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Items 1 to 34 of about 34
1. 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

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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2. Tucci S Jr, Cologna AJ, Suaid HJ, Valera ET, Tirapelli LF, Paschoalin EL, Martins AC: Results of novel strategies for treatment of Wilms' tumor. Int Braz J Urol; 2007 Mar-Apr;33(2):195-201; discussion 201-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Results of novel strategies for treatment of Wilms' tumor.
  • OBJECTIVE: To evaluate treatment outcomes in Wilms' tumor (WT).
  • Treatment consisted of surgical excision plus adjuvant (40 children) or neoadjuvant and adjuvant chemotherapy (unresectable tumor, n=8, or caval tumor extension, n=5).
  • Chemotherapy and radiotherapy followed protocols of Brazilian Wilms' Tumor Study Group excepting 16 cases with stage I disease that received a short duration postoperative treatment with vincristine (VCR - 11 doses) and dactinomycin (AMD - 4 doses).
  • One patient with resistant relapsed WT was treated by high-dose conditioning chemotherapy with stem cell rescue.
  • Short duration therapy for stage I tumor showed a disease-free survival rate of 100% in a median time of 101 months (range 14 to 248 months).
  • The child treated with high-dose chemotherapy plus stem cell transplant is alive without evidence of disease 84 months from relapse.
  • CONCLUSION: The postoperative chemotherapy in stage I disease can be reduced without compromising the cure rate.
  • The treatment of unfavorable stage III and IV disease or relapsed tumor remains a challenge.
  • [MeSH-major] Kidney Neoplasms / surgery. Wilms Tumor / surgery
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Combined Modality Therapy / methods. Disease-Free Survival. Female. Follow-Up Studies. Humans. Infant. Male. Neoplasm Staging. Nephrectomy. Recurrence

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  • [CommentIn] Int Braz J Urol. 2007 May-Jun;33(3):424-5 [17626664.001]
  • (PMID = 17488540.001).
  • [ISSN] 1677-5538
  • [Journal-full-title] International braz j urol : official journal of the Brazilian Society of Urology
  • [ISO-abbreviation] Int Braz J Urol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Brazil
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3. Shamberger RC, Anderson JR, Breslow NE, Perlman EJ, Beckwith JB, Ritchey ML, Haase GM, Donaldson M, Grundy PE, Weetman R, Coppes MJ, Malogolowkin M, Shearer PD, Kletzel M, Thomas PR, Macklis R, Huff V, Weeks DA, Green DM: Long-term outcomes for infants with very low risk Wilms tumor treated with surgery alone in National Wilms Tumor Study-5. Ann Surg; 2010 Mar;251(3):555-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Long-term outcomes for infants with very low risk Wilms tumor treated with surgery alone in National Wilms Tumor Study-5.
  • OBJECTIVE: To determine the event-free survival (EFS) and overall survival (OS) of children with very low risk Wilms tumor (VLRWT) treated with surgery only.
  • BACKGROUND: Previous studies suggested that postoperative chemotherapy had not improved the prognosis of children with VLRWT.
  • A total of 77 children <24 months of age with small (<550 g) Stage I favorable histology Wilms tumors were treated with surgery only.
  • Of these patients, 21 enrolled at the time of closure were recalled, treated with dactinomycin and vincristine (regimen EE4A), and censored for analysis thereafter.
  • One death was observed in each treatment group.
  • CONCLUSION: The surgery-only EFS was lower than anticipated but, coupled with a much higher than anticipated salvage rate of the chemotherapy naive patients whose disease recurred, led to an observed long-term OS equivalent to that seen with 2-drug chemotherapy.
  • This approach to the treatment of patients with VLRWT eliminates the toxic side-effects of chemotherapy for a large majority of patients.

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  • (PMID = 20142733.001).
  • [ISSN] 1528-1140
  • [Journal-full-title] Annals of surgery
  • [ISO-abbreviation] Ann. Surg.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / 5 U10-CA098413; United States / NCI NIH HHS / CA / U10 CA098413; United States / NCI NIH HHS / CA / U10 CA042326-15; United States / NCI NIH HHS / CA / R01 CA054498; United States / NCI NIH HHS / CA / CA-42326; United States / NCI NIH HHS / CA / U10 CA042326
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS169913; NLM/ PMC2836016
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4. Jacobs S, Fox E, Krailo M, Hartley G, Navid F, Wexler L, Blaney SM, Goodwin A, Goodspeed W, Balis FM, Adamson PC, Widemann BC: Phase II trial of ixabepilone administered daily for five days in children and young adults with refractory solid tumors: a report from the children's oncology group. Clin Cancer Res; 2010 Jan 15;16(2):750-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • PURPOSE: Ixabepilone is a microtubule-stabilizing agent with activity in adult solid tumors and in pediatric tumor xenograft models that are resistant to paclitaxel.
  • This study aimed to determine the response rate to ixabepilone in six solid tumor strata in children and young adults.
  • EXPERIMENTAL DESIGN: We conducted a phase II trial of ixabepilone (8 mg/m(2)/dose for 5 days every 21 days) using a two-stage design in taxane-naïve children and young adults with treatment-refractory, measurable rhabdomyosarcoma, Ewing sarcoma family tumors, osteosarcoma, synovial sarcoma, or malignant peripheral nerve sheath tumor, neuroblastoma, and Wilms tumor.
  • Seven patients received >or=3 cycles, and two had prolonged stable disease (Wilms' tumor, 38 cycles; synovial sarcoma, 8 cycles).

