In contrast, cells from adult kidney exhibit weak aggregation activity that is not inhibited by the antibody. In addition, ligand blot analysis has revealed that gicerins in embryonic kidney and nephroblastoma bind to purified neurite outgrowth factor, whereas extracts from adult kidney show no positive reaction. These findings suggest that the homophilic and heterophilic adhesive activities of gicerin are involved in the formation of both normal kidney and nephroblastoma.

Animals↗
PubMed1992

Incidence, histopathologic and electron microscopic features of spontaneous nephroblastomas in rats.

Primary renal neoplasms in the rats are uncommon. Nephroblastoma is the only renal embryonal tumor of the rat; all other tumors are reported in older rats. The occurrence of spontaneous nephroblastoma in rats has been reported. However, metastasis from the nephroblastoma in rat is extremely rare. Data from 2669 Sprague-Dawley control rats and 1060 Fischer-344 rats were reviewed and evaluated to determine the incidence and pathology of nephroblastoma. This tumor was observed in three Sprague-Dawley rats. Metastasis was observed in the lungs and renal lymph nodes in two different rats. No case of nephroblastoma was observed in Fischer-344 rats. Detailed histopathological and electron microscopic features of these neoplasms are described and discussed.

Animals↗