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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. 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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3. 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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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. 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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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. Balaguer Guill J, Fernández Navarro JM, Cañete Nieto A, Muro Velilla MD, Hernández Martí M, Castel Sánchez V: [Renal tumors in infants aged less than 1 year]. An Pediatr (Barc); 2006 May;64(5):433-8
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  • [Title] [Renal tumors in infants aged less than 1 year].
  • OBJECTIVE: To determine the frequency and distribution of primary renal tumors diagnosed in a pediatric oncology unit in children younger than 1 year and identify their clinical and histopathological characteristics, the treatment used, and outcomes.
  • The most prevalent tumor in this age group was Wilms' tumor (WT) in 15 patients, followed by mesoblastic nephroma (MN) in 9 patients and rhabdoid tumor in 1 patient.
  • Among the 15 WT, 9 were stage I, 1 was stage II, one was stage III, 2 were stage IV, and 1 was stage V.
  • Overall survival at 5 years was 0.67 (SE 0.12) for WT and 0.89 (SE 0.1) for MN, respectively, with a mean follow-up of 290 months.
  • The first-line therapy in these patients is surgery since this type of tumor shows little chemosensitivity and chemotherapy is poorly tolerated in infants.

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  • (PMID = 16756884.001).
  • [ISSN] 1695-4033
  • [Journal-full-title] Anales de pediatría (Barcelona, Spain : 2003)
  • [ISO-abbreviation] An Pediatr (Barc)
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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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. Seseke F, Rebmann S, Zöller G, Lakomek M, Ringert RH: [Risk factors for perioperative complications in renal surgery for Wilms' tumor]. Aktuelle Urol; 2007 Jan;38(1):46-51
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  • [Title] [Risk factors for perioperative complications in renal surgery for Wilms' tumor].
  • [Transliterated title] Risikofaktoren perioperativer Komplikationen in der Chirurgie des Wilms-Tumors.
  • BACKGROUND: There is controversy about preoperative chemotherapy in the treatment of Wilms' tumor.
  • Therefore, risk factors of perioperative complications were analysed in our series of patients with Wilms' tumor with a special focus on the effects of preoperative chemotherapy.
  • PATIENTS AND METHODS: Case histories of 37 patients [mean age 3.9 (range: 0.6 - 14) years] were retrospectively analysed concerning follow-up, clinical and histopathological stage, size of the primary tumor, as well as duration and extent of preoperative chemotherapy.
  • The mean maximal tumor diameter was 9.5 cm (range: 4 - 24 cm).
  • 11/37 patients had no or shortened preoperative chemotherapy.
  • There was one intraoperative tumor rupture, 4 small bowel obstructions, 1 pancreatitis.
  • All complications occurred in patients of clinical stages III and IV, maximal tumor diameter > 10 cm after unusually extended operative procedures.
  • 4 patients showed only poor response to preoperative chemotherapy.
  • Patients with doxorubicin pre-treatment showed a higher risk of postoperative small bowel obstruction.
  • CONCLUSIONS: The risk of perioperative complications was correlated with the local extent of the primary tumor and was higher with those requiring more extensive surgical interventions.
  • The influence of preoperative chemotherapy on the complications rate is inconstant.
  • Considering a good response of the primary tumor, the complication rate will be decreased.
  • However, the comorbidity of more intense preoperative chemotherapy in patients of stage IV may contribute to a higher risk of surgical complications.
  • [MeSH-major] Kidney Neoplasms / drug therapy. Kidney Neoplasms / surgery. Neoadjuvant Therapy. Postoperative Complications / etiology. Wilms Tumor / drug therapy. Wilms Tumor / surgery
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / adverse effects. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Dactinomycin / adverse effects. Dactinomycin / therapeutic use. Doxorubicin / adverse effects. Doxorubicin / therapeutic use. Female. Follow-Up Studies. Humans. Infant. Intestinal Obstruction / chemically induced. Male. Neoplasm Staging. Retrospective Studies. Risk Factors. Vincristine / adverse effects. Vincristine / therapeutic use