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  • (PMID = 20068084.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / U10 CA098413; United States / NCI NIH HHS / CA / U10 CA 98543; United States / Intramural NIH HHS / / ; United States / NCI NIH HHS / CA / U10 CA098543-08; None / None / / U10 CA098543-08; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / U10 CA098413-08; None / None / / U10 CA098413-08
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Epothilones; K27005NP0A / ixabepilone
  • [Other-IDs] NLM/ NIHMS160304; NLM/ PMC3086796
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5. Malogolowkin M, Cotton CA, Green DM, Breslow NE, Perlman E, Miser J, Ritchey ML, Thomas PR, Grundy PE, D'Angio GJ, Beckwith JB, Shamberger RC, Haase GM, Donaldson M, Weetman R, Coppes MJ, Shearer P, Coccia P, Kletzel M, Macklis R, Tomlinson G, Huff V, Newbury R, Weeks D, National Wilms Tumor Study Group: Treatment of Wilms tumor relapsing after initial treatment with vincristine, actinomycin D, and doxorubicin. A report from the National Wilms Tumor Study Group. Pediatr Blood Cancer; 2008 Feb;50(2):236-41
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  • [Title] Treatment of Wilms tumor relapsing after initial treatment with vincristine, actinomycin D, and doxorubicin. A report from the National Wilms Tumor Study Group.
  • OBJECTIVE: We evaluated the use of alternating cycles of cyclophosphamide/etoposide and carboplatin/etoposide in children entered on National Wilms Tumor Study (NWTS)-5 who were diagnosed between August 1, 1995 and May 31, 2002 and who relapsed after chemotherapy with vincristine, actinomycin D, and doxorubicin (VAD) and radiation therapy (DD-4A).
  • PATIENTS AND METHODS: One hundred three patients who relapsed or had progressive disease after initial VAD chemotherapy and radiation therapy were registered on stratum C of the NWTS-5 Relapse protocol.
  • Twelve patients were not evaluable: five due to insufficient data, six due to major protocol violations, and one for refusal of therapy.
  • Among the 91 remaining patients, 14 with stage V Wilms tumor (WT), 1 with contralateral relapse, and 16 who did not achieve a complete response (CR) to the initial three-drug chemotherapy were not included in this analysis.
  • Relapse treatment included alternating courses of the drug pairs cyclophosphamide/etoposide and carboplatin/etoposide, surgery, and radiation therapy.
  • CONCLUSION: These results demonstrate that approximately one-half of children with unilateral WT who relapse after initial treatment with VAD and radiation therapy can be successfully retreated.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Kidney Neoplasms / drug therapy. Wilms Tumor / drug therapy
  • [MeSH-minor] Carboplatin / administration & dosage. Child, Preschool. Combined Modality Therapy. Dactinomycin / administration & dosage. Doxorubicin / administration & dosage. Drug Administration Schedule. Etoposide / administration & dosage. Female. Humans. Infant. Infant, Newborn. Male. Neoplasm Staging. Recurrence

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17539021.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA-42326
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; BG3F62OND5 / Carboplatin
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6. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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7. Brisse HJ, Schleiermacher G, Sarnacki S, Helfre S, Philippe-Chomette P, Boccon-Gibod L, Peuchmaur M, Mosseri V, Aigrain Y, Neuenschwander S: Preoperative Wilms tumor rupture: a retrospective study of 57 patients. Cancer; 2008 Jul 1;113(1):202-13
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  • [Title] Preoperative Wilms tumor rupture: a retrospective study of 57 patients.
  • BACKGROUND: According to current International Society of Pediatric Oncology (SIOP) Wilms recommendations, all preoperative tumor ruptures should be classified as stage IIIc.
  • METHODS: The authors performed a retrospective analysis of 57 children with clinical and/or radiologic (computed tomography [CT]) signs of preoperative tumor rupture of a series of 250 patients enrolled in Wilms SIOP protocols at their institution.
  • Surgery was performed after chemotherapy in 55 of 57 patients.
  • Peritoneal disease recurrence occurred in 3 of 57 patients, including 2 patients with stage III tumors who had initial intraperitoneal rupture and 1 patient with a stage I tumor.
  • Among the 48 patients who had radiologic signs of retroperitoneal-only rupture, the final pathologic stage was stage III in 22 patients, stage II in 9 patients, and stage I in 17 patients, and no abdominal disease recurrence was observed, although only 23 of 48 patients received flank radiotherapy.
  • CONCLUSIONS: The use of CT scans significantly increased the number of patients who could be classified with "tumor rupture."
  • In contrast, patients who have radiologic signs of localized retroperitoneal-only rupture at diagnosis most likely should not be upstaged, and their treatment may be determined according to pathologic stage only.
  • [MeSH-major] Kidney Neoplasms / complications. Rupture, Spontaneous / complications. Wilms Tumor / complications
  • [MeSH-minor] Chemotherapy, Adjuvant. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Humans. Infant. Male. Neoadjuvant Therapy. Neoplasm Recurrence, Local. Preoperative Care. Retrospective Studies. Tomography, X-Ray Computed

