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1. Little SE, Bax DA, Rodriguez-Pinilla M, Natrajan R, Messahel B, Pritchard-Jones K, Vujanic GM, Reis-Filho JS, Jones C: Multifaceted dysregulation of the epidermal growth factor receptor pathway in clear cell sarcoma of the kidney. Clin Cancer Res; 2007 Aug 1;13(15 Pt 1):4360-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Multifaceted dysregulation of the epidermal growth factor receptor pathway in clear cell sarcoma of the kidney.
  • PURPOSE: Epidermal growth factor receptor (EGFR) is a receptor tyrosine kinase overexpressed in a variety of human malignancies, against which targeted therapies have shown efficacy in lung and brain tumors.
  • We sought to evaluate these variables in pediatric renal tumors.
  • EXPERIMENTAL DESIGN: We screened a series of 307 pediatric renal tumors for EGFR expression by immunohistochemistry and gene amplification by chromogenic in situ hybridization.
  • In identifying a striking predilection for certain tumor types, we further analyzed the clear cell sarcomas of the kidney (CCSK) for mutations in EGFR and PTEN.
  • Identification of factors predictive of poor response to targeted therapy, including the drug resistance T790M mutation, may provide a rationale for upfront trials with irreversible inhibitors of EGFR in children with these tumors.
  • [MeSH-major] Kidney Neoplasms / metabolism. PTEN Phosphohydrolase / metabolism. Receptor, Epidermal Growth Factor / metabolism. Sarcoma, Clear Cell / metabolism. Signal Transduction
  • [MeSH-minor] Child, Preschool. Gene Amplification. Humans. Immunoenzyme Techniques. In Situ Hybridization. Infant. Mutation. Nephroma, Mesoblastic / metabolism. Nephroma, Mesoblastic / pathology. Phosphorylation. Proto-Oncogene Proteins c-akt / metabolism. Rhabdoid Tumor / metabolism. Rhabdoid Tumor / pathology. Tissue Array Analysis. Wilms Tumor / metabolism. Wilms Tumor / pathology

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  • (PMID = 17646270.001).
  • [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
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.7.10.1 / Receptor, Epidermal Growth Factor; EC 2.7.11.1 / Proto-Oncogene Proteins c-akt; EC 3.1.3.48 / PTEN protein, human; EC 3.1.3.67 / PTEN Phosphohydrolase
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2. Rajalakshmi V, Chandran P, Selvambigai, Ganesh J: Metanephric stromal tumor: a novel pediatric renal neoplasm. Indian J Pathol Microbiol; 2009 Jul-Sep;52(3):389-91
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  • [Title] Metanephric stromal tumor: a novel pediatric renal neoplasm.
  • Metanephric stromal tumor of kidney is a novel pediatric benign stromal specific renal neoplasm.
  • This tumor is usually centered in the renal medulla with a characteristic microscopic appearance which differentiates this lesion from congenital mesoblastic nephroma and clear cell sarcoma of the kidney.
  • The differentiation of metanephric stromal tumor from clear cell sarcoma of the kidney will spare the child from the ill effects of adjuvant chemotherapy.
  • [MeSH-major] Kidney Neoplasms / diagnosis. Kidney Neoplasms / pathology. Stromal Cells / pathology
  • [MeSH-minor] Diagnosis, Differential. Female. Histocytochemistry. Humans. Infant, Newborn. Nephroma, Mesoblastic / diagnosis. Sarcoma, Clear Cell / diagnosis

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  • (PMID = 19679970.001).
  • [ISSN] 0974-5130
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
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3. Mitchell C, Jones PM, Kelsey A, Vujanic GM, Marsden B, Shannon R, Gornall P, Owens C, Taylor R, Imeson J, Middleton H, Pritchard J: The treatment of Wilms' tumour: results of the United Kingdom Children's cancer study group (UKCCSG) second Wilms' tumour study. Br J Cancer; 2000 Sep;83(5):602-8
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  • [Title] The treatment of Wilms' tumour: results of the United Kingdom Children's cancer study group (UKCCSG) second Wilms' tumour study.
  • (1) to further refine treatment for stage I and II favourable histology (FH) patients;.
  • (3) to improve the outlook for patients with inoperable primary tumours and those patients with stage IV and unfavourable histology disease.
  • Treatment consisted of primary nephrectomy, wherever possible, followed by chemotherapy and radiotherapy, as dictated by stage and histology.
  • Treatment was refined successfully for stage I and II FH patients.
  • The outlook for patients with clear cell sarcoma of the kidney is as good as for patients with favourable histology, whilst that for patients with anaplastic or rhabdoid variants remains poor.
  • [MeSH-major] Kidney Neoplasms / drug therapy. Kidney Neoplasms / radiotherapy. Kidney Neoplasms / surgery. Wilms Tumor / drug therapy. Wilms Tumor / radiotherapy. Wilms Tumor / surgery
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Disease-Free Survival. Female. Follow-Up Studies. Humans. Infant. Infant, Newborn. Male. Rhabdoid Tumor / drug therapy. Rhabdoid Tumor / radiotherapy. Rhabdoid Tumor / surgery. Sarcoma, Clear Cell / drug therapy. Sarcoma, Clear Cell / radiotherapy. Sarcoma, Clear Cell / surgery. Time Factors. Vincristine / therapeutic use