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  • (PMID = 17290329.001).
  • [ISSN] 0001-7868
  • [Journal-full-title] Aktuelle Urologie
  • [ISO-abbreviation] Aktuelle Urol
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; SIOP protocol
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11. Fernández-Pineda I, Cabello Laureano R, Fernández-Hurtado MA, Granero Cendón R, Tuduri Limousín I, Morcillo Azcárate J, Aspiazu Salinas D, García Vallés C, De Agustín Asensio JC: [Surgical management of bilateral Wilms' tumor: our experience with 18 cases]. Cir Pediatr; 2009 Oct;22(4):186-8
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  • [Title] [Surgical management of bilateral Wilms' tumor: our experience with 18 cases].
  • [Transliterated title] Manejo quirúrgico del tumor de Wilms bilateral: nuestra experiencia con 18 casos.
  • AIM: To show our experience in the surgical management of bilateral Wilms' tumor.
  • METHODS: We have reviewed the medical records of 18 patients diagnosed of bilateral Wilms' tumor between 1971 and 2007, evaluating age, sex, clinical situation, imaging studies, histology, treatment, complications and follow-up.
  • RESULTS: 65% of patients with synchronous Wilms' tumor was stage I-II, 30% stage III and 5% stage IV.
  • 100% of patients with metachronous Wilms' tumor was stage I-II.
  • 3 of these patients had a renal trasplant with a good evolution.
  • One patient died of a progressive renal failure and two patients died of the evolution of the oncological disease.
  • CONCLUSIONS: Preoperative chemotherapy allows a conservative surgical resection with a high overall survival (80-90%).
  • Individualized surgical treatment offers a conservative surgical resection with a lower incidence of long-term renal failure.
  • [MeSH-major] Kidney Neoplasms / surgery. Neoplasms, Multiple Primary / surgery. Wilms Tumor / surgery

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  • (PMID = 20405651.001).
  • [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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12. Büyükpamukçu M, Köksal Y, Varan A, Atahan L, Cağlar M, Akyüz C, Kutluk T, Büyükpamukçu N: Late recurrence in children with Wilms' tumor. Turk J Pediatr; 2007 Apr-Jun;49(2):226-30
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  • [Title] Late recurrence in children with Wilms' tumor.
  • We aimed in this study to evaluate the clinical and radiological features of the late recurrence of Wilms' tumor in children.
  • Among 553 children diagnosed with Wilms' tumor between 1972 and 2004, four cases were determined to be late recurrences.
  • Clinical, histopathological parameters, treatment details, and outcomes of the patients were evaluated retrospectively.
  • The ages of the patients at the time of diagnosis were 2, 5, 5, and 9 years and the male/female ratio was 1/3.
  • Two patients had stage II disease and two had stage IV characteristics.
  • Initial treatment was surgery and chemotherapy, which included vincristine and actinomycin-D.
  • Recurrence times were 36, 41, 51, and 96 months.
  • At the time of relapse, the chemotherapy protocols were as follows: vincristine, actinomycin-D, adriamycin, and cyclophosphamide in two patients; vincristine, actinomycin-D, and epirubicin in one patient; and vincristine, actinomycin-D, and adriamycin in the last patient.
  • Although the other patient had local spillage, regional lymph node involvement, and renal artery invasion, isolated lung recurrence was observed.
  • Only one patient had progressive disease and is still under treatment, whereas the other patients died with disease.
  • Late recurrence in patients with Wilms' tumor is a poor prognostic factor and should be treated with an intensified regimen.
  • [MeSH-major] Kidney Neoplasms / pathology. Wilms Tumor / pathology

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  • (PMID = 17907529.001).
  • [ISSN] 0041-4301
  • [Journal-full-title] The Turkish journal of pediatrics
  • [ISO-abbreviation] Turk. J. Pediatr.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Turkey
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13. 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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14. 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