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  • [Copyright] (Copyright) 2008 American Cancer Society.
  • (PMID = 18457331.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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8. Sinescu I, Manu MA, Hârza M, Serbãnescu B, Stefan B, Cerempei V, Tacu D, Kerezsy E, Bucşa C, Domnişor L, Daia D, Constantinescu I: Renal transplantation--substitution therapy in advanced stage uremia. J Med Life; 2008 Apr-Jun;1(2):108-17
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  • [Title] Renal transplantation--substitution therapy in advanced stage uremia.
  • Advanced stage chronic renal failure (CRF) uremia represents one of the most severe metabolic "catastrophes" of the organism.
  • The current therapeutic possibilities consist in: hemodialysis, peritoneal dialysis and renal transplantation.
  • This paper presents the experience of the Urological Surgery, Dialysis and Renal Transplantation Center of the "Fundcni" Clinical Institute in renal transplantation, the single complete morphological and functional therapeutic option in CRF.
  • During the last 10 years, renal transplantations with kidneys from brain dead donors (multiorgan harvesting) to an adult (1997), a child (1999), a diabetic recipient (1998) and an unephric child due to bilateral Wilms' tumor (2005) were performed at "Fundeni" Renal Transplantation Center as a national première.
  • A number of 870 renal transplantations with 82% functionality rate of the grati at 10 years were performed and reported.
  • Owing to obtained results (60% of all transplanted and functional orgmans in Romania on December 2007) and to its achieved performances, the Fundeni Center represents a reference point on the European map of renal transplant.
  • [MeSH-major] Graft Rejection / drug therapy. Immunosuppressive Agents / therapeutic use. Kidney Failure, Chronic / surgery. Kidney Transplantation. Uremia / surgery
  • [MeSH-minor] Humans. Severity of Illness Index. Tissue Donors

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  • [Cites] Am J Kidney Dis. 1995 Aug;26(2):387-98 [7645548.001]
  • (PMID = 20108457.001).
  • [ISSN] 1844-122X
  • [Journal-full-title] Journal of medicine and life
  • [ISO-abbreviation] J Med Life
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Romania
  • [Chemical-registry-number] 0 / Immunosuppressive Agents
  • [Number-of-references] 20
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9. Pascual Samaniego M, Calleja Escudero J, Alvarez Gago T, Gonzalo Rodríguez V, Müller Arteaga C, Fernández del Busto E: [Adult Wilms' tumor]. Actas Urol Esp; 2004 Jul-Aug;28(7):544-8
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  • [Title] [Adult Wilms' tumor].
  • [Transliterated title] Tumor de Wilms del adulto.
  • Wilms' tumor is a malignant embryonic renal neoplasm that is exceptional in adults.
  • There are not clinical data or radiographic investigations that can distinguish it from renal cell carcinoma.
  • The prognosis of Wilms' tumor in adults is worse than in children because of the high recurrence, the lower response rate to chemotherapy regimens and the advanced stage at the time of clinical presentation, like an asymptomatic abdominal mass in 75% of the cases.
  • We report a new case of nephroblastoma in a 29 years old woman presenting like a renal colic, with a cystic configuration by abdominal ultrasound initially, that changed into a solid renal mass later.
  • There is not a definitive treatment protocol currently but some authors suggest a combination chemotherapy with carboplatin and etoposide because it is very effective in recurrent or refractory adult Wilms' tumor.
  • [MeSH-major] Kidney / pathology. Kidney Neoplasms / pathology. Wilms Tumor / pathology
  • [MeSH-minor] Adult. Female. Humans. Nephrectomy / methods. Tomography, X-Ray Computed. Treatment Outcome. Urography

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  • (PMID = 15384282.001).
  • [ISSN] 0210-4806
  • [Journal-full-title] Actas urologicas espanolas
  • [ISO-abbreviation] Actas Urol Esp
  • [Language] spa
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Spain
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10. Blakely ML, Shamberger RC, Norkool P, Beckwith JB, Green DM, Ritchey ML, National Wilms' Tumor Study Group: Outcome of children with cystic partially differentiated nephroblastoma treated with or without chemotherapy. J Pediatr Surg; 2003 Jun;38(6):897-900
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  • [Title] Outcome of children with cystic partially differentiated nephroblastoma treated with or without chemotherapy.
  • BACKGROUND/PURPOSE: Cystic partially differentiated nephroblastoma (CPDN) is a rare variant of Wilms' tumor thought to be more favorable than standard nephroblastoma.
  • The purpose of this report is to examine the outcome of children with CPDN, after nephrectomy, treated with vincristine and dactinomycin based chemotherapy (+/- doxorubicin) or no chemotherapy.
  • METHODS: Patients were registered with the National Wilms' Tumor Study Group (NWTSG) and data were collected prospectively.
  • Thirteen patients received chemotherapy, and 8 patients did not.
  • In the chemotherapy group the stage distribution was as follows: stage I (n = 10), stage II (n = 2), stage V (n = 1).
  • In the no-chemotherapy group, all 8 patients were stage I.
  • All patients had complete tumor resection.
  • In patients receiving chemotherapy, 30% (n = 4) had toxicities causing dose reduction.
  • For stage I patients, treatment with complete tumor resection appears to be as efficacious as nephrectomy plus chemotherapy.
  • Stage II patients also have excellent outcome when treated with tumor resection and postoperative vincristine and dactinomycin.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Diseases, Cystic / drug therapy. Kidney Neoplasms / drug therapy. Wilms Tumor / drug therapy. Wilms Tumor / pathology
  • [MeSH-minor] Adolescent. Adult. Cell Differentiation / drug effects. Child. Child, Preschool. Dactinomycin / administration & dosage. Diagnosis, Differential. Doxorubicin / administration & dosage. Female. Follow-Up Studies. Humans. Male. Neoplasm Staging. Nephrectomy. Survival Analysis. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 12778388.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA-42326; United States / NICHD NIH HHS / HD / K23-HD/RR01473-01
  • [Publication-type] Journal Article; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin
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11. Stehr M, Deilmann K, Haas RJ, Dietz HG: Surgical complications in the treatment of Wilms' tumor. Eur J Pediatr Surg; 2005 Dec;15(6):414-9
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  • [Title] Surgical complications in the treatment of Wilms' tumor.
  • We present our data on the treatment of Wilms' Tumor (WT) with an emphasis on both the positive effect and the adverse effect of preoperative chemotherapy with regard to surgical intervention.
  • 57 % received preoperative chemotherapy (ChTx) and 43 % were operated on primarily.
  • After preoperative ChTx 54 % of the cases were regrouped as stage I, whereas after primary operation 46 % of the patients were grouped as stage I, thus indicating a downstaging with preoperative ChTx.
  • In 8 % of the patients with preoperative chemotherapy intraoperative complications occurred with a rupture of the tumor in 1 case.
  • In contrast, there were intraoperative complications in 25 % of the patients with a primary operation with rupture of the tumor in 3 cases.
  • 1 child (1.5 %) was treated with chemotherapy who did not have a Wilms' tumor but a benign nephroma (CMN).
  • 3 cases had a clear cell sarcoma (CCSK) and in one case histology revealed a rhabdoid tumor (MRTK).
  • Irrespective of the known adverse effects such as changing tumor histology, which may affect the correct staging, and the remaining risk of an initial inadequate treatment, our data show that the regimen of preoperative chemotherapy as proposed by the SIOP study should not be abandoned.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Intraoperative Complications. Kidney Neoplasms / drug therapy. Kidney Neoplasms / surgery. Nephrectomy. Wilms Tumor / drug therapy. Wilms Tumor / surgery
  • [MeSH-minor] Child, Preschool. Dactinomycin / administration & dosage. Humans. Neoadjuvant Therapy. Neoplasm Staging. Postoperative Complications. Vincristine / administration & dosage

