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1. 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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2. 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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3. Menéndez JA, Mehmi I, Griggs DW, Lupu R: The angiogenic factor CYR61 in breast cancer: molecular pathology and therapeutic perspectives. Endocr Relat Cancer; 2003 Jun;10(2):141-52
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  • [Title] The angiogenic factor CYR61 in breast cancer: molecular pathology and therapeutic perspectives.
  • CYR61 (CNN1), a member of the cysteine rich 61/connective tissue growth factor/nephroblastoma overexpressed (CYR61/CTFG/NOV) family of growth regulators (CNN), is a pro-angiogenic factor that mediates diverse roles in development, cell proliferation, and tumorigenesis.
  • We have recently shown that CYR61 is overexpressed in invasive and metastatic human breast cancer cells.
  • Moreover, we describe experimental evidence that establishes a novel role for CYR61 determining the protection of human breast cancer cells against chemotherapy-induced apoptosis through its interactions with the integrin receptor alpha(v)beta(3).
  • All these findings delineate a new noteworthy function of a CYR61/alpha(v)beta(3) autocrine-paracrine signaling pathway within both angiogenesis and breast cancer progression, which would allow a dual anti-angiogenic and anti-tumor benefit with a single drug.

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  • (PMID = 12790776.001).
  • [ISSN] 1351-0088
  • [Journal-full-title] Endocrine-related cancer
  • [ISO-abbreviation] Endocr. Relat. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Angiogenesis Inducing Agents; 0 / CYR61 protein, human; 0 / Cysteine-Rich Protein 61; 0 / Immediate-Early Proteins; 0 / Intercellular Signaling Peptides and Proteins
  • [Number-of-references] 77
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4. 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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5. 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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6. Pollono D, Drut R, Tomarchio S, Fontana A, Ibañez O: Fetal rhabdomyomatous nephroblastoma: report of 14 cases confirming chemotherapy resistance. J Pediatr Hematol Oncol; 2003 Aug;25(8):640-3
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  • [Title] Fetal rhabdomyomatous nephroblastoma: report of 14 cases confirming chemotherapy resistance.
  • The authors retrospectively reviewed their experience with 14 cases of fetal rhabdomyomatous nephroblastoma (15.6% of all renal tumors seen in the 1984-1998 period), analyzing the diagnostic and treatment approach as well outcome.
  • Nine patients received preoperative chemotherapy after fine-needle aspiration biopsy.
  • The response was null in five children and mild in three; in the remaining one the tumor increased in size.
  • The only patient showing greater than 50% tumor shrinkage was the one treated with radiotherapy.
  • Postoperative treatment according to staging followed the SIOP protocol.
  • Patients with fetal rhabdomyomatous nephroblastoma presenting with distant metastatic disease and high stage have unfavorable outcomes, confirming data from the literature indicating that chemotherapy is not as effective as in other variants of nephroblastoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Rhabdomyoma / drug therapy. Rhabdomyoma / pathology. Wilms Tumor / drug therapy. Wilms Tumor / pathology
  • [MeSH-minor] Age of Onset. Child, Preschool. Drug Resistance, Neoplasm. Female. Humans. Infant. Male. Prognosis. Retrospective Studies. Treatment Outcome

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  • (PMID = 12902919.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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7. 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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8. 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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9. Reinhard H, Schmidt A, Furtwängler R, Leuschner I, Rübe C, Von Schweinitz D, Zoubek A, Niggli F, Graf N: Outcome of relapses of nephroblastoma in patients registered in the SIOP/GPOH trials and studies. Oncol Rep; 2008 Aug;20(2):463-7
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  • [Title] Outcome of relapses of nephroblastoma in patients registered in the SIOP/GPOH trials and studies.
  • We registered 170 relapses in 1392 children with nephroblastoma in the SIOP/GPOH trials.
  • Age, gender, initial stage, metastatic disease, local stage, histology, time to relapse and tumour volume were analysed for their prognostic relevance.
  • Relapses were local in 28%, metastatic in 57% and combined in 15%.
  • Patients with initial stage I and II showed a significantly better prognosis than children with stage III (57 vs. 31%, p=0.008).
  • The tumour volume initially and after preoperative chemotherapy did not have any influence on outcome.
  • In conclusion, factors for poor prognosis after relapse are early relapse, local stage III, high-risk histology and combined relapse.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Neoplasm Recurrence, Local / diagnosis. Wilms Tumor / drug therapy
  • [MeSH-minor] Child. Child, Preschool. Female. Humans. Male. Neoplasm Staging. Prognosis. Remission Induction. Retrospective Studies. Risk Factors. Treatment Outcome

