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1. Ehrlich PF, Ferrer FA, Ritchey ML, Anderson JR, Green DM, Grundy PE, Dome JS, Kalapurakal JA, Perlman EJ, Shamberger RC: Hepatic metastasis at diagnosis in patients with Wilms tumor is not an independent adverse prognostic factor for stage IV Wilms tumor: a report from the Children's Oncology Group/National Wilms Tumor Study Group. Ann Surg; 2009 Oct;250(4):642-8
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  • [Title] Hepatic metastasis at diagnosis in patients with Wilms tumor is not an independent adverse prognostic factor for stage IV Wilms tumor: a report from the Children's Oncology Group/National Wilms Tumor Study Group.
  • OBJECTIVE: To determine event free survival (EFS) of children with Wilms tumor (WT) and metastatic liver disease at diagnosis.
  • SUMMARY AND BACKGROUND DATA: We reviewed patients with stage IV Wilms tumor treated on National Wilms Tumor Study 4 and 5 to ascertain if they have a worse prognosis than other Stage IV disease.
  • METHODS: A total of 742 patients (pts) with stage IV disease were assessed for EFS (95% confidence interval [CI]) at 5 years after diagnosis.
  • Cohorts included those who underwent resection of the liver lesions compared with those who received only chemotherapy and radiotherapy.
  • RESULTS: A total of 742 patients with stage IV Wilms tumor were enrolled on NWTS-4 and 5, 111 of who had liver metastases.
  • After chemotherapy and/or radiation, 13 patients underwent liver resection (wedge resection, 7; lobectomy, 5; and trisegmentectomy, 1).
  • In 14 patients, the liver disease disappeared with chemotherapy only.
  • EFS for the patients with metastatic FH Wilms tumor was 75% (95% confidence interval [CI]: (71%, 78%), EFS by Stage IV category was: lung only 76% (95% CI: 72%, 80%) (513 patients); liver, not lung 76% (95% CI: 58%, 87%) (34 patients), liver and lung 70% (95% CI: 57%, 80%) (62 patients), and other sites 64% (95% CI: 42%, 79%) (25 patients).
  • There were no significant differences among stage IV groups (P = 0.60).
  • There was no significant difference in EFS for patients with FH Wilms tumor treated with chemotherapy compared with that of patients treated with chemotherapy and radiation (P = 0.63).
  • CONCLUSION: Liver metastasis at diagnosis is not an adverse prognostic factor for stage IV metastatic FH WT.
  • [MeSH-major] Kidney Neoplasms / pathology. Liver Neoplasms / secondary. Wilms Tumor / secondary
  • [MeSH-minor] Chemotherapy, Adjuvant. Child. Child, Preschool. Female. Hepatectomy. Humans. Infant. Infant, Newborn. Liver Function Tests. Male. Neoplasm Staging. Nephrectomy. Prognosis. Radiotherapy, Adjuvant. Survival Rate. Treatment Outcome

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  • (PMID = 19730241.001).
  • [ISSN] 1528-1140
  • [Journal-full-title] Annals of surgery
  • [ISO-abbreviation] Ann. Surg.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / U10 CA042326; United States / NCI NIH HHS / CA / U10 CA042326-15
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS322334; NLM/ PMC3302661
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2. Dokmak S, Cabral C, Couvelard A, Aussilhou B, Belghiti J, Sauvanet A: Pancreatic metastasis from nephroblastoma: an unusual entity. JOP; 2009;10(4):396-9
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  • [Title] Pancreatic metastasis from nephroblastoma: an unusual entity.
  • CONTEXT: Pancreatic metastasis from renal cell carcinoma is a well-known entity.
  • When metastatic disease is limited to the pancreas, pancreatic resection is the optimal treatment.
  • A nephroblastoma is a frequent childhood cancer but can also occur in adults.
  • A metastatic nephroblastoma mainly affects the lung and the liver.
  • Pancreatic metastases from a nephroblastoma are very rare.
  • CASE REPORT: We report an extremely rare case of pancreatic metastases in a 20-year-old man who had a right nephroblastoma resected at 9 years of age and liver metastases treated by right hepatectomy at 18 years of age.
  • Surgical resection was performed without preoperative chemotherapy because the patient was symptomatic and had already received numerous chemotherapy protocols.
  • Pathological examination confirmed pancreatic and liver metastases from a nephroblastoma composed of blastematous cells mixed with embryonic tubular structures without lymph node metastases.
  • After resection, the patient received adjuvant high dose chemotherapy with autologous hematopoietic stem-cell support.
  • CONCLUSION: This is probably the first case of pancreatic metastasis from a nephroblastoma reported in a living patient.
  • A nephroblastoma, like clear cell renal carcinoma, can be considered a possible etiology of pancreatic metastasis from a primary renal tumor.
  • [MeSH-major] Kidney Neoplasms / pathology. Pancreatic Neoplasms / secondary. Wilms Tumor / pathology
  • [MeSH-minor] Combined Modality Therapy. Drug Therapy / methods. Humans. Male. Pancreaticoduodenectomy / methods. Treatment Outcome. Young Adult

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  • (PMID = 19581742.001).
  • [ISSN] 1590-8577
  • [Journal-full-title] JOP : Journal of the pancreas
  • [ISO-abbreviation] JOP
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
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3. Naguib SF, El Haddad A, El Badawy SA, Zaghloul AS: Multidisciplinary approach to wilms' tumor: a retrospective analytical study of 53 patients. J Egypt Natl Canc Inst; 2008 Dec;20(4):410-23

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  • [Title] Multidisciplinary approach to wilms' tumor: a retrospective analytical study of 53 patients.
  • AIM OF THE WORK: The aim of this work was to assess the epidemiologic aspects, clinico-pathological features and the results of multidisciplinary treatment of Wilms' tumor (WT) in pediatric patients treated at the National Cancer Institute (NCI), Cairo University, between January 2002 and December 2004.
  • Doppler study of the renal vein and vena cava and bone scan were done when needed.
  • Neoadjuvant chemotherapy was given to patients suffering from poor general condition, extensive tumor thrombus in the renal vein, irresectable and bilateral (stage V) nephroblastoma.
  • Otherwise, up-front nephrectomy was the standard therapeutic approach in this study.
  • Stage I and III were the most common (29.4% each), followed by stage II and IV (17.7% each), and finally by stage V (5.9%).
  • Neoadjuvant chemotherapy was given to 27 cases while up-front nephrectomy was undertaken in 26 cases.
  • Intra-operative spillage occurred in 12% of patients who had preoperative chemotherapy and 31% of those who had upfront nephrectomy.
  • Twenty five patients underwent renal bed irradiation only, while in the other 7 whole abdominal irradiation was used.
  • Complete remission (CR) was achieved in 74%, while death during neoadjuvant therapy took place in 4% of the cases.
  • Disease progression during treatment was noticed in 8%.
  • These patients were all treated with radio- and chemotherapy.
  • Therapy-related complications were mainly related to chemotherapy in 49% of patients and surgery in 5.9%.
  • Regional lymph node biopsy and accurate marking of residual disease are essential components of surgical treatment and heroic surgical attempts are unnecessary.
  • Neoadjuvant chemotherapy, which is still a fertile source of debate, could possibly help to avoid excessive post-operative radiotherapy and its potential complications.
  • Tumor stage and age of patient were found to affect the results of treatment of Wilms' tumor; but the only statistically significant determinant of prognosis was histologic differentiation.
  • Finally, further studies including molecular markers are needed to augment therapy for the blastemal predominance subtype or for favorable histology associated with loss of heterozygosity (LOA) at chromosomes 1p and 16q aiming at improved survival.
  • KEY WORDS: Wilms' tumor - Nephroblastoma.