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  • (PMID = 16418959.001).
  • [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
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine
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12. Vithayasai N, Ningsanond V, Niramis R: Wilms' tumor with metastasis to duodenum - the first reported case in Thailand. J Med Assoc Thai; 2000 Sep;83(9):1116-9
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  • [Title] Wilms' tumor with metastasis to duodenum - the first reported case in Thailand.
  • A case of Wilms' tumor with gastrointestinal tract metastasis of the duodenum was documented in a 22 month - old - child.
  • She had been diagnosed with Wilms' tumor stage III, treated surgically and by radiation and chemotherapy about 1 year previously.
  • While she was still on chemotherapy, she developed hematemesis and clinical signs of partial gut obstruction.
  • Gastroscopy and upper gastrointestinal series showed an intraluminal vascular mass in the duodenal bulb and histologically proved to be Wilms' tumor.
  • We believe this is the first report in the world of Wilms' tumor with gastrointestinal tract metastasis.
  • [MeSH-major] Duodenal Neoplasms / secondary. Kidney Neoplasms / pathology. Wilms Tumor / secondary

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  • (PMID = 11075982.001).
  • [ISSN] 0125-2208
  • [Journal-full-title] Journal of the Medical Association of Thailand = Chotmaihet thangphaet
  • [ISO-abbreviation] J Med Assoc Thai
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] THAILAND
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13. Tang JY, Pan C, Xu M, Xue HL, Chen J, Zhao HL, Gu LL, Wang YP: [Results of Wilms' tumor trial (WT-99) in Shanghai children's medical center]. Zhonghua Er Ke Za Zhi; 2003 Feb;41(2):131-4
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  • [Title] [Results of Wilms' tumor trial (WT-99) in Shanghai children's medical center].
  • OBJECTIVE: Wilms' Tumor Trial (WT-99) of Shanghai Children's Medical Center was designed and conducted by applying therapeutic regimens stratified by stage and histology in accordance with National Wilms' Tumor Study (NWTS) criteria of U.S.A.
  • The main aim of WT-99 was to reduce treatment of low-stage, favorable-histology (FH) tumors without impairing survival and to improve prognosis of stage III and IV (FH) and unfavorable-histology (UFH) tremors with more intensive chemotherapy.
  • METHODS: Diagnosis and treatment was decided by the multi-disciplinary team including oncologists, surgeons, pathologists, radiologists and diagnostic radiologists.
  • The regimen for patients at favorable-histology (FH) stage I and II and anaplastic stage I was vincristine (Vcr) and dactinomycin (Act-D) only, while for those at focal anaplastic stage II to IV and FH stage III and IV the regimen was Vcr, Act-D and adriamycin (Adr).
  • Patients at diffuse anaplastic stage II to IV and clear cell stage I to IV received four-drug regimen including Vcr, etoposide (VP16), Adr and cytoxan (CTX).
  • For those at rhabdoid stage I to IV the regimen was carboplatin, VP-16 and CTX.
  • Un-resectable patients received 2 courses of Ifosfamide, Vcr and VP-16 as pre-surgery therapy.
  • No radiation therapy was used for patients at stage I and FH stage II.
  • Pathologic analysis showed fourteen cases were at their FH, three at unfavorable-histology (UFH), two at clear cell and one at rhabdoid stage.
  • Five patients were at stage I, five at stage II, six at stage III, three at stage IV and one at stage V.
  • One relapsed after 24 months of CCR and reached the second CR after intensive chemotherapy.
  • No therapy-related death happened.
  • CONCLUSION: Multi-disciplinary team work model and protocol WT-99 are safe and effective for Wilms' tumor.
  • [MeSH-major] Kidney Neoplasms / therapy. Wilms Tumor / therapy
  • [MeSH-minor] Academic Medical Centers. Bone Transplantation. Child. Child, Preschool. China. Combined Modality Therapy. Female. Humans. Infant. Male. Neoplasm Staging. Transplantation, Autologous. Treatment Outcome