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  • (PMID = 18636212.001).
  • [ISSN] 1021-335X
  • [Journal-full-title] Oncology reports
  • [ISO-abbreviation] Oncol. Rep.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
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10. Tahri A, Benchekroun N, Karkouri M, Dahami Z, Sahraoui S, Acharki A, Benider A, Squalli S, Benjelloun S, Kahlain A: [Nephroblastoma in adults. Three case reports]. Ann Urol (Paris); 2001 Sep;35(5):257-61
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  • [Title] [Nephroblastoma in adults. Three case reports].
  • The authors report three nephroblastoma's cases occurred in adult and treated at Ibn Rochd Oncology Center at Casablanca.
  • At diagnosis, radiological exams revealed that patients presented an advanced stage and metastatic disease in one case.
  • The treatment was a total nephrectomy with ganglioma dissection when the tumor was resectable followed by chemotherapy and radiotherapy.
  • Two patients was in progressive disease despite treatment, and one patient was lost at follow-up.
  • The prognosis of adult's nephroblastoma remains very severe, due to the advanced stage at diagnosis and the mediocre reply to the treatment.
  • [MeSH-major] Kidney Neoplasms / pathology. Nephrectomy. Wilms Tumor / pathology
  • [MeSH-minor] Adult. Age of Onset. Chemotherapy, Adjuvant. Diagnosis, Differential. Disease Progression. Female. Humans. Male. Neoplasm Staging. Prognosis. Radiotherapy, Adjuvant

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  • (PMID = 11675960.001).
  • [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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11. Myers JB, Dall'Era J, Odom LF, McGavran L, Lovell MA, Furness P 3rd: Teratoid Wilms' tumor, an important variant of nephroblastoma. J Pediatr Urol; 2007 Aug;3(4):282-6

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  • [Title] Teratoid Wilms' tumor, an important variant of nephroblastoma.
  • PURPOSE: The teratoid histologic variant of Wilms' tumor is rare, with only 15 prior reported cases.
  • We review these and report an additional case in which a cytogenetic abnormality was identified that has not previously been reported in a teratoid Wilms' tumor.
  • MATERIALS AND METHODS: A medline search revealed 15 previously reported cases of the teratoid variant of Wilms' tumor.
  • We summarized the characteristics of these cases with attention to radiologic appearance, stage, laterality, histology, response to chemotherapy and outcomes.
  • RESULTS: Characteristic radiologic features suggesting teratoid Wilms' tumor were calcific densities and stippling, or areas of attenuation indicating adipose tissue.
  • The majority of teratoid Wilms' tumor patients had a high tumor stage at presentation (50% stage III or greater).
  • Chemotherapy was administered in nine cases and in only one (11%) was there a cytoreductive response.
  • CONCLUSIONS: Teratoid Wilms' tumors appear to present with a high stage, increased incidence of bilaterality and have a high mortality rate.
  • Treatment strategies should focus on total surgical extirpation, including metastatic sites when feasible, due to this entity's limited response to chemotherapy.