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  • (PMID = 20571600.001).
  • [ISSN] 1110-0362
  • [Journal-full-title] Journal of the Egyptian National Cancer Institute
  • [ISO-abbreviation] J Egypt Natl Canc Inst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
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4. Singam P, Ho C, Hong GE, Mohd A, Tamil AM, Cheok LB, Zainuddin Z: Clinical characteristics of renal cancer in Malaysia : a ten year review. Asian Pac J Cancer Prev; 2010;11(2):503-6
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  • [Title] Clinical characteristics of renal cancer in Malaysia : a ten year review.
  • Renal cancer is rare and its incidence is 1.9 per 100,000 in the Malaysian population, which consists of three major ethnic groups (Malay, Chinese and Indians).
  • The study included all renal cancer patients from a single medical institution over ten years, with a total of 75 cases.
  • Seventy-three patients underwent surgery while 2 received only radiotherapy or chemotherapy.
  • There were 26 (37.4%) patients with Stage I disease, 14 (18.7%) at Stage II, 23 (30.7%) at Stage III and 12 (16%) at Stage IV.
  • The Chinese race presented at mean older age (p= 0.02) and later stage of disease (p= 0.046).
  • Patients above 40 years old had more advanced stage disease (p= 0.023).
  • Tumour histology were clear cell (72%), urothelial cell (13.3%), sarcomatoid cell and nephroblastoma each contributed 2.7%.
  • In conclusion we observed significant influences of age and race in the clinical presentation of renal cancer in our institution based population.
  • [MeSH-major] Carcinoma, Papillary / therapy. Carcinoma, Renal Cell / therapy. Carcinoma, Squamous Cell / therapy. Kidney Neoplasms / therapy. Wilms Tumor / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Combined Modality Therapy. Female. Humans. Incidence. Malaysia. Male. Middle Aged. Neoplasm Staging. Prognosis. Radiotherapy Dosage. Risk Factors. Survival Rate. Time Factors. Young Adult

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  • (PMID = 20843141.001).
  • [ISSN] 2476-762X
  • [Journal-full-title] Asian Pacific journal of cancer prevention : APJCP
  • [ISO-abbreviation] Asian Pac. J. Cancer Prev.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] Thailand
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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5. Stefanowicz J, Sierota D, Połczyńska K, Balcerska A, Stoba C: [Stage IV Wilms' tumour -- analysis of treatment results, according to the SIOP 93-01 protocol, at the Medical University of Gdańsk]. Med Wieku Rozwoj; 2004 Apr-Jun;8(2 Pt 1):193-6
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  • [Title] [Stage IV Wilms' tumour -- analysis of treatment results, according to the SIOP 93-01 protocol, at the Medical University of Gdańsk].
  • [Transliterated title] Guz Wilmsa w IV stadium -- analiza wyników leczenia według protokołu SIOP 93-01 w ośrodku gdańskim.
  • THE AIM OF THE STUDY: To evaluate the results of treatment in patients with Wilm's' tumor in stage IV at the Gdansk Medical University Centre.
  • MATERIAL AND METHODS: Between 1993 and 2002, 44 children with nephroblastoma were treated according to SI0P 93-01 protocol, in the Department of Paediatrics, Haematology, Oncology and Endocrinology of the Medical University of Gdansk.
  • Stage IV tumour was confirmed in 6 children: 3 boys and 3 girls.
  • RESULTS: After preoperative chemotherapy complete regression of metastases was obtained in 3 patients.
  • In l patient there was no effect of preoperative treatment.
  • In all patients nephroblastoma of intermediate histological risk was diagnosed.
  • Among the 6 analysed patients with stage IV of the disease, 5 children are alive.
  • Time of observation is from 42 to 102 months.
  • 1 child died from treatment complications: necrotising enteritis.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Wilms Tumor / drug therapy. Wilms Tumor / pathology
  • [MeSH-minor] Child. Child, Preschool. Dactinomycin / administration & dosage. Disease-Free Survival. Female. Humans. Infant. Male. Neoplasm Staging. Poland. Prognosis. Remission Induction. Retrospective Studies. Risk Factors. Time Factors. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 15738593.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; SIOP protocol
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6. Moran C, Greiner RJ, Mardam-Bey SW 3rd, Hollingsworth CL, Kulbacki E, Wechsler DS: Synchronous occurrence of metastatic Wilms tumor and ganglioneuroma. Pediatr Blood Cancer; 2010 Sep;55(3):562-5
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  • [Title] Synchronous occurrence of metastatic Wilms tumor and ganglioneuroma.
  • We describe a 4-year-old female patient with a persistent paraspinal mass following chemotherapy for Wilms tumor.
  • A discordant response to chemotherapy prompted biopsy of the persistent mass, which revealed a ganglioneuroma.
  • This report highlights the synchronous occurrence of different tumors in the same patient, and suggests that repeat biopsies should be considered when contiguous tumor masses do not respond as expected.
  • [MeSH-major] Ganglioneuroma / pathology. Kidney Neoplasms / pathology. Neoplasms, Multiple Primary / pathology. Spinal Neoplasms / pathology. Wilms Tumor / secondary

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  • [Copyright] 2010 Wiley-Liss, Inc.
  • (PMID = 20658632.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NICHD NIH HHS / HD / K12 HD043494
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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7. Velez MC, Athale UH, Loe W Jr, Warrier RP: Acute perforative appendicitis during preoperative chemotherapy for Wilms tumor. Pediatr Hematol Oncol; 2003 Mar;20(2):147-50
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  • [Title] Acute perforative appendicitis during preoperative chemotherapy for Wilms tumor.
  • Infectious complications are not uncommon in children undergoing treatment for cancer.
  • The authors report the case of a child who developed acute perforative appendicitis requiring appendectomy while on preoperative chemotherapy for Wilms tumor, stage IV, favorable histology.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Appendicitis / complications. Intestinal Perforation / etiology. Kidney Neoplasms / complications. Neoadjuvant Therapy. Wilms Tumor / complications
  • [MeSH-minor] Abdominal Pain / etiology. Acute Disease. Ceftazidime / therapeutic use. Child. Combined Modality Therapy. Dactinomycin / administration & dosage. Doxorubicin / administration & dosage. Drug Therapy, Combination / therapeutic use. Emergencies. Gentamicins / therapeutic use. Humans. Immunocompromised Host. Male. Metronidazole / therapeutic use. Nephrectomy. Rupture, Spontaneous. Vincristine / administration & dosage