14. 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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15. Lugtenberg RT, Cransberg K, Loos WJ, Wagner A, Alders M, van den Heuvel-Eibrink MM: Topotecan distribution in an anephric infant with therapy-resistant bilateral Wilms tumor with a novel germline WT1 gene mutation. Cancer Chemother Pharmacol; 2008 Nov;62(6):1039-44
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  • [Title] Topotecan distribution in an anephric infant with therapy-resistant bilateral Wilms tumor with a novel germline WT1 gene mutation.
  • The therapeutic strategy for bilateral Wilms tumor (WT) remains a challenge.
  • Especially in cases with chemotherapy resistant disease, bilateral nephrectomy is sometimes inevitable.
  • For optimal cure rates stage V WT patients benefit from adjuvant treatment; however, there are limited data available on chemotherapy pharmacokinetics in anephric children.
  • In this report, we describe a 10-month old girl with bilateral Wilms tumor and a novel germline WT1 gene mutation.
  • This patient hardly showed any response on preoperative chemotherapy, and ultimately, underwent sequential bilateral tumor-nephrectomy.
  • Subsequently, during peritoneal dialysis, she received topotecan as adjuvant chemotherapy based on plasma levels, indicating that this is a reasonable option as adjuvant treatment in therapy-resistant Wilms tumor patients after bilateral nephrectomy.
  • [MeSH-major] Antineoplastic Agents / pharmacokinetics. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Drug Resistance, Neoplasm / genetics. Genes, Wilms Tumor. Germ-Line Mutation. Kidney Neoplasms / metabolism. RNA Splice Sites / genetics. Topotecan / pharmacokinetics. Wilms Tumor / metabolism
  • [MeSH-minor] Chemotherapy, Adjuvant. Combined Modality Therapy. DNA, Neoplasm / genetics. Dactinomycin / administration & dosage. Dactinomycin / pharmacology. Doxorubicin / administration & dosage. Doxorubicin / pharmacology. Exons / genetics. Female. Frameshift Mutation. Humans. Infant. Loss of Heterozygosity. Metabolic Clearance Rate. Nephrectomy. Peritoneal Dialysis. Polymorphism, Single Nucleotide. Sequence Analysis, DNA. Vincristine / administration & dosage. Vincristine / pharmacology

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  • (PMID = 18273617.001).
  • [ISSN] 1432-0843
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / DNA, Neoplasm; 0 / RNA Splice Sites; 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 7M7YKX2N15 / Topotecan; 80168379AG / Doxorubicin; VAD regimen 2
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16. Sidhom I, Hussien H, Kotb M, Anwer G, Aboul Naga S, Amin M, Ebied E, Ahmed H: Multidisciplinary Approach to Wilms' Tumor: 10 Years Experience of NCI, Egypt. J Clin Oncol; 2004 Jul 15;22(14_suppl):8544

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  • [Title] Multidisciplinary Approach to Wilms' Tumor: 10 Years Experience of NCI, Egypt.
  • : 8544 Background: This is a retrospective study of 140 nephroblastoma patients newly diagnosed and treated at the National Cancer Institute between January 1991 and December 2000.
  • The aim was to report results of combined modality treatment and to investigate the impact of biopsy, presence of blastema and preoperative chemotherapy on outcome Methods: Chemotherapy consisted of Vincristine 1.5mg/m<sup>2</sup> and Actinomycin-D 45μg/kg for stage I and stage II favorable.
  • Doxorubicin 40mg/m<sup>2</sup> was added in alternation with Actinomycin-D to stage II unfavorable and stages III-V.
  • Duration of therapy was 6 months for stage I and stage II favorable, 12 months for stages II unfavorable, III-V favorable and 18 months for stages III-V unfavorable.
  • Postoperative radiotherapy was given to tumor-bed (10-27Gy) in stage II unfavorable, stages III-V and to metastatic lung site (12Gy).
  • Preoperative chemotherapy was employed in massive or bilateral tumors or presence of metastases Results: Using NWTS staging system 21.4%, 36.4%, 20.7%, 15.7% and 5.7% were stages I-V respectively.
  • Preoperative chemotherapy resulted in down staging of 30/54 (55.6%) patients to stages I and II, of which only 4 relapsed.
  • For FH, 5-year DFS was 78.2±4.3% [stages I-V being 92%, 74.3%, 74%, 60%, 80%] and 51.4±7.9% for UH (p= .0007, log rank test) [stages I-V being 75%, 72.7%, 63.6%, 8.3%, 33.3%] Within FH, 5-year DFS was 76.6% for blastema versus 83.3% for non blastema (p= .46, log rank) Conclusions: This study suggests that biopsy and down staging after preoperative chemotherapy did not lead to increased risk of recurrence.

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  • (PMID = 28013808.001).
  • [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. 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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18. 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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19. Basta-Jovanović G, Radonjic V, Stolic I, Nenadovic M, Brasanac D, Jovanovic D, Radojevic-Skodric S: Significance of proto-oncogene Bcl-X(S/L) expression in Wilms tumor. Ren Fail; 2005;27(1):13-8
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  • [Title] Significance of proto-oncogene Bcl-X(S/L) expression in Wilms tumor.
  • The rate of apoptosis varies in malignant tumors, and it can be involved in diminishing tumor size.
  • In human renal diseases, such as the experimental model of acute renal failure, and many tumors, including Wilms' tumor, the expression of antiapoptotic members of Bcl-2 family is increased, while the expression of proapoptotic members is low.
  • AIM: The aim of our study was to investigate Bcl-X(S/L) protein expression in Wilms' tumor, to compare it with the expression in normal renal tissue, as well as to see if there is a correlation between Bcl-X(S/L) expression in Wilms' tumor with tumor stage, histological type, prognostic group, or response to preoperative chemotherapy.
  • MATERIALS AND METHODS: Twenty-eight cases of Wilms' tumor (two cases with metastasis) and two samples of normal kidney tissue were studied using streptavidin-biotin-complex technique.
  • RESULTS: The expression of Bcl-X(S/L) was observed in the majority of cases (60.7%), more often in the blastemal than in the epithelial component of Wilms' tumor: 60.7% and 28.6%, respectively (p=0.02).
  • There was a statistically significant inverse relationship between Bcl-X(S/L) expression and tumor stage (p=0.015).
  • Treated Wilms' tumors showed Bcl-X(S/L) expression more often than nontreated tumors, but the relationship was not statistically significant (p=0.08).
  • Expression of Bcl-X(S/L) was detected in various histological types of Wilms' tumor, but there was no statistically significant association (p=0.82) except in cases with diffuse anaplasia (p=0.012), which were always negative.
  • No Bcl-X(S/L) immunostaining was observed in two cases of metastasis or in one case of bilateral Wilms' tumor.
  • CONCLUSION: Our results suggest that the expression of Bcl-X(S/L) protein is associated with prognostic group, tumor stage, and presence of anaplasia.
  • [MeSH-major] Kidney / pathology. Proto-Oncogene Proteins c-bcl-2 / biosynthesis. Wilms Tumor / metabolism
  • [MeSH-minor] Anaplasia. Antineoplastic Agents / therapeutic use. Apoptosis. Child. Child, Preschool. Female. Humans. Infant. Male. Neoadjuvant Therapy. Neoplasm Staging. Prognosis. bcl-X Protein

