Bilaterally multicentric synchronous Wilms' tumor: Successful conservative treatment despite persistence of nephrogenic rests

We report a case of synchronous bilateral Wilms' tumor in a girl with incomplete Beckwith-Wiedemann syndrome and hemihypertrophy. Multiple small tumors were present in both kidneys. The initial diagnostic biopsy showed stage I monophasic blastematous Wilms' tumor of favorable histology, with multiple perilobar nephrogenic rests (nephroblastomatosis). By flow cytometry, tumor was diploid, with an S-phase fraction of 13.9%. Dactinomycin and vincristine were begun as per the National Wilms' Tumor Study IV (18 week course). After 1 month, only a single small lesion was evident, which persisted unchanged. Excisional biopsy 5 months after beginning chemotherapy showed entirely necrotic tumor but apparently unaltered nephrogenic rests. After completing chemotherapy, the child has done well, with normal renal function and no evidence of disease 3 years after diagnosis. Management of stage V Wilms' tumor is tailored to the individual case, being as conservative as possible to spare renal parenchyma. Given the high incidence of coexisting nephrogenic rests in bilateral Wilms' tumor, careful follow-up is required, as these potentially pre-malignant rests may resist chemotherapy. Med. Pediatr. Oncol. 28:420–423, 1997. © 1997 Wiley-Liss, Inc.     

Inflammatory pseudotumor of the liver masquerading as a metastasis in a child treated for nephroblastoma

A 31/2-year-old boy presented with a palpable hepatic tumor thought on clinical and radiological grounds to be a metastasis but which was found to be an inflammatory pseudotumor on histological examination. Eighteen months previously he had received chemotherapy and radiotherapy for a stage IV Wilms' tumor, which had been surgically excised 4 months after commencing treatment. This case illustrates the importance of obtaining a histological diagnosis in the management of patients with malignant tumors.     

Wilms' Tumor with Intracardlac Extension: Chemotherapy Before Surgery

The article describes two Chinese boys ages 2 and 3 years with unilateral Wilms' tumors complicated by intracaval and intracardiac extension. In contrast to the previously recommended treatment with surgery followed by chemotherapy and radiation therapy, the children were managed primarily with combination chemotherapy before definitive operation. Reduction of tumor sire on serial imaging was documented, and no viable tumor cells were found when the involved kidney and right atrium were explored. Both patients remained alive without evidence of disease more than 5 years after initial diagnosis. A literature search revealed case reports and retrospective analyses of 70 patients with Wilms's tumors and intracardiac involvement, and a tendency toward preoperative chemotherapy with or without the addition of radiation therapj was obsemed. The overall outcome of this group of patients parallels the outcome of those without intracardiac extension ty hology and stage. Wilms's tumor presenting with extension into the inferior vem cava and right atrium is thus rare and renders the affected child with additional cardiovascular complications and operative risks. As a result of the uncommon occurrence, a consensus on managemnt based on prospective study would be dfficult. The present report and the literature are supportive of the use of preoperative chemotherapy in the initial management of advanced Wilms's tumor extending into the right atrium.     

Prechemotherapy laparoscopic nephrectomy for Wilms' tumor

Laparoscopic radical nephrectomy is emerging as the treatment of choice for localized renal malignancies in adults. Despite the widespread use of laparoscopic nephrectomy for benign renal disease in infants and children, the laparoscopic approach has not been employed for pediatric Wilms' tumor except following systemic chemotherapy. We report the results of laparoscopic radical nephrectomy for removal of unilateral Wilms' tumor prior to the administration of systemic chemotherapy in two patients.     

Late recurrent metastasis in Wilms' tumour

Recurrence of Wilms' tumour after 5 years of disease-free survival is rare. We present the case of a 26-year-old man who had been diagnosed of Wilms' tumour at the age of 6 years treated by surgery, chemotherapy, and radiotherapy and who remained disease free for 8 years, after which lung metastases were detected. Second complete remission was attained with surgery and chemotherapy and 20 years after initial diagnosis he again presented with lung metastases, similarly achieving complete remission with surgery and secondline chemotherapy. The clinical and biological aspects of late metastasis in this neoplasm are discussed. © 1994 Wiley-Liss, Inc.     