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  • (PMID = 12554525.001).
  • [ISSN] 0888-0018
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Gentamicins; 140QMO216E / Metronidazole; 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 9M416Z9QNR / Ceftazidime
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8. Jagt CT, Zuckermann M, Ten Kate F, Taminiau JA, Dijkgraaf MG, Heij H, De Kraker J, Verschuur AC: Veno-occlusive disease as a complication of preoperative chemotherapy for Wilms tumor: A clinico-pathological analysis. Pediatr Blood Cancer; 2009 Dec 15;53(7):1211-5
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  • [Title] Veno-occlusive disease as a complication of preoperative chemotherapy for Wilms tumor: A clinico-pathological analysis.
  • BACKGROUND: Vincristine (VCR) and actinomycin D (ACD) form the backbone of chemotherapeutic regimens of Wilms tumor treatment.
  • OBJECTIVES: To investigate the incidence of VOD after preoperative chemotherapy and assess the effect of dose and frequency of administrating ACD on the occurrence of VOD.
  • METHODS: A single-center retrospective study of patients where liver biopsies were performed after 4 or 8 weeks of preoperative chemotherapy.
  • Patients had localized or metastatic Wilms tumor and were treated according to SIOP 9, 93-1, or 2001 protocol.
  • Long-term hepatic toxicity was assessed 5 years after the end of therapy.
  • Fifty-two percent of all patients had mild-to-severe abnormal liver enzymes 5 years after accomplishing therapy.
  • CONCLUSION: Despite short-course preoperative chemotherapy regimen, patients are at risk of developing histological VOD.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Hepatic Veno-Occlusive Disease / chemically induced. Kidney Neoplasms / drug therapy. Neoadjuvant Therapy / adverse effects. Wilms Tumor / drug therapy
  • [MeSH-minor] Alanine Transaminase / blood. Aspartate Aminotransferases / blood. Chemotherapy, Adjuvant / adverse effects. Child. Child, Preschool. Dactinomycin / administration & dosage. Dactinomycin / adverse effects. Doxorubicin / administration & dosage. Doxorubicin / adverse effects. Epirubicin / administration & dosage. Epirubicin / adverse effects. Female. Humans. Incidence. Infant. Liver / enzymology. Liver / pathology. Liver / ultrasonography. Male. Nephrectomy. Postoperative Complications / chemically induced. Retrospective Studies

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  • (PMID = 19672975.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 3Z8479ZZ5X / Epirubicin; 80168379AG / Doxorubicin; EC 2.6.1.1 / Aspartate Aminotransferases; EC 2.6.1.2 / Alanine Transaminase
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9. Koranyi KI, Ranalli MA: Mycobacterium aurum bacteremia in an immunocompromised child. Pediatr Infect Dis J; 2003 Dec;22(12):1108-9
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  • Mycobacterium aurum was cultured from the Broviac catheter of a 5-year-old child with metastatic Wilms tumor.
  • [MeSH-minor] Anti-Bacterial Agents. Child, Preschool. Drug Therapy, Combination / therapeutic use. Equipment Contamination. Follow-Up Studies. Humans. Kidney Neoplasms / drug therapy. Kidney Neoplasms / immunology. Male. Risk Assessment. Treatment Outcome. Wilms Tumor / drug therapy. Wilms Tumor / immunology

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  • (PMID = 14688579.001).
  • [ISSN] 0891-3668
  • [Journal-full-title] The Pediatric infectious disease journal
  • [ISO-abbreviation] Pediatr. Infect. Dis. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents
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10. Boccon-Gibod L, Rey A, Sandstedt B, Delemarre J, Harms D, Vujanic G, De Kraker J, Weirich A, Tournade MF: Complete necrosis induced by preoperative chemotherapy in Wilms tumor as an indicator of low risk: report of the international society of paediatric oncology (SIOP) nephroblastoma trial and study 9. Med Pediatr Oncol; 2000 Mar;34(3):183-90
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  • [Title] Complete necrosis induced by preoperative chemotherapy in Wilms tumor as an indicator of low risk: report of the international society of paediatric oncology (SIOP) nephroblastoma trial and study 9.
  • BACKGROUND: The SIOP Nephroblastoma therapeutic protocols include a period of preoperative chemotherapy followed by nephrectomy and a period of postoperative chemotherapy.
  • From the outset, identification of low-risk groups has been an aim of the SIOP Nephroblastoma Trials and Studies.
  • Now that 90% of children with Wilms tumor can be cured, attention is even more focused on the identification of patients who could benefit from less aggressive postoperative therapy, thus minimizing the morbidity and late effects associated with treatment.
  • The prognostic implications of total necrosis in nephroblastoma after chemotherapy have not been investigated hitherto.
  • PROCEDURE: Between November 1, 1987 and June 30, 1993, 599 patients referred to the SIOP-9 Nephroblastoma Trial and Study were preoperatively treated and classified as stages I-IV nonanaplastic Wilms tumor.
  • RESULTS: Of these 599 patients, pathologic examination of the nephrectomy specimen revealed a completely necrotic Wilms tumor (CNWT) with no viable tumor remaining in 59 (10%): these comprised 37 stages I-III and 22 stage IV.
  • The only death was related to veno-occlusive disease and occurred in a stage I patient in the month following nephrectomy.
  • Stage IV patients represented 37% of CNWT (vs. only 10% of all other cases of unilateral nonanaplastic Wilms tumor) and had a 100% rate of survival.
  • Children with CNWT were older (mean 59 months vs. 43 months); their tumor at diagnosis was larger and had regressed more significantly at subsequent ultrasound examination.
  • The data also uphold the hypothesis that Wilms tumors of blastemic pattern are most aggressive, but also are extremely responsive to chemotherapy.
  • CONCLUSIONS: Patients with unilateral nonanaplastic WT that showed total necrosis following preoperative chemotherapy had excellent outcome and should benefit from less aggressive postoperative treatment in further trials.
  • Other very responsive tumors, such as Wilms with <10% viable tumor, should also be assessed.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Wilms Tumor / drug therapy
  • [MeSH-minor] Adolescent. Child. Europe. Female. Humans. Male. Necrosis. Neoplasm Staging. Prognosis. Remission Induction. Risk Factors. Survival Analysis. Treatment Outcome