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  • [Copyright] Copyright 2000 Cancer Research Campaign.
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  • (PMID = 10944599.001).
  • [ISSN] 0007-0920
  • [Journal-full-title] British journal of cancer
  • [ISO-abbreviation] Br. J. Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] SCOTLAND
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine
  • [Other-IDs] NLM/ PMC2363501
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4. Hannachi Sassi S, Braham E, Oubiche F, Mrad K, Abbes I, Barsaoui S, Ben Romdhane K: [Clear-cell sarcoma of the kidney. Two pediatric cases]. Ann Pathol; 2008 Feb;28(1):36-40
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  • [Title] [Clear-cell sarcoma of the kidney. Two pediatric cases].
  • [Transliterated title] Sarcome à cellules claires du rein. A propos de deux cas pédiatriques.
  • Clear-cell sarcoma of the kidney is a rare pediatric renal sarcoma with poor prognosis and propensity to metastasize to bone.
  • It is a distinctive renal malignancy regarded as a morphologic feature of Wilms' tumor.
  • They were treated by surgery and chemotherapy.
  • The histological diagnosis was clear cell sarcoma.
  • [MeSH-major] Carcinoma, Renal Cell / pathology. Carcinoma, Renal Cell / surgery. Kidney Neoplasms / pathology. Kidney Neoplasms / surgery
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Child, Preschool. Combined Modality Therapy. Dactinomycin / therapeutic use. Humans. Infant. Male. Treatment Outcome. Vincristine / therapeutic use

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  • (PMID = 18538713.001).
  • [ISSN] 0242-6498
  • [Journal-full-title] Annales de pathologie
  • [ISO-abbreviation] Ann Pathol
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine
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5. Jones C, Rodriguez-Pinilla M, Lambros M, Bax D, Messahel B, Vujanic GM, Reis-Filho JS, Pritchard-Jones K: c-KIT overexpression, without gene amplification and mutation, in paediatric renal tumours. J Clin Pathol; 2007 Nov;60(11):1226-31
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  • [Title] c-KIT overexpression, without gene amplification and mutation, in paediatric renal tumours.
  • AIMS: To investigate the presence and prognostic relevance of KIT expression in paediatric renal tumours, and to determine whether receptor overexpression is associated with gene amplification and/or mutation.
  • METHODS: Immunohistochemistry without antigen retrieval for CD117 was carried out on tissue microarrays consisting of 274 Wilms' tumours, 13 clear cell sarcomas of the kidney (CCSK), 10 mesoblastic nephromas (MN), and 7 rhabdoid tumours of the kidney (RTK).
  • RESULTS: Only 8/200 (4.0%) Wilms' tumours exhibited any degree of moderate-strong KIT staining in any of their assessable cell types.
  • This small group of KIT-positive tumours had a shorter time to relapse (p = 0.0044, log-rank test).
  • CONCLUSIONS: KIT overexpression in rare in Wilms' tumours, although does appear to confer a worse prognosis, in particular for patients primarily treated with preoperative chemotherapy.
  • The potential of anti-KIT therapeutic strategies in the treatment of paediatric renal tumours appears to be limited.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Kidney Neoplasms / metabolism. Proto-Oncogene Proteins c-kit / metabolism. Sarcoma, Clear Cell / metabolism. Wilms Tumor / metabolism
  • [MeSH-minor] Antineoplastic Agents. Child. DNA Mutational Analysis. DNA, Neoplasm / genetics. Gene Amplification. Humans. In Situ Hybridization / methods. Neoadjuvant Therapy. Nephrectomy. Prognosis. Survival Analysis. Tissue Array Analysis / methods