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  • (PMID = 18947756.001).
  • [ISSN] 1873-4898
  • [Journal-full-title] Journal of pediatric urology
  • [ISO-abbreviation] J Pediatr Urol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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12. 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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13. 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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14. Ekenze SO, Agugua-Obianyo NE, Odetunde OA: The challenge of nephroblastoma in a developing country. Ann Oncol; 2006 Oct;17(10):1598-600
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  • [Title] The challenge of nephroblastoma in a developing country.
  • BACKGROUND: Advances in paediatric oncology has tremendously improved the outcome in children with nephroblastoma.
  • OBJECTIVE: To study the outcome and the impediments to the management of nephroblastoma in Southeast Nigeria.
  • METHODS: Analysis of 42 children managed for nephroblastoma over a 10-year period (January 1995-December 2004) at the University of Nigeria Teaching Hospital, Enugu, Nigeria is made.
  • Treatment consisted of nephroureterectomy followed by adjuvant chemotherapy with Vincristine, Actinomycin D and Cyclophosphamide.
  • Adriamycin was added for metastatic disease.
  • Twenty-two children (52.3%) had stage III disease, 13 (31.0%) had stage IV, while the remaining seven (16.7%) children had stage II.
  • Stage I disease was not encountered.
  • Seven children had inoperable tumor requiring pre-operative chemotherapy.
  • Four children died of complications of treatment, while 11 relapsed with poor outcome.
  • CONCLUSION: Morbidity and mortality with nephroblastoma is high in our environment.
  • Late presentation, poverty, ignorance and poor compliance to treatment constitute a great challenge to the paediatric oncologist in a developing country.

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  • (PMID = 16873431.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] England
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15. Szavay P, Luithle T, Graf N, Furtwängler R, Fuchs J: Primary hepatic metastases in nephroblastoma--a report of the SIOP/GPOH Study. J Pediatr Surg; 2006 Jan;41(1):168-72; discussion 168-72
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  • [Title] Primary hepatic metastases in nephroblastoma--a report of the SIOP/GPOH Study.
  • PURPOSE: Remarkable progress could be achieved in the treatment of nephroblastoma within the last decades.
  • In all children with Wilms' tumor, 5-year overall survival rate reaches more than 90% in the SIOP/GPOH Study Group.
  • Despite this fact, there is a small group of patients who have tumor lesions in the liver primarily representing a challenge in treatment.
  • METHODS: To define survival and success of treatment in this group of patients, we reviewed the records of 29 of 1365 patients enrolled in the SIOP 93-01/GPOH Study and the SIOP 2001/GPOH Study between April 1, 1994, and September 30, 2004.
  • Liver metastases were operated in 11 children at time of nephrectomy, in 4 patients secondarily, whereas in 11 patients, liver lesions were treated alone with chemotherapy and radiotherapy, respectively.
  • In 3 children, no treatment could be initiated.
  • These included 7 patients who never had surgery for their liver lesions and 4 patients who had incomplete and/or atypical resections of their metastatic liver lesions.
  • Eight children survived with a nonsurgical treatment.
  • Our report suggests that Wilms' tumor complicated by metastases of the liver primarily has a less favorable outcome.
  • Chemotherapy and radiotherapy play an important role in treatment.
  • [MeSH-major] Kidney Neoplasms / pathology. Liver Neoplasms / secondary. Nephrectomy. Wilms Tumor / secondary
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Prognosis. Survival Analysis