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  • (PMID = 10696124.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, Non-U.S. Gov't
  • [Publication-country] UNITED STATES
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11. Rogers T, Bowley DM, Poole J, Swanepoel P, Wainwright J, Beale P, Pitcher G: Experience and outcomes of nephroblastoma in Johannesburg, 1998 - 2003. Eur J Pediatr Surg; 2007 Feb;17(1):41-4
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  • [Title] Experience and outcomes of nephroblastoma in Johannesburg, 1998 - 2003.
  • The purpose of this study is to document and analyse our experience and the outcomes of children with nephroblastoma over recent years.
  • METHODS: This is a retrospective review of all patients who underwent combined oncological and surgical treatment for nephroblastoma in the Paediatric Oncology Unit between 1998 and 2003.
  • RESULTS: Sixty-three patients were treated for Wilms' tumour; the mean age was 3 years 8 months (range 4 months to 11 years).
  • Preoperative chemotherapy was given in forty-six cases (73 %).
  • The tumour stage distribution was 11/63 stage I (17 %), 11/63 stage II (17 %), 21/63 stage III (33 %), 16/63 stage IV (25 %) and 4/63 stage V (6 %).
  • Postoperative chemotherapy and radiotherapy was given according to the SIOP protocol.
  • Children with stage I and stage II had a disease-free survival at 4 years of 89 %.
  • However, those with stage III, IV and V disease had 4-year survival of 66.75 % (p = 0.07).
  • CONCLUSION: Outcomes for children with cancer have improved dramatically over recent years; however, in the developing world, the scarcity of hospital resources and the overwhelming burden of non-cancer diseases can mean that oncological treatment is extremely challenging.
  • In our society, children tend to present with nephroblastoma at an advanced stage; however, treatment by dedicated, multidisciplinary teams can achieve good results.
  • [MeSH-major] Kidney Neoplasms. Wilms Tumor
  • [MeSH-minor] Child. Child, Preschool. Developing Countries. Female. Humans. Infant. Male. Retrospective Studies. South Africa. Survival Analysis. Treatment Outcome

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  • (PMID = 17407020.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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12. Amel L, Leila BF, Lamia K, Olfa G, Abdelfattah Z, Mondher G, Faouzi M, Chakib K, Abdelatif N, Amor G, Slim BA: [Histologic and prognostic study of nephroblastoma in central Tunisia]. Ann Urol (Paris); 2003 Aug;37(4):164-9
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  • [Title] [Histologic and prognostic study of nephroblastoma in central Tunisia].
  • [Transliterated title] Etude anatomoclinique et pronostique des néphroblastomes dans le centre tunisien.
  • Nephroblastoma is a common malignant tumour in childhood that benefited from therapeutic progress.
  • This is a retrospective study of 35 nephroblastoma diagnosed and treated in the central region of Tunisia within the last 8 years (1991-1999).
  • We report and compare clinical features, therapeutic results and prognostic factors with those reported in the literature.
  • Pre-operative chemotherapy was done in 32 cases and the objective response rate was 58%.
  • Histologic analysis concluded to an anaplastic nephroblastoma in 2 cases.
  • Using the classification of the international society of pediatric oncology, 11.4% of tumours were stage I, 48.6% stage II, 5.7% stage III, 11.4% stage IV and 2.9% stage V.
  • Prognosis of nephroblastoma has been improved with chemotherapy and the pluridisciplinar treatment.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Nephrectomy. Wilms Tumor / drug therapy. Wilms Tumor / pathology

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  • (PMID = 12951705.001).
  • [ISSN] 0003-4401
  • [Journal-full-title] Annales d'urologie
  • [ISO-abbreviation] Ann Urol (Paris)
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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13. Abdallah FK, Macharia WM: Clinical presentation and treatment outcome in children with nephroblastoma in Kenya. East Afr Med J; 2001 Jul;78(7 Suppl):S43-7
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  • [Title] Clinical presentation and treatment outcome in children with nephroblastoma in Kenya.
  • RESULTS: Out of 803 children with cancer, 71 (8.8%) had histologically proven nephroblastoma.
  • At presentation, 1.5% were in stage I, 13.2% stage II, 36.8% stage III, 41.2% stage IV and 7.4% stage V.
  • Eighty five per cent presented with stage III-V disease.
  • Ninety five per cent had nepherectomy and received chemotherapy.
  • Nine patients died before commencement of chemotherapy, two of whom died in the immediate post-operative period.
  • Pre-operative chemotherapy was given to 42% of the patients.
  • Approximately 25.5% of the patients received little or no induction chemotherapy due to unavailability of drugs while only 2.8% received the prescribed maintenance treatment with the remainder getting erratic or no treatment.
  • Overall, only 34.7% remained disease free two years from time of diagnosis.
  • CONCLUSION: Late presentation, poor availability of cytotoxic drugs and frequent treatment interruptions for various reasons have contributed to the poor outcome of nephroblastoma in Kenya.
  • [MeSH-major] Kidney Neoplasms / therapy. Wilms Tumor / therapy
  • [MeSH-minor] Age Distribution. Child. Child, Preschool. Humans. Kenya / epidemiology. Remission Induction. Treatment Outcome

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  • (PMID = 11957249.001).
  • [ISSN] 0012-835X
  • [Journal-full-title] East African medical journal
  • [ISO-abbreviation] East Afr Med J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Kenya
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14. Gandola L, Nantron M, Marchianò A, Pession A, Indolfi P, Di Cataldo A, Collini P, Arcamone G, Fossati Bellani F, Spreafico F: Outcome in stage IV Wilms tumor treated according to the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) trials. J Clin Oncol; 2009 May 20;27(15_suppl):10031

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  • [Title] Outcome in stage IV Wilms tumor treated according to the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) trials.
  • : 10031 Background: Children with metastases at diagnosis for Wilms tumor (WT) still display a worse prognosis compared to localized disease.
  • METHODS: We analyzed survival results in patients with stage IV WT enrolled in the AIEOP CNR92 and TW2003 clinical trials (3/1992-3/2008).
  • Treatment strategy across these trials evolved in terms of sparing whole lung radiotherapy (RT) in case of complete disappearance of lung metastases after primary chemotherapy and a doxorubicin cumulative dose reduction from 360 mg/m<sup>2</sup> to 240 mg/m<sup>2</sup> in TW2003.
  • RESULTS: Of 553 in-study patients aged less than 18 years, 68 (12%) were classified as stage IV (38 patients in CNR92, 30 in TW2003; median age 58 months).
  • Children displaying millimetric nodules visible only on computed tomography scan were excluded.
  • Initial treatment consisted of 6-week 3-drug (vincristine, dactinomycin, doxorubicin) phase in 60 cases, while 8 had up-front nephrectomy.
  • Adjuvant therapy included 8-month 3-drug chemotherapy for non anaplastic "local" tumor stage I to III (flank RT for stage III), or an intensified regimen for anaplastic histology, adding etoposide, carboplatinum and ifosfamide (6 patients).
  • Overall 19 tumor failure occurred (3 in anaplastic tumors): metastases progression 9, abdominal relapse 5 (combined to liver and mediastinum in 1 case each), lung 4, liver 1.
  • CONCLUSIONS: Failure to obtain metastases complete remission, and maybe site other than lung, should be considered for chemotherapy intensification for metastatic WT.
  • The impact of metastatic tumor burden deserves further analysis.

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  • (PMID = 27962576.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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15. Ekenze SO, Agugua-Obianyo NE, Odetunde OA: The challenge of nephroblastoma in a developing country. Ann Oncol; 2006 Oct;17(10):1598-600
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  • [Title] The challenge of nephroblastoma in a developing country.
  • BACKGROUND: Advances in paediatric oncology has tremendously improved the outcome in children with nephroblastoma.
  • OBJECTIVE: To study the outcome and the impediments to the management of nephroblastoma in Southeast Nigeria.
  • METHODS: Analysis of 42 children managed for nephroblastoma over a 10-year period (January 1995-December 2004) at the University of Nigeria Teaching Hospital, Enugu, Nigeria is made.
  • Treatment consisted of nephroureterectomy followed by adjuvant chemotherapy with Vincristine, Actinomycin D and Cyclophosphamide.
  • Adriamycin was added for metastatic disease.
  • Twenty-two children (52.3%) had stage III disease, 13 (31.0%) had stage IV, while the remaining seven (16.7%) children had stage II.
  • Stage I disease was not encountered.
  • Seven children had inoperable tumor requiring pre-operative chemotherapy.
  • Four children died of complications of treatment, while 11 relapsed with poor outcome.
  • CONCLUSION: Morbidity and mortality with nephroblastoma is high in our environment.
  • Late presentation, poverty, ignorance and poor compliance to treatment constitute a great challenge to the paediatric oncologist in a developing country.