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  • (PMID = 17965221.001).
  • [ISSN] 1472-4146
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Grant] United Kingdom / Cancer Research UK / / 11886
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Biomarkers, Tumor; 0 / DNA, Neoplasm; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
  • [Other-IDs] NLM/ PMC2095465
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6. Koga F, Kawano K, Honda M, Sumi S, Horimi H, Kondo S, Yoshida K: Sarcomatoid renal cell carcinoma with scant carcinomatous components. Int J Urol; 2000 Feb;7(2):58-60; discussion 61
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  • [Title] Sarcomatoid renal cell carcinoma with scant carcinomatous components.
  • A 30-year-old male underwent radical nephrectomy for a right renal tumor 15 cm in diameter.
  • At that time a diagnosis of rhabdomyosarcoma of the kidney was made.
  • However, further microscopic examination of another eight sections revealed small areas of clear cell-type renal cell carcinoma (RCC) which transited to sarcomatous components and led to a diagnosis of sarcomatoid RCC.
  • The patient underwent three cycles of adjuvant chemotherapy.
  • He has been free of the disease for 14 months after nephrectomy.
  • [MeSH-major] Carcinoma, Renal Cell / pathology. Kidney Neoplasms / pathology
  • [MeSH-minor] Adult. Humans. Male. Sarcoma / pathology

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  • (PMID = 10710249.001).
  • [ISSN] 0919-8172
  • [Journal-full-title] International journal of urology : official journal of the Japanese Urological Association
  • [ISO-abbreviation] Int. J. Urol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] AUSTRALIA
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7. Wang E, Lichtenfels R, Bükur J, Ngalame Y, Panelli MC, Seliger B, Marincola FM: Ontogeny and oncogenesis balance the transcriptional profile of renal cell cancer. Cancer Res; 2004 Oct 15;64(20):7279-87
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  • [Title] Ontogeny and oncogenesis balance the transcriptional profile of renal cell cancer.
  • We compared the transcriptional profile of primary renal cell cancers (RCCs) with that of normal kidney tissue and several epithelial cancers of nonrenal origin to weigh the contribution that ontogeny and oncogenesis make in molding their genetic profile.
  • Unsupervised global transcript analysis demonstrated that RCCs retain transcriptional signatures related to their ontogeny and cluster close to normal renal epithelium.
  • When renal lineage-associated genes are removed from the analysis and cancer-specific genes are analyzed, RCCs segregate with other cancers with limited lineage specificity underlying a predominance of the oncogenic process over lineage specificity.
  • Genes responsible for lineage specificity may represent poor molecular targets for immune or drug therapy.
  • Most genes associated with oncogenesis are shared with other cancers and may represent better therapeutic targets.
  • Finally, a small subset of genes is associated with lineage-specific oncogenesis, and these may provide information regarding the biological behavior of RCCs and facilitate diagnostic classification of RCCs.
  • [MeSH-major] Carcinoma, Renal Cell / genetics. Kidney Neoplasms / genetics
  • [MeSH-minor] Adenocarcinoma, Clear Cell / enzymology. Adenocarcinoma, Clear Cell / genetics. Adenocarcinoma, Clear Cell / metabolism. Carbonic Anhydrases / biosynthesis. Carbonic Anhydrases / genetics. Disease Progression. Gene Expression Profiling. Glutathione Transferase / biosynthesis. Glutathione Transferase / genetics. Humans. Melanoma / genetics. Melanoma / metabolism. Multigene Family. Prognosis. Sarcoma / genetics. Sarcoma / metabolism. Transcription, Genetic

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  • (PMID = 15492247.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.5.1.18 / Glutathione Transferase; EC 4.2.1.1 / Carbonic Anhydrases
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8. Rosso D, Ghignone GP, Bernardi D, Zitella A, Casetta G, De Zan A, Tizzani A: Clear cell sarcoma of the kidney with invasion of the inferior vena cava. Urol Int; 2003;70(3):251-2
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  • [Title] Clear cell sarcoma of the kidney with invasion of the inferior vena cava.
  • OBJECTIVE: We present a case of clear cell sarcoma of the kidney (CCSK) in 53-year-old white man who was treated with surgery.
  • This case represents the oldest patient with CCSK published in the English literature.
  • RESULTS: Histological findings indicated a CCSK.
  • CONCLUSION: CCSK is considered a rare and highly malignant renal tumor.
  • The malignant nature may relate not only to the biological features of these tumor cells, but also to the high resistance against radiation and chemotherapy.
  • The treatment of CCSK has been a subject of controversy.
  • [MeSH-major] Kidney Neoplasms / pathology. Neoplasms, Germ Cell and Embryonal / pathology. Vena Cava, Inferior / pathology