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  • (PMID = 16410128.001).
  • [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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16. Emerson RE, Ulbright TM, Zhang S, Foster RS, Eble JN, Cheng L: Nephroblastoma arising in a germ cell tumor of testicular origin. Am J Surg Pathol; 2004 May;28(5):687-92
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  • [Title] Nephroblastoma arising in a germ cell tumor of testicular origin.
  • We report a nephroblastoma arising in a germ cell tumor of testicular origin occurring in a 22-year-old man.
  • Orchiectomy demonstrated a malignant mixed germ cell tumor composed of mature and immature teratoma with nephroblastoma and rhabdomyosarcoma.
  • Following chemotherapy, the patient developed supraclavicular and retroperitoneal lymphadenopathy.
  • Excision demonstrated metastatic teratoma at both sites.
  • Using tissue microdissection and loss of heterozygosity analysis, we investigated the clonality of the mature teratoma, immature teratoma, nephroblastoma, and rhabdomyosarcoma components of the primary tumor and of the metastatic mature teratoma at the two separate distant sites.
  • Nine microsatellite polymorphic makers were used to examine the pattern of allelic loss in both primary and metastatic tumors.
  • Loss of heterozygosity was found in 4 DNA loci, and the same pattern of allelic loss was demonstrated at all 4 loci in all of the different components of the primary tumor and the metastatic mature teratomas, supporting the germ cell tumor origin of the nephroblastoma component.
  • Loss of heterozygosity on chromosome 17p13 (TP53) was detected in metastatic mature teratoma, but not in the primary tumor.
  • Loss of heterozygosity was observed at 11p13, the locus of WT1 inactivation in patients genetically predisposed to nephroblastoma, and this loss may be an important genetic mechanism in nephroblastomatous differentiation of germ cell tumors.
  • These data support a common clonal origin for nephroblastoma and the other germ cell tumor components.
  • [MeSH-major] Germinoma / pathology. Neoplasms, Second Primary / pathology. Testicular Neoplasms / pathology. Wilms Tumor / pathology

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  • (PMID = 15105660.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm
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17. 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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18. Varan A, Büyükpamukçu N, Cağlar M, Köksal Y, Yalçn B, Akyüz C, Kutluk T, Büyükpamukçu M: Prognostic significance of metastatic site at diagnosis in Wilms' tumor: results from a single center. J Pediatr Hematol Oncol; 2005 Apr;27(4):188-91
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  • [Title] Prognostic significance of metastatic site at diagnosis in Wilms' tumor: results from a single center.
  • The purpose of this study was to identify the prognostic significance of pulmonary and liver involvements present at diagnosis in children with Wilms' tumor.
  • From 1971 to 2002, 57 patients with stage IV Wilms' tumor were diagnosed, treated, and followed in the authors' institution.
  • Metastases were detected by chest radiography, abdominal ultrasonography, and/or thoracoabdominal computed tomography.
  • Nephrectomy with tumor resection was performed and appropriate chemotherapy was given to all the patients.
  • Wilms' tumor patients with liver metastases should be treated with more intensive regimens than those with lung metastases.
  • [MeSH-major] Kidney Neoplasms / pathology. Liver Neoplasms / secondary. Lung Neoplasms / secondary. Wilms Tumor / secondary
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Infant. Male. Prognosis. Radiography, Thoracic. Survival Rate. Tomography, X-Ray Computed


19. Godziński J, Weirich A, Tournade MF, Gauthier F, Buerger D, Moorman-Voestermans CG, de Kraker J, Voute P, Ludwig R, Sawicz-Birkowska K, Vujanić G, Ducourtieux M: Primary nephrectomy for emergency: a rare event in the International Society of Paediatric Oncology Nephroblastoma Trial and Study no. 9. Eur J Pediatr Surg; 2001 Feb;11(1):36-9
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  • [Title] Primary nephrectomy for emergency: a rare event in the International Society of Paediatric Oncology Nephroblastoma Trial and Study no. 9.
  • Experience of the International Society of Paediatric Oncology (SIOP) Trials and Studies indicates that the preoperative chemotherapy in Wilms' tumour improves stage distribution, decreases complication rate and reduces postoperative treatment.
  • Records of 720 patients with non-metastatic unilateral nephroblastoma who were registered in the SIOP Trial and Study 9 were reviewed.
  • Postoperative treatment included radiotherapy in 71% of cases and anthracyclines in 92%.
  • [MeSH-major] Kidney Neoplasms / surgery. Nephrectomy. Wilms Tumor / surgery