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  • (PMID = 16873431.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] England
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16. Glekov IV, Lebedev VI, Belova VP, Dzhabarov FR, Danilova VS, Boĭchenko EI, Sharoev TA: [Radiotherapy in multiple modality treatment of children with nephroblastoma]. Vestn Ross Akad Med Nauk; 2002;(1):68-71
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  • [Title] [Radiotherapy in multiple modality treatment of children with nephroblastoma].
  • [Transliterated title] Luchevaia terapiia v kompleksnom lechenii nefroblastomy u deteĭ.
  • The role of radiotherapy in multiple modality treatment of Wilms' tumor is evaluated in 225 children aged 3 months to 11.5 years (mean age 3.5 years) with stage III-IV.
  • 184 (81.8%) patients presented with stage III, 93.7% with typical nephroblastoma.
  • Intervention was combined with drug and radiotherapy in 99.6% patients.
  • Exposure of the abdominal cavity in total focal doses of 10.5-50.2 Gy (mean dose 28 Gy) was carried out in 219 (97.3%) of 225 patients.
  • Special attention is paid to the incidence of relapses, remote metastases, and survival of patients in relation to prognostic factors (sex, age, stage of tumor process, terms of exposure, and total focal doses).
  • Relapses were more incident during the first year of treatment (in 65% children) and outside the exposed field (72.5% cases).
  • 33.2% patients with stage III developed metastases after 1-49 months; the lungs were involved most often.
  • The absence of relationship between the incidence of local relapses and life span after exposure to a total focal dose of up to 21.6 Gy in comparison with higher doses recommends reduced doses for therapy without notable deterioration of the survival of patients with nephroblastoma.

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  • (PMID = 11882978.001).
  • [ISSN] 0869-6047
  • [Journal-full-title] Vestnik Rossiiskoi akademii meditsinskikh nauk
  • [ISO-abbreviation] Vestn. Akad. Med. Nauk SSSR
  • [Language] RUS
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
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17. Burgos L, Martínez Urrutia MJ, Lobato R, López Santamaría M, Aroca A, Jaureguízar E: [Strategy for high cavoatrial tumor thrombus in children with nephroblastoma]. Cir Pediatr; 2008 Apr;21(2):70-2
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  • [Title] [Strategy for high cavoatrial tumor thrombus in children with nephroblastoma].
  • [Transliterated title] Tratamiento del nefroblastoma con trombosis tumoral cavoatrial en niños.
  • INTRODUCTION: Wilms tumor surgery with intracaval-atrial extension is a challenge.
  • Nine of them had renal-caval (RC) or cavo-atrial(CA) thrombus extension.
  • RESULTS: All cases were treated with pre-postoperative chemotherapy SIOP protocols.
  • A multidisciplinary team was necessary to plan surgery and in all patients thrombus and tumor could be removed under cardiopulmonary by-pass in 3 cases (CPBP).
  • Three cases were stage III, one stage IV and 1 stage II.
  • Conclusions. Tumor size can be significantly reduced by preoperative chemotherapy.
  • [MeSH-major] Heart Diseases / etiology. Kidney Neoplasms / pathology. Neoplastic Cells, Circulating. Vena Cava, Superior. Wilms Tumor / secondary

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  • (PMID = 18624272.001).
  • [ISSN] 0214-1221
  • [Journal-full-title] Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
  • [ISO-abbreviation] Cir Pediatr
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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18. Varan A, Büyükpamukçu N, Cağlar M, Köksal Y, Yalçn B, Akyüz C, Kutluk T, Büyükpamukçu M: Prognostic significance of metastatic site at diagnosis in Wilms' tumor: results from a single center. J Pediatr Hematol Oncol; 2005 Apr;27(4):188-91
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  • [Title] Prognostic significance of metastatic site at diagnosis in Wilms' tumor: results from a single center.
  • The purpose of this study was to identify the prognostic significance of pulmonary and liver involvements present at diagnosis in children with Wilms' tumor.
  • From 1971 to 2002, 57 patients with stage IV Wilms' tumor were diagnosed, treated, and followed in the authors' institution.
  • Metastases were detected by chest radiography, abdominal ultrasonography, and/or thoracoabdominal computed tomography.
  • Nephrectomy with tumor resection was performed and appropriate chemotherapy was given to all the patients.
  • Wilms' tumor patients with liver metastases should be treated with more intensive regimens than those with lung metastases.
  • [MeSH-major] Kidney Neoplasms / pathology. Liver Neoplasms / secondary. Lung Neoplasms / secondary. Wilms Tumor / secondary
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Infant. Male. Prognosis. Radiography, Thoracic. Survival Rate. Tomography, X-Ray Computed


19. Jayasinghe C, Siegler N, Leuschner I, Fleischhack G, Born M, Müller AM: Renal cell carcinoma with Xp11.2 translocation in a 7-year-old boy. Klin Padiatr; 2010 May;222(3):187-9
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  • [Title] Renal cell carcinoma with Xp11.2 translocation in a 7-year-old boy.
  • BACKGROUND: More than 90% of pediatric renal tumors are nephroblastomas while renal cell carcinomas (RCC) are rare in children (< 5%).
  • PATIENT: According to the clinical diagnoses of a nephroblastoma stage IV a 7-year-old boy with a kidney tumor and peripheral pulmonary lesion was preoperatively treated for 8 weeks with Vincristine, Actinomycin D and Adriamycin.
  • There was no tumor regression and the pulmonary lesion was no longer detectable.
  • Hence chemotherapy was put to a halt.
  • CONCLUSION: Fine needle aspiration biopsy (FNA) would have allowed to adjust the tumor subtype.
  • Prognosis of pediatric RCC with translocation seems more favourable than without translocation though definitive evidence will only be possible by documentation in a clinical diagnose-related register.
  • [MeSH-major] Carcinoma, Renal Cell / genetics. Carcinoma, Renal Cell / secondary. Chromosomes, Human, X / genetics. Kidney Neoplasms / genetics. Lung Neoplasms / genetics. Lung Neoplasms / secondary. Sex Chromosome Aberrations. Translocation, Genetic / genetics
  • [MeSH-minor] Child. Humans. Kidney / pathology. Male. Neoplasm Staging. Nephrectomy. Prognosis. Tomography, X-Ray Computed