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  • (PMID = 15717629.001).
  • [ISSN] 0886-022X
  • [Journal-full-title] Renal failure
  • [ISO-abbreviation] Ren Fail
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / BCL2L1 protein, human; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / bcl-X Protein
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20. Hung IJ, Chang WH, Yang CP, Jaing TH, Liang DC, Lin KH, Lin DT, Hsiao CC, Hsieh YL, Chen JS, Chang TT, Peng CT, Shu SG, Lin MT, Chen BW, Lin KS, Taiwan Pediatric Oncology Group: Epidemiology, clinical features and treatment outcome of Wilms' tumor in Taiwan: a report from Taiwan Pediatric Oncology Group. J Formos Med Assoc; 2004 Feb;103(2):104-11
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  • [Title] Epidemiology, clinical features and treatment outcome of Wilms' tumor in Taiwan: a report from Taiwan Pediatric Oncology Group.
  • BACKGROUND AND PURPOSE: Taiwan Pediatric Oncology Group (TPOG)-W-91 is the first multi-institutional Wilms' tumor study for children in Taiwan.
  • This clinical trial used a multidisciplinary approach, based on and similar to the National Wilms' Tumor Study 4.
  • The study was conducted to evaluate the epidemiological characteristics and analyze the outcome of Wilms' tumor patients treated with this protocol.
  • METHODS: Ninety eight children with Wilms' tumor (WT) were analyzed for distributions of age, gender, associated congenital anomalies, tumor sites, histology, tumor weights, and clinical stages.
  • Patients received individualized multimodality treatment based upon the histology of the tumor and clinicopathologic stage.
  • The treatment included surgery, radiotherapy and 2-, 3-, and 4-agent active chemotherapeutic agents.
  • Seventy patients were eligible for analysis of treatment outcome.
  • Patients were divided into various subgroups according to the chemotherapy regimen used, tumor stage, age at diagnosis, gender, and tumor weight.
  • The stage distribution was: I, 43.2%; II, 19.3%; III, 23.9%; IV, 6.8%; and V, 6.8%.
  • The median follow-up time was 89.1 months (range, 1.8 to 128.1 months).
  • The 5-year PFS rate was 0.7841 (SE, 0.0494; 53 of 70 patients) and the 5-year OS rate was 0.886 (SE, 0.038; 63 of 70 patients).
  • CONCLUSIONS: This study evaluated the epidemiological characteristics, clinical features, multimodality therapy regimens, and treatment outcome of WT in Taiwan.
  • Data obtained from this study may lead to further improvement in the prognosis of pediatric malignant solid tumor.

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  • (PMID = 15083240.001).
  • [ISSN] 0929-6646
  • [Journal-full-title] Journal of the Formosan Medical Association = Taiwan yi zhi
  • [ISO-abbreviation] J. Formos. Med. Assoc.
  • [Language] ENG
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Singapore
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21. Giordano G, Campanini N, Rocco A, Donofrio V, Bertolini P, Falleti J, Pettinato G: C-kit protein expression in Wilms' tumour: an immunohistochemical study. Eur J Surg Oncol; 2009 Jun;35(6):629-35
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  • [Title] C-kit protein expression in Wilms' tumour: an immunohistochemical study.
  • AIM: The use of a non-toxic tyrosine kinase receptor inhibitor, Imatinib Mesylate (IM), has become an ever-more common therapeutic alternative in some Kit (CD117) over-expressing neoplasms.
  • As the treatment eligibility for these drugs hinges on CD117 expression, Kit immunostaining has recently been widely examined in various tumours.
  • There are only limited data in the literature on the expression of c-kit expression in Wilms' Tumour.
  • We examined CD117 expression in Wilms' tumour in order to correlate this marker with clinico-pathological data and to clarify its prognostic impact.
  • METHODS: This study included 40 cases of Wilms' tumour.
  • On univariate analysis, significantly higher c-kit expression was observed in neoplasms in a more advanced stage of development than those in a less advanced stage (p=0.0055).
  • Moreover, all our Wilms' tumour anaplastic subtypes showed over-expression of c-kit and this was significantly higher than in favourable histology examples (p=0.0182).
  • However, it seems likely that c-kit expression could be a secondary event related to tumour progression and could be influenced by chemotherapy and unfavourable histology.
  • [MeSH-major] Biomarkers, Tumor / biosynthesis. Kidney Neoplasms / metabolism. Proto-Oncogene Proteins c-kit / biosynthesis. Wilms Tumor / metabolism