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  • (PMID = 11370981.001).
  • [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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20. Toranzo-Fernandez JM, Falcon-Escobedo R, Sanchez-Hermosillo E, Gonzalez-Mendoza E: Clear cell sarcoma of the kidney metastatic to jaw: case report. J Clin Pediatr Dent; 2000;24(2):137-9
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  • [Title] Clear cell sarcoma of the kidney metastatic to jaw: case report.
  • A case of an 8 year-old boy with history of nephroblastoma (Wilms' tumor) three years prior, which was managed with nephrectomy, chemotherapy and radiotherapy, is presented.
  • Re-evaluation of the previous renal tumor was interpreted as a clear cell sarcoma of the kidney.
  • Biopsy of the lesion of the left jaw showed similar histology, therefore, the diagnosis of clear cell sarcoma of the kidney metastatic to the left jaw was rendered.
  • We emphasize the diagnostic challenge of both the primary neoplasm and the metastasis and discuss the differential diagnosis of primary and metastatic sarcomas.

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  • (PMID = 11314323.001).
  • [ISSN] 1053-4628
  • [Journal-full-title] The Journal of clinical pediatric dentistry
  • [ISO-abbreviation] J Clin Pediatr Dent
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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21. Lee TP, Tzao C, Liu JH, Yu CP, Hsu HH, Chien HN: Isolated endobronchial metastasis of Wilms' tumor. J Pediatr Surg; 2005 Sep;40(9):e33-5
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  • [Title] Isolated endobronchial metastasis of Wilms' tumor.
  • We report an isolated endobronchial metastasis of Wilms' tumor in a 4-year-old boy.
  • He underwent right nephrectomy on account of Wilms' tumor at the age of 3 years.
  • We performed a wedge resection of a metastasis to the right lower lobe of the lung a year later, followed by chemotherapy and irradiation of the involved lung over a period of 30 weeks.
  • One and a half years later, he developed obstructive pneumonitis of the left lung.
  • Fiberoptic bronchoscopy identified a tumor at the left main bronchus with near total bronchial obstruction, compatible with findings on a computed tomographic scan.
  • Rigid bronchoscopy was performed to core out the obstructing tumor.
  • It was reported as a metastatic Wilms' tumor.
  • Successful reexpansion of the left upper lobe was achieved and he was discharged uneventfully, followed by adjuvant chemotherapy.
  • [MeSH-major] Kidney Neoplasms / pathology. Lung Neoplasms / secondary. Wilms Tumor / secondary
  • [MeSH-minor] Bronchoscopy. Child, Preschool. Combined Modality Therapy. Humans. Male. Pneumonia / etiology

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  • (PMID = 16150331.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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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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  • [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. Lamalmi N, Rouas L, Cherradi N, Malihy A, Khattab M, Alhamany Z: [Botryoid Wilms tumor extending into the duodenum]. Arch Pediatr; 2010 Dec;17(12):1664-6
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  • [Title] [Botryoid Wilms tumor extending into the duodenum].
  • We report on a rare case of botryoid Wilms tumor extending into the duodenum.
  • This uncommon macroscopic form of nephroblastoma consists entirely of a polypoid renal intrapelvic mass.
  • The main differential diagnosis of this unusual tumor is botryoid rhabdomyosarcoma.
  • Radiology revealed a large heterogeneous mass in the renal calyx, protruding into the ureter.
  • The tumor was found to extend into the 2nd portion of the duodenum.
  • The pathologic diagnosis was mixed type nephroblastoma, SIOP 2001 stage III.
  • The patient was given a course of postoperative chemotherapy.
  • No local recurrence or metastatic spread has been detected after 2 years.
  • To our knowledge, botryoid nephroblastoma has not previously been described as extending into the digestive system.
  • [MeSH-major] Duodenal Neoplasms / diagnosis. Kidney Neoplasms / diagnosis. Neoplasms, Multiple Primary / diagnosis. Ureteral Neoplasms / diagnosis. Wilms Tumor / diagnosis
  • [MeSH-minor] Chemotherapy, Adjuvant / methods. Diagnosis, Differential. Humans. Infant. Male. Neoplasm Invasiveness. Neoplasm Staging. Nephrectomy. Rhabdomyosarcoma / diagnosis. Treatment Outcome