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  • (PMID = 20514625.001).
  • [ISSN] 1439-3824
  • [Journal-full-title] Klinische Pädiatrie
  • [ISO-abbreviation] Klin Padiatr
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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20. Weirich A, Ludwig R, Graf N, Abel U, Leuschner I, Vujanic GM, Mehls O, Boos J, Beck J, Royer-Pokora B, Voûte PA: Survival in nephroblastoma treated according to the trial and study SIOP-9/GPOH with respect to relapse and morbidity. Ann Oncol; 2004 May;15(5):808-20
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  • [Title] Survival in nephroblastoma treated according to the trial and study SIOP-9/GPOH with respect to relapse and morbidity.
  • BACKGROUND: Recent Wilms' tumor (WT) trials and studies have tried to determine the minimal therapy needed for cure.
  • PATIENTS AND METHODS: From January 1989 to March 1994 the German Society of Pediatric Oncology and Hematology registered 440 patients (median age 2.9 years; 231 male, 209 female) with WTs (preoperative chemotherapy 362) for therapy according to the International Society of Pediatric Oncology Trial and Study 9.
  • Therapy for relapse depended on site of relapse and therapy already received.
  • In preoperatively treated WTs, anaplasia was a strong prognostic factor for death [relative risk (RR) 4.7], followed by poor response to preoperative therapy (RR 3.6), stage IV (RR 3.2) and abdominal stage III (RR 2.2).
  • In the 334 unilateral stage I-IV WTs (median age 3.2 years), diffuse anaplasia (21 patients) had a 5-year relapse-free survival of 38.1%, versus 58.4% in blastemal WTs (25 patients); survival was 42.9% in diffuse anaplasia versus 84% in blastemal WTs.
  • None of 46 patients (median age 1.9 years; 91.3% stages I or II) with differentiated WTs (nine epithelial, 37 stromal) relapsed despite their non-response; two died (one therapy related, one due to bilaterization).
  • In the 25 non-anaplastic bilateral WTs, differentiated cases (one epithelial, eight stromal, 33.3% abdominal stage III) were more frequent (P = 0.048) than in unilateral WTs (one stromal, abdominal stage III relapsed).
  • Three WTs developed renal failure due to Drash syndrome, but none due to tumor therapy.
  • After adriamycin 1.9% of WTs (9% of those receiving 400 mg/m(2)) required therapy for cardiac toxicity.
  • CONCLUSIONS: Initial therapy should be more individualized, taking the above risk groups (age in non-anaplastic WTs, poor response, anaplasia, etc.) into account, as morbidity even after relapse therapy with ifosfamide, carboplatin and etoposide was not high.
  • Milder therapy in low stages of differentiated and of well responding WTs should be tested.

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  • (PMID = 15111352.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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21. Sawicz-Birkowska K, Czernik J, Bagłaj M, Czauderna P, Kantorowicz-Szymik S, Poznański WA, Mańkowski P, Madziara W, Prokurat A, Osemlak J: [Renal neoplasms in children]. Przegl Lek; 2004;61 Suppl 2:20-3
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  • [Title] [Renal neoplasms in children].
  • Nephroblastoma is the most common kidney tumor in Polish children.
  • OBJECTIVE: to present clinical material and outcome of 533 children with renal tumors.
  • MATERIAL: 500 pts with nephroblastoma and 33 of non-Wilms: CMN, RCC,CSSK, RTK and others tumors were registered, mean age 4.5 years between 1993 till 2002.
  • Stage: CS I--148, CS II--191, CS III--114, CS IV--51, CS V--29 pts.
  • All pts with nephroblastoma were treated according to the first national PPGGL 01-92 protocol with pre-operative chemotherapy (ACT, VCR) for CS I-III and ACT, VCR, DOX in pts of stage IV, over the age of 6 months.
  • Pre-operative chemotherapy was done to 93.8% pts.
  • RESULTS: Radical nephrectomy post pre op chemotherapy was performed in 451 (98%) pts over 6 months and in 44 (8.2%) infants less than 6 months with nephroblastoma.
  • Partial nephrectomy for unilateral tumor post preoperative chemotherapy was made in 6 (1.2%).
  • In 26/29 (89.65%) of CS V nephroblastoma kidney sparing surgery was possible, and in 12 uni-lateral nephrectomy was performed.
  • RESULTS: 5-years overall survival of CS I pts (favorable and standard histology) is 93.48%, CS II--96.8%, CS III--84.4%, CS IV--67%, CS V--58%.
  • The results of treatment of 33 pts with non-Wilms renal tumors have improved lately.
  • CONCLUSIONS: The use of systemic neoadjuvant chemotherapy in all pts over 6 months according to the recommendation of SIOP Nephroblastoma protocol (01-92) produced tumor shrinkage, facilitated complete surgical nephrectomy, and was very advantageous in the treatment of renal tumors in children.
  • The results of treatment of non-Wilms tumor have also improved thanks to introduction of new and more aggressive regimens of chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Kidney Neoplasms / surgery. Nephrectomy. Wilms Tumor / drug therapy. Wilms Tumor / surgery
  • [MeSH-minor] Adolescent. Chemotherapy, Adjuvant. Child. Child, Preschool. Disease-Free Survival. Female. Humans. Infant. Infant, Newborn. Male. Neoadjuvant Therapy. Neoplasm Staging. Poland. Prognosis. Remission Induction. Retrospective Studies. Risk Factors. Survival Analysis. Treatment Outcome

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  • (PMID = 15686041.001).
  • [ISSN] 0033-2240
  • [Journal-full-title] Przegla̧d lekarski
  • [ISO-abbreviation] Prz. Lek.
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
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22. Uba AF, Chirdan LB: Childhood Wilms' tumour: prognostic factors in North Central Nigeria. West Afr J Med; 2007 Jul-Sep;26(3):222-5
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  • [Title] Childhood Wilms' tumour: prognostic factors in North Central Nigeria.
  • BACKGROUND: Childhood Wilms' tumour or nephroblastoma represents one of the challenges for paediatric oncologists in developing countries.
  • OBJECTIVE: To review the clinical characteristics and outcome of management of childhood nephroblastoma in North- Central Nigeria.
  • METHODS: The clinical, operative and histological records of children aged 15 years and below, that were managed for Wilms' tumour at the Jos University Teaching Hospital between 1998 and 2005 were retrospectively reviewed.
  • RESULTS: There were 32 children (M:F=1.9:1) with histologically confirmed nephroblastoma seen over the 7-year period.
  • At presentation, 1 (3.1%) patient was in stage I, 8 (25%) stage II, 11 (34.4%) stage III and 12 (37.5%) stage IV.
  • About 72% of the patients presented with stage III-IV disease.
  • Nephrectomy and chemotherapy were the modality of treatment.
  • Fifteen (46.9%) of the patients received little or no induction chemotherapy due to unavailability of drugs while only 12 (37.5%) received the prescribed maintenance treatment with the remainder getting erratic or no treatment.
  • Overall, only 43.8% were alive between 1 and 9 months (median: 6 months) of follow-up period, but there was no survivor at two years after treatment.
  • CONCLUSION: Childhood nephroblastoma has a high mortality rate in north central Nigeria because of late clinical presentation with advanced disease, poor availability of cytotoxic drugs and frequent interruptions in treatment and inadequate follow-up.
  • [MeSH-major] Treatment Outcome. Wilms Tumor / diagnosis
  • [MeSH-minor] Adolescent. Antibiotics, Antineoplastic / therapeutic use. Child. Child, Preschool. Disease Progression. Doxorubicin / therapeutic use. Female. Humans. Infant. Male. Nephrectomy. Nigeria / epidemiology. Prognosis. Retrospective Studies. Time Factors. Vincristine / therapeutic use