Bone marrow micrometastases in a patient with localized Wilms' tumor

The case of a 7-year-old boy presenting at diagnosis with a localized (stage III) Wilms' tumor of favorable histology is presented. Immunocytologic analysis of bone marrow aspirates revealed cells positive for neural cell adhesion molecule (NCAM) and negative for class I major histocompatibility complex (MHC) antigens. These cells were interpreted as deriving from the tumor blastemal component. Postoperatively the child underwent radiotherapy and chemotherapy, and he remains free of disease 12 months after completion of therapy. In patients with non-metastatic Wilms' tumor at onset, the evaluation of the actual frequency of occult marrow involvement and the assessment of its clinical significance may necessitate further investigation. © 1996 Wiley-Liss, Inc.     

Racial variation in incidence of Wilms' tumor: Relationship to congenital anomalies

This is a study of the occurrence of Wilms' tumor and associated anomalies in all incident cases in the Greater Delaware Valley by race. The average annual incidence of Wilms' tumor in this population of 2 million children is significantly higher among nonwhites than whites. A significantly larger proportion of black cases has a Wilms' tumor-associated condition including aniridia, genito-urinary anomalies, the Beckwith-Wiedemann Syndrome, and hemihypertrophy. For very young patients, there was a greater tendency for blacks to have bilateral tumors or a tumor-associated anomaly, features characteristic of the hereditary form of Wilms' tumor. The excess risk of Wilms' tumor among blacks may be a result of a higher proportion having a hereditary predisposition or more common exposure to agents capable of inducing germinal mutations.     

Hemihypertrophy,bilateral Wilms' tumor,and clear-cell adenocarcinoma of the uterine cervix in a young girl

We report the case of a young girl who had hemihypertrophy and developed Wilms' tumor in both kidneys and clear-cell adenocarcinoma of the uterine cervix with no maternal history of stilbestrol ingestion during pregnancy. Karyotype on the second Wilms' tumor showed monosomy 22. The link between hemihypertrophy and Wilms' tumor is well known, but their association with clear-cell carcinoma of the cervix has not been previously described. © 1993 Wiley-Liss, Inc.     

Screening for Wilms' tumor in children with high-risk congenital syndromes: Considerations for an intervention trial

Screening for cancer in children is uncommon. However, in children with congenital syndromes associated with Wilms' tumor, conditions exist that potentially make screening effective. This select population of children 1) are relatively easily identified; 2) have a high incidence of Wilms' tumor; 3) if identified before development of Wilms' tumor, may have a decrement in morbidity/mortality; and 4) are amenable to a simple and acceptable screening technology, renal sonography exams. Many clinicians have recommended screening for cancer in children with congenital syndromes associated with Wilms' tumor. However, neither costs nor effectiveness of such recommendations have been evaluated systematically. The strongest evidence for or against Wilms' tumor screening in this select population would be provided by a randomized screening trial. Prior to undertaking such a trial, the key parameters that dominate the cost and effectiveness of screening should be identified. Simulation models, such as cost-effectiveness analysis, offer a starting point for deciding whether cancer screening is appropriate, and if so, under what set of conditions. We review basic conditions required for a successful screening trial in children with syndromes that are at increased risk of Wilms' tumor. We also discuss the use of cost-effectiveness analysis as a preliminary step in determining the feasibility of an intervention trial. © 1996 Wiley-Liss, Inc.     

Wilms' tumor: Cure of malignant pleural effusion exclusively with chemotherapy

A malignant pleural effusion was diagnosed by thoracentesis in a 12-year-old patient with an established diagnosis of Wilms' tumor. He was treated with chemotherapy and achieved a reduction in the size of the primary tumor and resolution of the effusion. The primary tumor was then resected and chemotherapy continued for a total of 18 months. At the request of the parents, radiation therapy to the thorax was withheld. The patient has remained free of disease for the past 8 years. © 1995 Wi1ey-Liss Inc.     

Diagnosis of veno-occlusive disease of the liver by color-coded Doppler sonography

In a girl with bilateral Wilms' tumor veno-occlusive disease occurred during chemotherapy. The diagnosis was established by reversed flow in the portal vein displayed by color and pulsed Doppler sonography. After successful conservative treatment normalization of flow in the portal vein could be shown by both Doppler techniques.     