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  • [Copyright] Copyright 2003 S. Karger AG, Basel
  • (PMID = 12660471.001).
  • [ISSN] 0042-1138
  • [Journal-full-title] Urologia internationalis
  • [ISO-abbreviation] Urol. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Switzerland
  • [Number-of-references] 13
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9. 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.
  • [MeSH-major] Kidney Neoplasms / pathology. Mandibular Neoplasms / secondary. Sarcoma, Clear Cell / secondary

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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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10. Benchekroun A, Zannoud M, el Alj HA, Nouini Y, Marzouk M, Faik M: [Clear cell sarcoma of the kidney: 3 case reports]. Prog Urol; 2002 Jun;12(3):469-73
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  • [Title] [Clear cell sarcoma of the kidney: 3 case reports].
  • [Transliterated title] Sarcome à cellules claires du rein (à propos de trois observations).
  • Clear cell carcoma of the kidney is a distinct, highly malignant pediatric neoplasm.
  • MATERIAL AND METHODS: We report 3 cases of clear cell sarcoma of the kidney in one men and two women between 23 and 65 years old (mean age is 40 years).
  • A combination chemotherapy regiment (cisplatin and doxorubicin) was performed on 6 cycles in 1 case.
  • The other 2 case was not underwent chemotherapy or radiation.
  • RESULTS: In the patient underwent the combination chemotherapy there was not evidence of tumor in the abdomen and thorax on CT Scan 4 years later.
  • In one of the two patient not underwent chemotherapy or radiation, the CT scan revealed a left psoas reccurrence three month later; therapy consisted for surgery without chemotherapy or radiation.
  • The other patient not underwent chemotherapy or radiation was dead seven month after nephrectomy.
  • CONCLUSION: Optimal treatment is unknown, and surgery; radiotherapy and chemotherapy are used alone but mostly in combination.
  • [MeSH-major] Kidney Neoplasms. Sarcoma, Clear Cell
  • [MeSH-minor] Adult. Aged. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cisplatin / administration & dosage. Doxorubicin / administration & dosage. Fatal Outcome. Female. Humans. Male. Nephrectomy / methods

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  • (PMID = 12189758.001).
  • [ISSN] 1166-7087
  • [Journal-full-title] Progrès en urologie : journal de l'Association française d'urologie et de la Société française d'urologie
  • [ISO-abbreviation] Prog. Urol.
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 80168379AG / Doxorubicin; Q20Q21Q62J / Cisplatin
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11. Radulescu VC, Gerrard M, Moertel C, Grundy PE, Mathias L, Feusner J, Diller L, Dome JS: Treatment of recurrent clear cell sarcoma of the kidney with brain metastasis. Pediatr Blood Cancer; 2008 Feb;50(2):246-9
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  • [Title] Treatment of recurrent clear cell sarcoma of the kidney with brain metastasis.
  • BACKGROUND: Clear cell sarcoma of the kidney (CCSK) is known for its propensity to metastasize to bone, but it also spreads to other sites including the brain.
  • This study was undertaken to describe the treatment and outcomes of patients with recurrent CCSK involving the brain.
  • METHODS: A retrospective records review was conducted on eight patients with CCSK who developed brain metastases after complete responses to initial therapy.
  • RESULTS: The recurrences occurred at a median of 24.5 months after initial diagnosis (range, 12-53 months).
  • At the time of recurrence, patients were treated with multimodal therapy including biopsy or resection, radiation therapy, and chemotherapy.
  • Four patients received high-dose chemotherapy with autologous stem cell rescue.
  • One patient died from complications of bacteremia 8 weeks after starting chemotherapy.
  • The other seven patients achieved a complete response after either surgery or ICE chemotherapy.
  • Of these, six patients were alive without disease with a median follow-up of 30 months from the time of recurrence (range, 24 to 71 months).
  • All six survivors received radiation therapy and four had gross total resections.
  • Three survivors received high-dose chemotherapy with stem cell rescue.
  • CONCLUSION: Patients with recurrent CCSK involving the brain can have durable survival after recurrence.
  • ICE chemotherapy, together with radiation therapy and surgery, provides a reasonable salvage regimen for recurrent CCSK.
  • It is unclear whether high-dose chemotherapy confers a benefit compared to conventional-dose chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / secondary. Brain Neoplasms / therapy. Kidney Neoplasms / therapy. Neoplasm Recurrence, Local / therapy. Sarcoma, Clear Cell / secondary. Sarcoma, Clear Cell / therapy
  • [MeSH-minor] Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Neoplasm Staging. Retrospective Studies. Treatment Outcome