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  • (PMID = 18399339.001).
  • [ISSN] 0189-160X
  • [Journal-full-title] West African journal of medicine
  • [ISO-abbreviation] West Afr J Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Nigeria
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin
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23. Watanabe R, Takahashi A, Suzuki M, Toki F, Kanazawa T, Hirato J, Morikawa A, Kuwano H: Adolescent wilms tumor with intraspinal and bone metastases: a case report and the review of literature. J Pediatr Hematol Oncol; 2009 Jan;31(1):45-8
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  • [Title] Adolescent wilms tumor with intraspinal and bone metastases: a case report and the review of literature.
  • A 14-year-old girl was referred for a large tumor of the left kidney, with intraspinal and vertebral metastases.
  • Left nephrectomy and intraspinal tumor resection were performed.
  • The histology of both tumors was nephroblastoma with no anaplasia and favorable histology, and they were diagnosed as stage IV.
  • The tumor bed and vertebras were irradiated.
  • We started chemotherapy according to the DD-4A regimen of Japanese Wilms' Tumor Study Group.
  • Intensive multimodality therapy including DD-4A regimen of National Wilms' Tumor Study can result in long-term disease-free remission.
  • [MeSH-major] Bone Neoplasms / secondary. Kidney Neoplasms / pathology. Spinal Neoplasms / secondary. Wilms Tumor / secondary
  • [MeSH-minor] Adolescent. Combined Modality Therapy. Female. Humans. Magnetic Resonance Imaging. Neoplasm Staging

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  • (PMID = 19125087.001).
  • [ISSN] 1536-3678
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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24. Stefanowicz J, Sierota D, Balcerska A, Stoba C: [Wilms' tumour of unfavorable histology--results of treatment with the SIOP 93-01 protocol at the Gdańsk centre. Preliminary report]. Med Wieku Rozwoj; 2004 Apr-Jun;8(2 Pt 1):197-200
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  • [Title] [Wilms' tumour of unfavorable histology--results of treatment with the SIOP 93-01 protocol at the Gdańsk centre. Preliminary report].
  • BACKGROUND: Wilms' tumour is the most common primary renal tumour of childhood.
  • The aim or the study was to evaluate the results of treatment in patients with unfavourable histology Wilms' tumour.
  • MATERIAL AND METHODS: 44 children were treated for nephroblastoma (Wilms' tumour) according to the SIOP 93-01 protocol in the Department of Paediatrics. Haematology.
  • Among these 2 cases of nephroblastoma with diffuse anaplasia, 6 with sarcomatous stroma and 2 cases with clear cell sarcoma of the kidney were diagnosed.
  • In one patient file tumour structure was undefined due to complete necrosis of the tumour tissue.
  • The staging stratification was as follows: stage I-l, II N (-)-l, II N (+) -3, III- 6, including a neoplasmic occluding thrombus in the inferior vena cava, IV - 0, V-0.
  • Fatal outcome was attributed in one case to cancer progression (primary resistance to chemotherapy after 14 months of treatment) and in the other, to the treatment complications (fungal sepsis after undergoing the third course of chemotherapy Carbo+ VP-16).
  • In the analysed group there were no patients with stage IV disease, no relapses were observed.
  • CONCLUSIONS: our observations support the opinion, that high-grade malignancy histology is the basic unfavourable prognostic factor in nephroblastoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Wilms Tumor / drug therapy. Wilms Tumor / pathology
  • [MeSH-minor] Carcinosarcoma / drug therapy. Carcinosarcoma / pathology. Child. Child, Preschool. Dactinomycin / administration & dosage. Female. Humans. Infant. Male. Neoplasm Staging. Poland. Prognosis. Remission Induction. Retrospective Studies. Risk Factors. Sarcoma, Clear Cell / drug therapy. Sarcoma, Clear Cell / pathology. Survival Analysis. Time Factors. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 15738594.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; SIOP protocol
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25. Lee TP, Tzao C, Liu JH, Yu CP, Hsu HH, Chien HN: Isolated endobronchial metastasis of Wilms' tumor. J Pediatr Surg; 2005 Sep;40(9):e33-5
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  • [Title] Isolated endobronchial metastasis of Wilms' tumor.
  • We report an isolated endobronchial metastasis of Wilms' tumor in a 4-year-old boy.
  • He underwent right nephrectomy on account of Wilms' tumor at the age of 3 years.
  • We performed a wedge resection of a metastasis to the right lower lobe of the lung a year later, followed by chemotherapy and irradiation of the involved lung over a period of 30 weeks.
  • One and a half years later, he developed obstructive pneumonitis of the left lung.
  • Fiberoptic bronchoscopy identified a tumor at the left main bronchus with near total bronchial obstruction, compatible with findings on a computed tomographic scan.
  • Rigid bronchoscopy was performed to core out the obstructing tumor.
  • It was reported as a metastatic Wilms' tumor.
  • Successful reexpansion of the left upper lobe was achieved and he was discharged uneventfully, followed by adjuvant chemotherapy.
  • [MeSH-major] Kidney Neoplasms / pathology. Lung Neoplasms / secondary. Wilms Tumor / secondary
  • [MeSH-minor] Bronchoscopy. Child, Preschool. Combined Modality Therapy. Humans. Male. Pneumonia / etiology

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  • (PMID = 16150331.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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26. Paulino AC, Wen BC, Brown CK, Tannous R, Mayr NA, Zhen WK, Weidner GJ, Hussey DH: Late effects in children treated with radiation therapy for Wilms' tumor. Int J Radiat Oncol Biol Phys; 2000 Mar 15;46(5):1239-46
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  • [Title] Late effects in children treated with radiation therapy for Wilms' tumor.
  • PURPOSE: To determine the frequency and types of late effects in children receiving radiation therapy (RT) for Wilms' tumor.
  • MATERIALS AND METHODS: From 1968 to 1994, 55 children received megavoltage RT at our institution as part of treatment for Wilms' tumor.
  • There were 12 Stage I, eight Stage II, 15 Stage III, six Stage IV, and one Stage V patient.
  • All patients received chemotherapy; the most common agents were actinomycin-D/vincristine/adriamycin in 13 and actinomycin-D/vincristine in 18.
  • RESULTS: Of 42 patients, 13 (31.0%) did not have late effects of treatment.
  • The number of patients who developed muscular hypoplasia, limb length inequality, kyphosis, and iliac wing hypoplasia were seven (16.7%), five (11.9%), three (7.1%), and three (7.1%), respectively.
  • Median time to development of scoliosis was 102 months, with a range of 16-146 months.
  • Only one of 12 Group A patients developed scoliosis.
  • Of 23 patients, five irradiated within 10 days of surgery and one of 19 irradiated after 10 days developed bowel obstruction (p = 0.09, log rank test).
  • Three patients developed hypertension with normal blood urea nitrogen (BUN) and creatinine levels; another patient had chronic renal insufficiency in a nonirradiated kidney.
  • One patient developed diffuse interstitial pneumonitis.
  • Four patients developed benign neoplasms; three were in the RT field (two osteochondroma, one lipoma) and one outside (cervical intraepithelial neoplasia II).
  • There were three second malignancies (chronic myelogenous leukemia at 9 years, osteosarcoma at 11 years, and breast cancer at 25 years after initial diagnosis of nephroblastoma); both solid malignancies occurred in the RT field.
  • CONCLUSIONS: Late effects of therapy were seen in more than two thirds of children treated for Wilms' tumor.
  • [MeSH-major] Kidney Neoplasms / radiotherapy. Radiation Injuries / complications. Wilms Tumor / radiotherapy
  • [MeSH-minor] Child. Child, Preschool. Dose-Response Relationship, Radiation. Female. Fertility / radiation effects. Follow-Up Studies. Humans. Infant. Intestinal Obstruction / etiology. Intestine, Small / radiation effects. Kidney Diseases / etiology. Kyphosis / etiology. Male. Muscles / radiation effects. Neoplasm Staging. Neoplasms, Second Primary / etiology. Puberty, Delayed / etiology. Scoliosis / etiology. Time Factors