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  • [Copyright] Copyright © 2010 Elsevier Masson SAS. All rights reserved.
  • (PMID = 20943355.001).
  • [ISSN] 1769-664X
  • [Journal-full-title] Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
  • [ISO-abbreviation] Arch Pediatr
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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24. Sultan I, Ajlouni F, Al-Jumaily U, Al-Ashhab M, Hashem H, Ghandour K, Masarweh M, Al-Hussaini M: Distinct features of teratoid Wilms tumor. J Pediatr Surg; 2010 Oct;45(10):e13-9
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  • [Title] Distinct features of teratoid Wilms tumor.
  • Teratoid Wilms tumor is an unusual variant of nephroblastoma in which heterologous tissue predominates.
  • Typically, histopathologic examination shows a mixture of mature squamous and mucus-producing columnar epithelium and a prominent component of mature adipose tissue.
  • The treatment of this tumor has not been established because of its rarity with some reports doubting the value of chemotherapy.
  • The first case was a 2-year-old boy with a huge tumor that occupied most of the abdominal cavity.
  • The second case was a 5-year-old girl with a metastatic tumor in association with horseshoe kidney and is the first case of teratoid Wilms tumor with brain metastasis.
  • Failure to respond to preoperative chemotherapy was seen in our patients and in most previously reported cases that were treated with preoperative chemotherapy.
  • We recommend treating this entity with protocols designed for Wilms tumor and to conduct further basic research to understand its biologic nature.
  • [MeSH-major] Kidney / pathology. Kidney Neoplasms / pathology. Teratoma / pathology. Wilms Tumor / pathology
  • [MeSH-minor] Child, Preschool. Female. Humans. Infant. Male. Tomography, X-Ray Computed

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  • [Copyright] Copyright © 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20920703.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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25. Kaste SC, Dome JS, Babyn PS, Graf NM, Grundy P, Godzinski J, Levitt GA, Jenkinson H: Wilms tumour: prognostic factors, staging, therapy and late effects. Pediatr Radiol; 2008 Jan;38(1):2-17
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  • [Title] Wilms tumour: prognostic factors, staging, therapy and late effects.
  • Wilms tumour is the most common malignant renal tumour in children.
  • Dramatic improvements in survival have occurred as the result of advances in anaesthetic and surgical management, irradiation and chemotherapy.
  • Current therapies are based on trials and studies primarily conducted by large multi-institutional cooperatives including the Société Internationale d'Oncologie Pédiatrique (SIOP) and the Children's Oncology Group (COG).
  • The primary goals are to treat patients according to well-defined risk groups in order to achieve the highest cure rates, to decrease the frequency and intensity of acute and late toxicity and to minimize the cost of therapy.
  • The SIOP trials and studies largely focus on the issue of preoperative therapy, whereas the COG trials and studies start with primary surgery.
  • This paper reviews prognostic factors and staging systems for Wilms tumour and its current treatment with surgery and chemotherapy.
  • Surgery remains a crucial part of treatment for nephroblastoma, providing local primary tumour control and adequate staging and possibly controlling the metastatic spread and central vascular extension of the disease.
  • The late effects for Wilms tumour and its treatment are also reviewed.
  • The treatment of Wilms tumour has been a success story, and currently in excess of 80% of children diagnosed with Wilms tumour can look forward to long-term survival, with less than 20% experiencing serious morbidity at 20 years from diagnosis.
  • The late complications are a consequence of the type and intensity of treatment required, which in turn reflects the nature and extent of the original tumour.
  • Continual international trial development and participation will improve matching of treatment needs with prognosis, reducing long-term complications in the majority.
  • [MeSH-major] Kidney Neoplasms / pathology. Kidney Neoplasms / therapy. Wilms Tumor / pathology. Wilms Tumor / therapy
  • [MeSH-minor] Child. Clinical Trials as Topic. Combined Modality Therapy. Humans. Male. Neoplasm Metastasis. Neoplasm Staging. Prognosis. Risk Factors. Survival Analysis