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17226850.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA-21765
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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12. Dundar E, Arslantas A, Acikalin MF, Tel E: Cerebellar metastasis from clear cell sarcoma of the kidney. A case report with immunohistochemistry. J Neurosurg Sci; 2001 Dec;45(4):228-31; discussion 231
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  • [Title] Cerebellar metastasis from clear cell sarcoma of the kidney. A case report with immunohistochemistry.
  • The aim of this study was to describe a child with a right cerebellar hemisphere metastasis from primary clear cell sarcoma of the kidney without evidence of bone metastases, and to investigate the immunohistochemical features of primary and metastatic tumors.
  • Abdominal computed tomography revealed a large right renal tumor.
  • Histopathologic examination of tumor revealed clear cell sarcoma of the kidney.
  • The patient received radiotherapy and chemotherapy in postoperative period.
  • A computed tomography scan revealed a tumor that was enhanced with contrast medium at right cerebellar hemisphere concomitant with ventricular enlargement.
  • After ventriculo-peritoneal shunting procedure, tumor was excised totally and histopathologic diagnosis showed metastasis of clear cell sarcoma of the kidney.
  • The treatment consisted of surgery, radiotherapy and chemotherapy.
  • Clear cell sarcoma of the kidney is most commonly associated with bone metastasis.
  • Cerebellar metastasis of clear cell sarcoma of the kidney is very unusual.
  • With review of the literature, our immunohistochemical findings support the theory that relapse and metastasis of primary clear cell sarcoma of the kidney are not related with increase of aggressiveness.
  • [MeSH-major] Cerebellar Neoplasms / secondary. Kidney Neoplasms / pathology. Sarcoma, Clear Cell / secondary
  • [MeSH-minor] Humans. Immunohistochemistry. Infant. Magnetic Resonance Imaging. Male. Tomography, X-Ray Computed

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  • (PMID = 11912476.001).
  • [ISSN] 0390-5616
  • [Journal-full-title] Journal of neurosurgical sciences
  • [ISO-abbreviation] J Neurosurg Sci
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Italy
  • [Number-of-references] 11
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13. Benchekroun A, Ghadouane M, Zannoud M, Alami M, Amhajji R, Faik M: Clear cell sarcoma of the kidney in an adult. A case report. Ann Urol (Paris); 2002 Jan;36(1):33-5
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  • [Title] Clear cell sarcoma of the kidney in an adult. A case report.
  • DEFINITION: Clear cell sarcoma of the kidney was initially thought to be a variant of Wilms tumour with an unfavourable prognosis.
  • METHODS: A 23 years-old woman presented with a right abdominal mass.
  • CT scan revealed a solid 23 cm tumour of the right kidney.
  • The histological diagnosis was clear cell sarcoma.
  • A combination chemotherapy regimen (cisplatin and doxorubicin) was performed on six cycles.
  • CONCLUSION: Optimal treatment is unknown, and surgery, radiotherapy and chemotherapy are used alone but mostly in combination.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / surgery. Nephrectomy. Sarcoma, Clear Cell / surgery
  • [MeSH-minor] Adult. Cisplatin / administration & dosage. Combined Modality Therapy. Doxorubicin / administration & dosage. Female. Humans. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 11859574.001).
  • [ISSN] 0003-4401
  • [Journal-full-title] Annales d'urologie
  • [ISO-abbreviation] Ann Urol (Paris)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 80168379AG / Doxorubicin; Q20Q21Q62J / Cisplatin
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14. Hadley GP, Sheik-Gafoor MH: Clear cell sarcoma of the kidney in children: experience in a developing country. Pediatr Surg Int; 2010 Apr;26(4):345-8
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  • [Title] Clear cell sarcoma of the kidney in children: experience in a developing country.
  • INTRODUCTION: Clear cell sarcoma of the kidney (CCSK) is a rare tumour comprising 4% of primary renal tumours in children.
  • AIM: To describe the clinical and pathological features of CCSK in children in our practice and to identify factors contributing to poor patient outcomes.
  • METHOD: A retrospective review of patients with a confirmed diagnosis of CCSK who presented for treatment at a single institution between 1990 and 2008.
  • They represented 4% of 356 patients presenting with primary renal tumours during the review period.
  • Lung and lymph node metastases were more common than skeletal disease.
  • Initial diagnosis by needle biopsy was correct in only two of seven patients (29%) leading to inappropriate neoadjuvant chemotherapy.
  • Overall survival is poor with 57% of patients alive and disease free from 1 to 7 years off treatment.
  • CONCLUSION: In a developing country, CCSK is rare and clinically and radiologically indistinguishable from Wilms tumour.
  • Pretreatment diagnosis is difficult and sampling errors using needle biopsies may be unavoidable.
  • Treatment results are poor and, given the propensity for late recurrence in CCSK, may not be sustained.
  • [MeSH-major] Bone Neoplasms / epidemiology. Developing Countries / statistics & numerical data. Kidney Neoplasms / epidemiology. Lung Neoplasms / epidemiology. Neoplasm Recurrence, Local / epidemiology. Sarcoma, Clear Cell / epidemiology
  • [MeSH-minor] Biopsy, Needle. Child. Child, Preschool. Cohort Studies. Comorbidity. Diagnosis, Differential. Female. Humans. Hypertension / epidemiology. Infant. Lymphatic Metastasis. Male. Neoplasm Staging. Retrospective Studies. South Africa / epidemiology. Survival Analysis. Tomography, X-Ray Computed / methods. Wilms Tumor / diagnostic imaging