Der(16)t(1;16)(q21;q13) in Wilms' tumor: Friend or foe

The der(16)t(1;16)(q21;q13) chromosomal abnormality has been reported rarely in Wilms' tumor. This abnormality has also been found in several other pediatric and adult neoplasms, and may imply a poor prognosis in at least some of these solid tumors. To investigate its clinical significance in Wilms' tumor, we examined the records of 65 consecutive children with Wilms' tumor whose tumor cells were successfully karyotyped. The t(1;16) was present in seven patients (10%) whose ages ranged from 2.5 to 4.7 years (median 3.5 years) at diagnosis. Six of the seven patients were female. All four stages of Wilms' tumor were represented (two patients had stage IV disease). No patient had bilateral disease. All tumors were of “favorable histology.” All seven patients are alive and off therapy with a median follow-up of 3.2 years (range, 2 to 8.5 years). One patient with this abnormality developed brain metastases within 4 months of completion of therapy. Comparison of these patients with the remaining 58 Wilms' tumor patients revealed no significant differences with regard to disease stage, histology, survival, or relapse-free survival. Cytogenetic evidence of der(16)t(1;16)(q21;q13) in Wilms' tumor does not appear to portend an adverse clinical outcome, although only a larger study that includes molecular detection methods can provide more conclusive evidence. © 1996 Wiley-Liss, Inc.     

Reproductive problems and birth defects in survivors of Wilms' tumor and their relatives

In a retrospective cohort study of 47 Wilms' tumor survivors and their 77 sibling controls, female survivors had a fourfold excess risk (risk ratio, 4.1; 95% confidence interval, 1.7-10.1) for any adverse livebirth outcome, including birth defects, compared with their sibling controls. Wives of male survivors had no apparent excess risk for problem pregnancies. The families had a number of severe reproductive problems and major birth defects, such as primary amenorrhea in two survivors, bicornuate uterus in two survivors and one control, and mental retardation in one male survivor and a male control. The son of a female survivor died after bilateral Wilms' tumors. Birth defects in the offspring of female survivors are compatible either with intrauterine constraint, possibly due to radiation-induced fibrosis or with the complex of malformations associated with Wilms' tumor. Female survivors of Wilms' tumor appear to be at increased risk for a variety of reproductive problems, from sterility to fetal loss, early delivery, and birth defects in offspring. Furthermore, relatives of survivors of Wilms' tumor may be at risk of having associated birth defects, with clinically significant consequences.     

Wilms' tumor with transient dermolysis of the newborn: Recurrence of skin lesions during chemotherapy

Transient bullous dermolysis of the new-born consists of congenital skin defects and a tendency for blistering of the skin and mucous membranes during the neonatal period. A case of transient bullous dermolysis of the newborn associated with Wilms' tumor is reported. Transient bullous dermolysis of the newborn does not represent an obstacle to administering appropriate chemotherapy when simple precautions are taken.     

Bilateral Wilm's Tumor: A Case Report and a Survey of Nineteen Cases in Japanese Literature

A eleven month old boy with bilateral Wilms' tumor has been reported. Hemine-phrectomy was performed and the boy lived fifteen months after surgery ultimately dying of metastatic disease. The remaining kidney was free from tumor at autopsy. The nineteen cases of bilateral Wilms' tumor in the Japanese literature were summarized.     

Multiple genetic abnormalities of 11p15 in Wilms' tumor

Wilms' tumor has served as a model of multiple genetic alterations in childhood cancer. This review summarizes work in our laboratory identifying several of these alterations. These include the localization to 11p15 of an embryonal tumor suppressor gene and at least one gene for Beckwith-Wiedemann syndrome, which predisposes to Wilms' tumor; as well as a novel mutational mechanism in man, loss of imprinting. © 1996 Wiley-Liss, Inc.     

Genetics and epidemiology of Wilms' tumor: The French Wilms' tumor study

A complete family history was obtained for 501 patients with Wilms' tumor, treated in departments of pediatric oncology in whole France. The information was collected by self-questionnaire and/or by interview of parents. The proportion of bilateral cases is 4.6% and there are 12 patients (2.4%) with a positive family history of Wilms' tumor. The affected relatives are most often distant and no first degree relative was affected. Apart from the well-known associations with aniridia, hemihypertrophy, genitourinary anomalies, Beckwith-Wiedeemann, and Drash syndromes, there is also a significant excess of congenital heart defects (P = .008) which remains to be explained. Several findings support the bimutational hypothesis such as earlier diagnosis and increased parental age in bilateral cases. No particular anomalies and no increased frequency of childhood cancer were found in patients' relatives. The frequency of Wilms' tumor in relatives was estimated to be less than 0.4% in sibs, 0.06% in unclesand aunts, and 0.04% in first cousins. These figures are very different from those found in retinoblastoma and suggest that the mechanism may be more complex in Wilms' tumor. This conclusion is in agreement with molecular biology studies in tumors and linkage analysis in multiple case families which suggest that more than one locus is involved. © 1992 Wiley-Liss, Inc.     