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  • [CommentIn] Pediatr Radiol. 2008 Apr;38(4):483; author reply 484-5 [18299824.001]
  • [CommentIn] Pediatr Radiol. 2008 Jan;38(1):1 [18026722.001]
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  • (PMID = 18026723.001).
  • [ISSN] 0301-0449
  • [Journal-full-title] Pediatric radiology
  • [ISO-abbreviation] Pediatr Radiol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 119
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26. Reinhard H, Aliani S, Ruebe C, Stöckle M, Leuschner I, Graf N: Wilms' tumor in adults: results of the Society of Pediatric Oncology (SIOP) 93-01/Society for Pediatric Oncology and Hematology (GPOH) Study. J Clin Oncol; 2004 Nov 15;22(22):4500-6
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  • [Title] Wilms' tumor in adults: results of the Society of Pediatric Oncology (SIOP) 93-01/Society for Pediatric Oncology and Hematology (GPOH) Study.
  • PURPOSE: In the Society of Pediatric Oncology (SIOP) 93-01 study, 30 patients older than 16 years were found to have Wilms' tumor.
  • They were treated according to the pediatric protocol and were analyzed for clinical presentation, stage distribution, and prognosis.
  • Tumor stages were defined according to SIOP, and treatment was risk-adapted according to SIOP 93-01/Society for Pediatric Oncology and Hematology (GPOH) protocol.
  • RESULTS: Ten patients (33%) had metastatic disease at the time of diagnosis (liver, four patients; lung, three patients; liver and lung, three patients).
  • The local stage distribution showed a predominance of higher stages (stage I, eight patients; stage IIN-, three patients; stage IIN+, four patients; stage III, 15 patients).
  • Two of 30 patients showed a nephroblastoma and a renal cell carcinoma simultaneously in the same kidney.
  • A complete remission was achieved in 24 patients; four patients relapsed after complete remission; and three of them reached a second remission with further treatment.
  • Event-free survival was 57%, with an overall survival of 83% (median observation time, 4 years).
  • CONCLUSION: Adults can be cured in a high percentage by a multimodal treatment according to pediatric protocols.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Wilms Tumor / drug therapy. Wilms Tumor / pathology
  • [MeSH-minor] Adolescent. Adult. Age Factors. Disease-Free Survival. Female. Humans. Male. Middle Aged. Neoplasm Staging. Prognosis. Retrospective Studies. Treatment Outcome

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  • (PMID = 15542800.001).
  • [ISSN] 0732-183X
  • [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; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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27. Schwinger W, Klass V, Benesch M, Lackner H, Dornbusch HJ, Sovinz P, Moser A, Schwantzer G, Urban C: Feasibility of high-dose interleukin-2 in heavily pretreated pediatric cancer patients. Ann Oncol; 2005 Jul;16(7):1199-206
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  • BACKGROUND: The administration of high-dose interleukin-2 (IL-2) seems to be a therapeutic option for children with refractory and metastatic solid malignancies.
  • METHODS: We prospectively studied treatment-related toxicities, quality of life and laboratory parameters in 10 children with progressive or metastatic solid tumors (metastatic osteosarcoma, n=4; neuroblastoma stage IV, n=3; metastatic Ewing's sarcoma, n=2; metastatic Wilms' tumor, n=1) during IL-2 therapy.
  • RESULTS: All patients developed fever >39 degrees C and influenza-like symptoms, with a significant decrease in Karnofsky score.
  • In two patients treatment had to be stopped after three cycles because of severe side-effects.
  • During IL-2 therapy a statistical significant increase in white blood cells (WBC), creatinine, gamma-glutamyltransferase, C-reactive protein, glucose and body weight was observed.
  • No constant quantitative changes in total lymphocytes and subsets were observed during IL-2 therapy.
  • CONCLUSIONS: IL-2 treatment in children with refractory and relapsed solid malignancies is associated with severe, but reversible, side-effects.
  • However, five of the 10 patients with diseases of worst prognosis could be rescued by this treatment.

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  • (PMID = 15849223.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Interleukin-2
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