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  • (PMID = 20127337.001).
  • [ISSN] 1437-9813
  • [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. El Kababri M, Khattab M, El Khorassani M, Hessissen L, Kili A, Nachef MN, Cherradi N, Malihy A, Alhamany Z, Msefer-Alaoui F: [Clear cell sarcoma of the kidney. A study of 13 cases]. Arch Pediatr; 2004 Jul;11(7):794-9
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  • [Title] [Clear cell sarcoma of the kidney. A study of 13 cases].
  • [Transliterated title] Sarcome rénal à cellules claires. A propos d'une série de 13 cas.
  • Clear cell sarcoma of the kidney (CCSK) also called a "bone-metastasizing renal tumor of childhood" is the second common pediatric renal neoplasm.
  • This tumor is associated with a higher rate of relapse and a wider distribution of metastases than Wilms' tumor.
  • PATIENTS AND METHODS: We have reviewed records of 13 cases of CCSK among 277 renal tumors (5%) diagnosed at the children's hospital of Rabat between 1990 and 2002.
  • RESULTS: The median age at diagnosis was 14 months (5 months-9 years).
  • Preoperative chemotherapy was given according to the SIOP9, SIOP93-01 and GFAOP 98 protocols.
  • The classic morphologic pattern was seen in nine cases (69%).
  • Postoperative chemotherapy and radiotherapy (21 600-30 600 cGy) was done in 10 cases.
  • With a median follow up of 44 months, four patients showed bone metastases (31%), four are alive in CR, four are lost for follow up and five died.
  • CONCLUSION: CCSK remains the pediatric renal tumor most frequently misdiagnosed.
  • Its aggressiveness and its ability to give bone metastases need to recognize early this diagnosis for an adapted treatment.
  • [MeSH-major] Kidney Neoplasms / pathology. Kidney Neoplasms / surgery. Nephrectomy. Sarcoma, Clear Cell / pathology. Sarcoma, Clear Cell / surgery
  • [MeSH-minor] Age of Onset. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Neoadjuvant Therapy. Neoplasm Staging. Prognosis. Retrospective Studies. Sex Factors. Survival Analysis