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  • (PMID = 10725637.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
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27. Schwinger W, Klass V, Benesch M, Lackner H, Dornbusch HJ, Sovinz P, Moser A, Schwantzer G, Urban C: Feasibility of high-dose interleukin-2 in heavily pretreated pediatric cancer patients. Ann Oncol; 2005 Jul;16(7):1199-206
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  • BACKGROUND: The administration of high-dose interleukin-2 (IL-2) seems to be a therapeutic option for children with refractory and metastatic solid malignancies.
  • METHODS: We prospectively studied treatment-related toxicities, quality of life and laboratory parameters in 10 children with progressive or metastatic solid tumors (metastatic osteosarcoma, n=4; neuroblastoma stage IV, n=3; metastatic Ewing's sarcoma, n=2; metastatic Wilms' tumor, n=1) during IL-2 therapy.
  • RESULTS: All patients developed fever >39 degrees C and influenza-like symptoms, with a significant decrease in Karnofsky score.
  • In two patients treatment had to be stopped after three cycles because of severe side-effects.
  • During IL-2 therapy a statistical significant increase in white blood cells (WBC), creatinine, gamma-glutamyltransferase, C-reactive protein, glucose and body weight was observed.
  • No constant quantitative changes in total lymphocytes and subsets were observed during IL-2 therapy.
  • CONCLUSIONS: IL-2 treatment in children with refractory and relapsed solid malignancies is associated with severe, but reversible, side-effects.
  • However, five of the 10 patients with diseases of worst prognosis could be rescued by this treatment.

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  • (PMID = 15849223.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Interleukin-2
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28. Hadley GP, Govender D, Landers G: Malignant solid tumours in neonates: an African perspective. Pediatr Surg Int; 2002 Dec;18(8):653-7

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  • Neuroblastoma (NB) was the commonest tumour seen (11), but the soft-tissue sarcomas were the dominant group (14).
  • Chemotherapy, despite appropriate dose reduction, had significant morbidity and mortality.
  • Stage I disease was associated with a good prognosis, whilst stage IV disease was uniformly fatal.
  • Stage IVs disease had only 50% early survival.
  • Patients with renal tumours, whether nephroblastoma or mesoblastic nephroma, did well.
  • [MeSH-minor] Female. Humans. Infant, Newborn. Male. Neoplasm Staging. South Africa / epidemiology. Survival Analysis. Treatment Outcome

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  • (PMID = 12598957.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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29. Linni K, Urban C, Lackner H, Höllwarth ME: Nephron-sparing procedures in 11 patients with Wilms' tumor. Pediatr Surg Int; 2003 Aug;19(6):457-62
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  • [Title] Nephron-sparing procedures in 11 patients with Wilms' tumor.
  • PURPOSE: In unilateral Wilms' tumor (WT), tumor nephrectomy is the standard surgical approach, whereas partial nephrectomy (PN) is controversially discussed.
  • The aim of our retrospective study was to show that in selected cases of unilateral WT kidney-sparing operations could be a reasonable alternative to nephrectomy and to discuss the results of patients with bilateral WT treated by tumor enucleation.
  • MATERIALS AND METHODS: From 1981 to 1998, seven patients with unilateral nephroblastoma (four stage I, one stage III and two stage IV) had tumor resection by PN (five right side, two left side), which was planned when the tumor volume was reduced after 4 to 6 weeks of chemotherapy by at least 50%, when the tumor occupied one pole or was easily resectable, when 50% or more of the kidney tissue remained and when paraaortic lymph nodes were free by intraoperative histological examination.
  • In four patients with bilateral WT (stage V) bilateral tumor enucleation was carried out-except in one patient in whom the contralateral kidney had to been removed because of extension of the tumor via the inferior vena cava to the right atrium.
  • All patients ( n = 11) received pre- and postoperative chemotherapy followed by radiotherapy in four patients.
  • RESULTS: All patients with unilateral WT ( n = 7) are still alive and disease free (follow-up time: mean 6.6 years, range: 28 months to 11 years) with normal renal function, although two patients with secondary nephrectomy revealed creatinine clearance levels at the lower range.
  • In a stage III tumor patient (intraperitoneal metastasis, free lymph nodes), secondary nephrectomy was necessary due to renal arterial thrombosis 2 days after PN.
  • In one stage IV tumor patient (lung metastasis, free lymph nodes), the primary resection was not far enough away from the tumor margin so that an additional slice of tissue with then tumor-free margins had to be resected.
  • In the group of bilateral WT patients ( n = 4), one child died 2 months after surgery during chemotherapy because of central venous line sepsis.
  • One patient who additionally suffered from inferior vena cava tumor thrombosis extending to the right atrium making nephrectomy of the right kidney necessary developed chronic renal failure 4.7 years postoperatively.
  • The other two stage V tumor patients have creatinine clearance levels within the normal range.
  • CONCLUSIONS: Kidney-sparing procedures remain the operative approach of choice in patients with bilateral WT, but bear the risk of chronic renal failure when one kidney has to be removed.
  • PN in children with unilateral WT, carried out by an experienced surgeon, is a reasonable alternative to nephrectomy if strict guidelines such as excellent tumor response to preoperative chemotherapy and easy resectability far away from the tumor margins through healthy kidney tissue are followed.
  • Paraaortic lymph nodes must be free of tumor invasion in order to avoid local radiotherapy.
  • PN prevents the patient from having to have dialysis in cases of contralateral nephrectomy resulting from metachronous WT or subsequent renal trauma.
  • [MeSH-major] Kidney Neoplasms / surgery. Nephrectomy / methods. Wilms Tumor / surgery
  • [MeSH-minor] Algorithms. Child. Child, Preschool. Female. Humans. Infant. Male. Postoperative Complications. Retrospective Studies. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 12756594.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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