Response of untreated stage IV Wilms' tumor to single dose carboplatin assessed by “up front” window therapy

A total of nine children with previously untreated stage IV Wilms' tumor of favorable histology were treated according to the Austrian/Hungarian Wilms' Tumor Protocol 89 and received a preoperative single dose of carboplatin as an “up front” window therapy. The treatment consisted of carboplatin as a single-dose of 600 mg/m² over 30 minutes on day 1. Response evaluation by chest X-ray, serial CT scans, and sonography was performed on day 22. Investigation of the abdominal tumors revealed seven partial responses (78%), one nonresponse, and one progressive disease with a median tumor volume reduction of 62%. Response of metastases evaluated by CT scans was as follows: four complete remission, four partial response, and one nonresponse. Thrombocytopenia (WHO grade III 1, grade II 2, grade I 2) and leukocytopenia (WHO grade II 1, grade I 5) were the main side effects. No renal or liver toxicity were observed. The overall response rate after a preoperative single-dose of 600 mg/m² carboplatin in untreated patients with stage IV Wilms' tumor is encouraging and the toxicity acceptable. This data indicate that carboplatin seems to be an additional effective drug in patients with previously untreated Wilms' tumor of favorable histology. © 1995 Wiley-Liss, Inc.     

Laparoscopic treatment of renal cancer in children: A multicentric study and review of oncologic and surgical complications

ObjectiveThe aim was to report a multicentric study with a longer follow-up to evaluate the laparoscopic radical nephrectomy in children with renal cancer.Material and methodsThis was a retrospective multicentric study, from October 2005 to January 2012, of children who underwent a laparoscopic radical nephrectomy for small renal malignant tumors.ResultsSeventeen children were included in this study. Sixteen underwent chemotherapy before surgery according the SIOP (Société Internationale d'Oncologie Pédiatrique) protocol and one was treated by surgery only for a carcinoma. All except one could be treated by laparoscopy; the biggest tumoral size was 8 cm. The median hospital stay was 3 days (2–10). The pathologic examination showed 15 Wilms' tumors, one clear cell sarcoma and one TFE3 renal cell carcinoma. With a median follow-up of 42 months (range 12 and 77 months) after laparoscopic radical nephrectomy, 15 children had no oncological complications (port site or local recurrence, pulmonary metastasis) and one had a local recurrence without intraoperative tumoral rupture. The child with TFE3 renal cell carcinoma died 4 years after surgery from brain and lung metastases without local recurrence. No small bowel obstruction occurred.ConclusionsRadical nephrectomy in children for Wilms' tumor or other renal cancer can be safely performed laparoscopically and our indications can be summarized, for trained laparoscopic surgeons, by small tumors under about 8 cm diameter, especially without crossing the lateral edge of the vertebra on the CT scan at the time of surgery.     

Late orthopedic effects in children with Wilms' tumor treated with abdominal irradiation

Between 1970 and 1984, 31 children with biopsy-proven Wilms' tumor received nephrectomy, chemotherapy, and abdominal irradiation and were followed beyond skeletal maturity. Three patients (10%) developed late orthopedic abnormalities requiring intervention. Ten children received orthovoltage irradiation, and all cases requiring orthopedic intervention or developing a scoliotic curve of >20d? were confined to this group, for a complication frequency of 50%. Those children who developed a significant late orthopedic abnormality (SLOA) as defined were treated to a higher median dose (2,890 cGy) and a larger field size (150 cm²) than those who did not (2,580 cGy and 120 cm²). Age at irradiation, sex, and initial stage of disease did not appear to influence the risk of developing an SLOA. No child who received megavoltage irradiation developed an SLOA despite treatment up to 4,000 cGy or to field sizes of 400 cm². We conclude that modern radiotherapy techniques rarely lead to significant late orthopedic abnormalities previously associated with abdominal irradiation in children with Wilms' tumor.