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  • (PMID = 15234374.001).
  • [ISSN] 0929-693X
  • [Journal-full-title] Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
  • [ISO-abbreviation] Arch Pediatr
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] France
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16. Seibel NL, Li S, Breslow NE, Beckwith JB, Green DM, Haase GM, Ritchey ML, Thomas PR, Grundy PE, Finklestein JZ, Kim T, Shochat SJ, Kelalis PP, D'Angio GJ: Effect of duration of treatment on treatment outcome for patients with clear-cell sarcoma of the kidney: a report from the National Wilms' Tumor Study Group. J Clin Oncol; 2004 Feb 1;22(3):468-73
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  • [Title] Effect of duration of treatment on treatment outcome for patients with clear-cell sarcoma of the kidney: a report from the National Wilms' Tumor Study Group.
  • PURPOSE: To evaluate the effect of conventional and standard (ST) versus pulse-intensive (PI) chemotherapy and short-duration versus long-duration chemotherapy on relapse-free survival (RFS) and overall survival rates of patients with clear-cell sarcoma of the kidney (CCSK) entered onto the National Wilms' Tumor Study (NWTS)-4.
  • PATIENTS AND METHODS: The 5-year and 8-year RFS rates were determined for patients with CCSK treated on the NWTS-4.
  • After August 6, 1986, 40 previously untreated children younger than 16 years with CCSK were randomly assigned, after the completion of 6 months of chemotherapy, to discontinue (short) or continue 9 additional months (long) of treatment with chemotherapy regimens that included vincristine and either divided-dose (ST) courses (5 days) or single-dose (PI) treatment with dactinomycin and divided-dose (ST) courses (3 days) or single-dose (PI) treatment with doxorubicin.
  • RESULTS: For patients with CCSK, the 5- and 8-year RFS rates were 65.2% and 60.6%, respectively, for patients randomly assigned to the short chemotherapy and 87.8% (both 5- and 8-year RFS) for patients randomly assigned to the long chemotherapy (P =.08).
  • The overall survival rates for patients at 5 and 8 years were 95.5% and 85.9%, respectively, for the short chemotherapy and 87.5% (both 5- and 8-year overall survival) for the long chemotherapy (P =.99).
  • In NWTS-4, the overall survival rates for patients with CCSK improved from NWTS-3 (83% v 66.9% at 8 years, respectively; P <.01).
  • CONCLUSION: CCSK patients exhibit an improved RFS from a longer course of therapy when using vincristine, doxorubicin, and dactinomycin, but their long-term survival is unchanged compared with patients receiving 6 months of therapy.
  • The overall survival rates for patients with CCSK have improved from NWTS-3.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Neoplasm Recurrence, Local / prevention & control. Sarcoma, Clear Cell / drug therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Dactinomycin / administration & dosage. Disease-Free Survival. Doxorubicin / administration & dosage. Female. Humans. Infant. Infant, Newborn. Male. Middle Aged. Neoplasm Staging. Survival Rate. Time Factors. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 14752069.001).
  • [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
  • [Grant] United States / NCI NIH HHS / CA / CA 42326
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Randomized Controlled Trial; 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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17. Kukreja M, Kamal M, Iyer VK, Mannan AA, Agarwal S: Anaplastic variant of clear cell sarcoma of the kidney: a rare case report. Gulf J Oncolog; 2010 Jul;(8):55-8
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  • [Title] Anaplastic variant of clear cell sarcoma of the kidney: a rare case report.
  • Clear cell sarcoma (CCSK) of the kidney is an uncommon but distinctive pediatric renal tumor with a characteristic histological pattern and marked propensity for bone metastasis.
  • The rare anaplastic variant constitutes about 3% of cases of CCSK and carries an unfavorable prognosis, with increased tumor recurrence and resistant to chemotherapy.
  • This variant show high frequency of p53 gene mutation and p53 over expression in comparison to the usual CCSK.
  • We present a case of anaplastic variant of CCSK in an 10-year-old boy with both cytologic and histologic features, highlighting the importance of recognizing this rare entity.
  • [MeSH-major] Kidney Neoplasms / pathology. Sarcoma, Clear Cell / pathology

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  • (PMID = 20601342.001).
  • [ISSN] 2078-2101
  • [Journal-full-title] The Gulf journal of oncology
  • [ISO-abbreviation] Gulf J Oncolog
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Kuwait
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18. Hempel L, Sauerbrey A, Zintl F, Weirich A, Lemmer A, Graf N: Successful management of a child with clear cell sarcoma of the kidney (CCSK) and multifocal bone metastases at diagnosis. Med Pediatr Oncol; 2003 Jul;41(1):97-9
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  • [Title] Successful management of a child with clear cell sarcoma of the kidney (CCSK) and multifocal bone metastases at diagnosis.
  • [MeSH-major] Bone Neoplasms / therapy. Kidney Neoplasms / therapy. Sarcoma, Clear Cell / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child, Preschool. Combined Modality Therapy. Dactinomycin / administration & dosage. Diagnosis, Differential. Drug Administration Schedule. Female. Hematopoietic Stem Cell Mobilization. Humans. Radiotherapy, Adjuvant. Vincristine / administration & dosage