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  • (PMID = 19010635.001).
  • [ISSN] 1532-2157
  • [Journal-full-title] European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
  • [ISO-abbreviation] Eur J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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22. Granzen B, Efferth T, Keller U, Beniers AJ, Mertens R, Jakse G, Füzesi L: Differential expression of the drug resistance markers DNA topoisomerase II alpha and glutathione S-transferase-pi in the histological compartments of Wilms' tumors. Anticancer Res; 2001 Jan-Feb;21(1B):771-6
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  • [Title] Differential expression of the drug resistance markers DNA topoisomerase II alpha and glutathione S-transferase-pi in the histological compartments of Wilms' tumors.
  • More than 80% of the patients presenting with Wilms' tumor can be cured today.
  • Some patients, however, fail to respond to chemotherapy.
  • The objective of this study was to analyze the immunohistochemical distribution of two markers of cytostatic drug resistance, e.g.
  • DNA topoisomerase II alpha (Topo II alpha) and glutathione S-transferase-pi (GST-pi) in 23 Wilms' tumor patients who had undergone an operation between 1984 and 1997.
  • Eight patients had stage I disease, seven stage II, three stage III, four stage IV, and one stage V disease.
  • Investigations were carried out on formalin-fixed and paraffin-embedded tissue sections using the indirect immunoperoxidase method.
  • Topo II alpha was, however, less expressed in the blastemal and stromal elements of specimens after preoperative treatment.
  • While no expression of GST-pi was found in preoperatively untreated Wilms' tumors, it was present in epithelial compartments in 57% of tumors after chemotherapy.
  • In conclusion, preoperative chemotherapy led to compartment-specific alterations in the expression levels of both markers indicating a contribution to treatment response of Wilms' tumors.
  • [MeSH-major] DNA Topoisomerases, Type II / analysis. Drug Resistance, Neoplasm. Glutathione Transferase / analysis. Isoenzymes / analysis. Kidney Neoplasms / enzymology. Neoplasm Proteins / analysis. Wilms Tumor / enzymology
  • [MeSH-minor] Antigens, Neoplasm. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. DNA-Binding Proteins. Epithelial Cells / enzymology. Female. Follow-Up Studies. Humans. Infant. Male. Neoplasm Staging. Neoplastic Stem Cells / enzymology. Nephrectomy. Premedication. Stromal Cells / enzymology

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  • [ErratumIn] Anticancer Res 2001 Jul-Aug;21(4A):2861
  • (PMID = 11299842.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / DNA-Binding Proteins; 0 / Isoenzymes; 0 / Neoplasm Proteins; EC 2.5.1.18 / Glutathione Transferase; EC 5.99.1.3 / DNA Topoisomerases, Type II; EC 5.99.1.3 / DNA topoisomerase II alpha
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23. 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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24. Suita S, Kinoshita Y, Tajiri T, Hara T, Tsuneyoshi M, Mizote H, Inada H, Takamatsu H, Kawano Y, Inomata Y, Nagasaki A, Ono Y, Handa N, Okamura J, Ishii E, Kawakami K, Committee for pediatric solid malignant tumors in the Kyushu area: Clinical characteristics and outcome of Wilms tumors with a favorable histology in Japan: a report from the Study Group for Pediatric Solid Malignant Tumors in the Kyushu Area, Japan. J Pediatr Surg; 2006 Sep;41(9):1501-5
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  • [Title] Clinical characteristics and outcome of Wilms tumors with a favorable histology in Japan: a report from the Study Group for Pediatric Solid Malignant Tumors in the Kyushu Area, Japan.
  • BACKGROUND/PURPOSE: Since 1996, the standard treatment of Wilms tumors in Japan has been based on the regimen of the Japanese Wilms Tumor Study.
  • However, in Japan, there have been no reports about Wilms tumors that analyzed the clinical features and patient outcome in a large series until now.
  • This study aims to assess the clinical characteristics of patients with Wilms tumor with a favorable histology from a retrospective standpoint in the Kyushu area in Japan and, furthermore, to analyze the historical changes of clinical features and outcome from the 1980s to the 1990s.
  • METHODS: Between 1982 and 1996, 90 cases of Wilms tumors with a favorable histology were registered in the Kyushu area.
  • RESULTS: The clinical features (age, sex, initial symptom, location, stage) demonstrated no definite differences between group A and group B.
  • All stage V cases in group B undewent a bilateral tumor biopsy instead of a radical nephrectomy as the initial operation.
  • Of particular note, the outcome of patients with stage I and stage V in group B substantially improved in comparison to that in group A.
  • CONCLUSIONS: This is the first report about the clinical features and outcome for Wilms tumors with a favorable histology in Japan from the 1980s to the 1990s.
  • The present study suggested that in the early-stage cases, an initially complete resection followed by standard postoperative chemotherapy substantially improved the outcome of the patients in group B.
  • In the stage V cases, the performance of renal salvage surgery may have positively contributed to the improvement in the outcome in group B.
  • However, in the advanced stage cases, no definite improvement was noted.
  • In the future, an improved efficacy of the treatments for Wilms tumors based on the standard protocol established by the Japanese Wilms Tumor Study in 1996 is expected in Japan.
  • [MeSH-major] Kidney Neoplasms / mortality. Wilms Tumor / mortality
  • [MeSH-minor] Child, Preschool. Female. Humans. Infant. Infant, Newborn. Japan. Male. Neoplasm Staging. Nephrectomy. Retrospective Studies. Survival Analysis. Treatment Outcome