15. 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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16. Feusner JH, Green D, Norkool P, Ritchey ML: Chemotherapy dosing for Wilms tumor patients on hemodialysis: Experience of the National Wilms Tumor Study (NWTS). J Clin Oncol; 2004 Jul 15;22(14_suppl):8558

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  • [Title] Chemotherapy dosing for Wilms tumor patients on hemodialysis: Experience of the National Wilms Tumor Study (NWTS).
  • : 8558 Background: There is very little published data re chemotherapy dosing for children with Wilms tumor (WT) on dialysis.
  • METHODS: The treatment and outcome of patients (pts) enrolled on NWTS protocols I-IV (1969 thru 1994) were abstracted from files in the NWTS data center.
  • RESULTS: Twenty-eight pts were identified who had been treated with chemotherapy while being dialyzed.
  • For the remaining with unilateral tumors, the initial stages were: I-5, II-5, III-1, IV-2.
  • At the time of dialysis, eighteen pts were being treated for either relapsed (14) or persistent (4) WT.
  • The chemotherapy utilized was variable and included vincristine (VCR), actinomycin (AMD), doxorubicin (DOX), ifosfamide, etoposide, cisplatin, and carboplatin.
  • Of the 17 deaths, 9 were directly due to WT and 8 to toxicity of end stage renal disease.
  • This is of note since there is some controversy in the literature re the need to dose adjust AMD and DOXO in pts with severe renal insufficiency.
  • Given the mixture of pts, disease states, and complicated renal failure treatments, their survival outcome is difficult to interpret.

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  • (PMID = 28013889.001).
  • [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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17. 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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18. Neville H, Ritchey ML, Shamberger RC, Haase G, Perlman S, Yoshioka T: The occurrence of Wilms tumor in horseshoe kidneys: a report from the National Wilms Tumor Study Group (NWTSG). J Pediatr Surg; 2002 Aug;37(8):1134-7
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  • [Title] The occurrence of Wilms tumor in horseshoe kidneys: a report from the National Wilms Tumor Study Group (NWTSG).
  • BACKGROUND/PURPOSE: An increased incidence of Wilms tumor has been noted in patients with a horseshoe kidney.
  • These represent a difficult diagnostic and therapeutic challenge.
  • The charts of all National Wilms Tumor Study Group (NWTSG) patients with Wilms tumor occurring in a horseshoe kidney were reviewed.
  • Forty-one patients were found to have a Wilms tumor arising in a horseshoe kidney for an incidence of 0.48%.
  • RESULTS: Horseshoe kidney was not recognized preoperatively in 13 patients, 10 of whom were evaluated with computed tomography (CT).
  • Four of the 10 also had renal ultrasonography and one an intravenous pyelogram (IVP).
  • Stage at presentation was stage I, 10 pts; stage II, 10; stage III, 12; stage IV, 6; stage V, 3.
  • Fifteen children were treated with preoperative chemotherapy after initial biopsy of the tumor.
  • The mean total remaining renal parenchyma after all operations (excluding treatment of relapses) was approximately 75%.
  • Two patients had transient renal failure.
  • Although 37% of patients with Wilms tumor arising in a horseshoe kidney were judged inoperable at initial exploration, all were amenable to resection after chemotherapy.
  • [MeSH-major] Congenital Abnormalities / epidemiology. Kidney / abnormalities. Kidney Neoplasms / epidemiology. Wilms Tumor / epidemiology
  • [MeSH-minor] Biopsy. Chemotherapy, Adjuvant. Child, Preschool. Comorbidity. Female. Humans. Incidence. Male. Neoplasm Staging. Nephrectomy. Premedication. Retrospective Studies. Survival Rate. Tomography, X-Ray Computed

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  • [Copyright] Copyright 2002, Elsevier Science (USA). All rights reserved.
  • (PMID = 12149688.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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19. 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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20. 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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21. 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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22. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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23. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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24. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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25. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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26. El Kababri M, Khattab M, El Khorassani M, Hessissen L, Kili A, Nachef MN, Cherradi N, Malihy A, Alhamany Z, Msefer-Alaoui F: [Clear cell sarcoma of the kidney. A study of 13 cases]. Arch Pediatr; 2004 Jul;11(7):794-9
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  • Clear cell sarcoma of the kidney (CCSK) also called a "bone-metastasizing renal tumor of childhood" is the second common pediatric renal neoplasm.
  • This tumor is associated with a higher rate of relapse and a wider distribution of metastases than Wilms' tumor.
  • PATIENTS AND METHODS: We have reviewed records of 13 cases of CCSK among 277 renal tumors (5%) diagnosed at the children's hospital of Rabat between 1990 and 2002.
  • No congenital malformation syndrome or familial Wilms' tumor were observed.
  • Preoperative chemotherapy was given according to the SIOP9, SIOP93-01 and GFAOP 98 protocols.
  • Tumor measurements varied through 450-3450 g and 7-26 cm.
  • The distribution local stage was I: three cases; II: three cases; III: six cases; IV: one case.
  • Postoperative chemotherapy and radiotherapy (21 600-30 600 cGy) was done in 10 cases.
  • CONCLUSION: CCSK remains the pediatric renal tumor most frequently misdiagnosed.
  • Its aggressiveness and its ability to give bone metastases need to recognize early this diagnosis for an adapted treatment.
  • [MeSH-minor] Age of Onset. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Neoadjuvant Therapy. Neoplasm Staging. Prognosis. Retrospective Studies. Sex Factors. Survival Analysis

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  • (PMID = 15234374.001).
  • [ISSN] 0929-693X
  • [Journal-full-title] Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
  • [ISO-abbreviation] Arch Pediatr
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] France
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27. Takamizawa S, Okamoto S, Bishop W, Wen J, Kimura K, Sandler A: Differential apoptosis gene expression in pediatric tumors of the kidney. J Pediatr Surg; 2000 Feb;35(2):390-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND/PURPOSE: Apoptosis, or programmed cell death, is essential in maintaining normal homeostasis of tissues.
  • Variations in expression of such factors may account for some variations in tumor behavior.
  • This study evaluates the expression of apoptotic mRNA species in pediatric renal tumors to determine whether a pattern of differential apoptosis gene expression correlates with tumor grade and type.
  • METHODS: Twenty-five frozen tissue specimens were obtained from patients undergoing biopsy or resection of pediatric renal tumors before chemotherapy: Wilms' tumor stage II (WT-II, n = 4); Wilms' tumor stage III/IV (WT-III/IV, n = 4); clear cell sarcoma of the kidney stage III (CCSK, n = 2); rhabdoid tumor of the kidney stage III/IV (RTK, n = 4); and normal kidney (NK, n = 11).
  • [MeSH-minor] Blotting, Western. Child. Genes, bcl-2 / physiology. HLA-DR Antigens / physiology. Humans. Neoplasm Staging. Neoplasms, Germ Cell and Embryonal / genetics. Neoplasms, Germ Cell and Embryonal / pathology. Neoplasms, Germ Cell and Embryonal / physiopathology. RNA, Messenger / isolation & purification. RNA, Messenger / metabolism. Rhabdoid Tumor / genetics. Rhabdoid Tumor / pathology. Rhabdoid Tumor / physiopathology. Tumor Necrosis Factor-alpha / metabolism. Wilms Tumor / genetics. Wilms Tumor / pathology. Wilms Tumor / physiopathology

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  • (PMID = 10693703.001).
  • [ISSN] 0022-3468
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / HLA-DR Antigens; 0 / RNA, Messenger; 0 / Tumor Necrosis Factor-alpha
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28. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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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