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  • (PMID = 16952581.001).
  • [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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25. Bergeron C, Iliescu C, Thiesse P, Bouvier R, Dijoud F, Ranchere-Vince D, Basset T, Chappuis JP, Buclon M, Frappaz D, Brunat-Mentigny M, Philip T: Does nephroblastomatosis influence the natural history and relapse rate in Wilms' tumour? A single centre experience over 11 years. Eur J Cancer; 2001 Feb;37(3):385-91
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  • [Title] Does nephroblastomatosis influence the natural history and relapse rate in Wilms' tumour? A single centre experience over 11 years.
  • Nbm may be a predisposing factor for Wilms' tumour (WT).
  • We assessed the outcome of 81 children with Wilms tumours and practical implications of Nbm in the treatment and follow-up.
  • There was no statistical difference between the percentage of stage IV in both groups, but bilaterality (stage V) was present only in the group B.
  • The discovery of Nbm in the non-tumoral part of the kidney with WT can be an adverse factor and in particular favours the subsequent development of a new Wilms tumour.
  • [MeSH-major] Kidney Neoplasms / etiology. Wilms Tumor / etiology
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Infant. Male. Neoplasm Recurrence, Local / drug therapy. Neoplasm Recurrence, Local / etiology. Neoplasm Recurrence, Local / pathology. Retrospective Studies. Survival Analysis. Treatment Outcome

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  • (PMID = 11239761.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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26. 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.
  • 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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27. Vezzadini C, Cremonini N, Sforza A, Presutti L, Chiarini V: Treated Wilm's tumor in childhood as potential risk factor for second thyroid cancer. Panminerva Med; 2002 Sep;44(3):275-7
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  • [Title] Treated Wilm's tumor in childhood as potential risk factor for second thyroid cancer.
  • The potential risk of a treatment-induced second neoplasia affecting the thyroid is well known after radiation therapy for several types of cancer, but few cases have been related to incidental irradiation for Wilms' tumor.
  • We report a case of a papillary thyroid carcinoma discovered in a young patient 15 years after treatment of a Wilms' tumor.
  • His past medical history included at the age of 2 years surgical resection, chemotherapy (actinomycin-D and vincristine) and cesium radiation therapy to the right side for a Wilms' tumor in stage III: a total dose of 7700 rads was delivered to an area of 17 x 10 cm in the right flank.
  • Residual thyroid tissue was ablated by iodine-131 administration (3700 MBq), but scanning after therapeutic iodine showed radioactive uptake in the left regional lymph nodes, with elevated serum thyroglobulin off therapy (830 ng/ml).
  • Magnetic resonance imaging confirmed the presence of lymph node enlargements and bilateral neck dissection was performed, followed by radioiodine treatment (3700 MBq) and thyroxine suppressive therapy.
  • Although few cases of thyroid cancer have been reported in the literature after irradiation for a Wilms' tumor during childhood, this association should be considered in the long-term follow-up.
  • [MeSH-major] Carcinoma, Papillary / etiology. Kidney Neoplasms / radiotherapy. Neoplasms, Radiation-Induced. Neoplasms, Second Primary / etiology. Thyroid Neoplasms / etiology. Wilms Tumor / radiotherapy
  • [MeSH-minor] Adolescent. Combined Modality Therapy. Humans. Male. Risk Factors


28. 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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29. 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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30. 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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31. 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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32. 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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  • 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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33. Takamizawa S, Scott D, Wen J, Grundy P, Bishop W, Kimura K, Sandler A: The survivin:fas ratio in pediatric renal tumors. J Pediatr Surg; 2001 Jan;36(1):37-42
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The survivin:fas ratio in pediatric renal tumors.
  • Fas is a pro-apoptotic receptor that induces cell death when bound by its ligand and is expressed at greater levels in pediatric renal tumors of good prognosis.
  • This study evaluates the expression of survivin, as well as the prognostic value of the survivin:fas ratio in various types and stages of pediatric renal tumors.
  • METHODS: Multiple apoptosis mRNA species were quantified by Rnase protection assay (RPA) in 32 pediatric renal tumors and adjacent normal kidney specimens before chemotherapy: Wilms' tumor (WT), n = 9; clear cell sarcoma (CCS), n = 4; rhabdoid tumor of the kidney (RTK), n = 5; mesoblastic nephroma (MN), n = 3 and normal kidney, n = 11.
  • RESULTS: Pediatric renal tumors express greater levels of both pro- and antiapoptotic factors than normal kidney.
  • Survivin and fas appeared to be expressed differentially in the tumor specimens sampled.
  • The mean survivin:fas ratio was significantly greater in the 10 tumors that went on to recur after treatment (4 RTK, 3 CCS, 3 WT), than in tumors not recurring (2.16+/-1.4 v 1.0+/-1.07; P =.01, Kruskal-Wallis test).
  • The positive predictive value of tumor recurrence was 85.7% (CI: 42.1%, 99.6%) and the negative predictive value was 71.4% (CI: 41.9%, 91.6%) when a cutoff ratio of 1.6 was considered.
  • CONCLUSIONS: The survivin:fas mRNA ratio is of prognostic value in its ability to predict recurrent disease in children undergoing treatment for pediatric renal tumors.
  • In this series, a ratio of greater than 1.6 predicted recurrent disease with a high probability irrespective of clinical stage or pathologic type.
  • Determining the survivin:fas ratio may guide treatment, follow-up and counseling of patients with pediatric renal tumors.

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  • (PMID = 11150435.001).
  • [ISSN] 0022-3468
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Antigens, CD95; 0 / BIRC5 protein, human; 0 / Inhibitor of Apoptosis Proteins; 0 / Microtubule-Associated Proteins; 0 / Neoplasm Proteins; 0 / Proteins; 0 / RNA, Messenger
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34. 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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