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  • (PMID = 12764765.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; SIOP protocol
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19. Palese MA, Ferrer F, Perlman E, Gearhart JP: Metanephric stromal tumor: a rare benign pediatric renal mass. Urology; 2001 Sep;58(3):462
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  • [Title] Metanephric stromal tumor: a rare benign pediatric renal mass.
  • We report a rare case and description of a benign pediatric renal mass.
  • It is possible that the identification of this renal tumor could spare a child the toxic adjuvant chemotherapy that would be administered if confused with histologically similar tumors such as clear cell sarcoma of the kidney.
  • [MeSH-major] Adenofibroma / diagnosis. Kidney Diseases, Cystic / diagnosis. Kidney Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Diagnosis, Differential. Humans. Kidney / pathology. Male. Nephroma, Mesoblastic / diagnosis. Nephroma, Mesoblastic / pathology. Sarcoma, Clear Cell / diagnosis. Stromal Cells / pathology. Tomography, X-Ray Computed

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  • (PMID = 11549506.001).
  • [ISSN] 1527-9995
  • [Journal-full-title] Urology
  • [ISO-abbreviation] Urology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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20. Ng A, Jenkinson H, Morland B, Grundy R: Clear cell sarcoma: a dilemma on pathological staging and clinical management. Pediatr Hematol Oncol; 2005 Apr-May;22(3):257-61
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  • [Title] Clear cell sarcoma: a dilemma on pathological staging and clinical management.
  • Clear cell sarcoma of the kidney (CCSK) has been classified as high risk tumour in the previous UK and international Wilms tumor studies.
  • The current Society of Paediatric Oncology (SIOP) trial, UK version, advocates chemotherapy including doxorubicin prior to nephrectomy.
  • Pathological staging and histology of the resected tumor are then applied to determine the extent and intensity of the postoperative therapy.
  • We describe an unusual case with a trilobed tumor and debate the appropriate postoperative management.
  • [MeSH-major] Kidney Neoplasms / pathology. Sarcoma, Clear Cell / pathology
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Doxorubicin / therapeutic use. Humans. Infant. Male. Neoplasm Staging. Nephrectomy / methods. Postoperative Care / methods

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  • (PMID = 16020110.001).
  • [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] 80168379AG / Doxorubicin
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21. Radhika S, Bakshi A, Rajwanshi A, Nijhawan R, Das A, Kakkar N, Joshi K, Marwaha RK, Rao KL: Cytopathology of uncommon malignant renal neoplasms in the pediatric age group. Diagn Cytopathol; 2005 May;32(5):281-6
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  • [Title] Cytopathology of uncommon malignant renal neoplasms in the pediatric age group.
  • Malignant renal neoplasms are common solid tumors in pediatric oncology practice.
  • These include the common Wilms' tumor/nephroblastoma and the uncommon neoplasms such as clear-cell sarcoma of the kidney (CCSK), rhabdoid tumor, renal-cell carcinoma, and others.
  • The aim of this study was to describe in detail the cytopathological features of the histopathologically proven uncommon pediatric renal tumors.
  • CCSK, classic subtype, is characterized by round to oval cells arranged perivascularly and also in sheets and clusters intimately associated with a metachromatic matrix mucopolysaccharide material better appreciated in May-Grunwald-Giemsa (MGG)-stained smears.
  • Spindle-cell pattern of CCSK is difficult to diagnose on aspiration cytology.
  • Renal-cell carcinoma of childhood shows similar cytological features as its adult counterpart.
  • Rhabdoid tumor of the kidney is characterized by a monomorphic population of cells with abundant cytoplasm, eccentric nuclei with prominent nucleoli.
  • Further, non-Wilms' renal malignant neoplasms must be distinguished from the common Wilms' tumor so that appropriate chemotherapy protocols may be instituted in cases where the tumor is in an advanced stage of malignancy.
  • [MeSH-major] Biopsy, Fine-Needle / methods. Carcinoma, Renal Cell / pathology. Endodermal Sinus Tumor / pathology. Kidney Neoplasms / pathology. Rhabdoid Tumor / pathology. Sarcoma, Clear Cell / pathology
  • [MeSH-minor] Adolescent. Cell Nucleus / pathology. Child. Child, Preschool. Diagnosis, Differential. Humans. Infant. Staining and Labeling. Wilms Tumor / pathology

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  • [Copyright] Copyright 2005 Wiley-Liss, Inc.
  • (PMID = 15830360.001).
  • [ISSN] 8755-1039
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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22. Argani P, Beckwith JB: Metanephric stromal tumor: report of 31 cases of a distinctive pediatric renal neoplasm. Am J Surg Pathol; 2000 Jul;24(7):917-26
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  • [Title] Metanephric stromal tumor: report of 31 cases of a distinctive pediatric renal neoplasm.
  • We report 31 cases of a novel pediatric renal neoplasm, metanephric stromal tumor (MST).
  • Gross examination typically revealed a fibrous lesion centered in the renal medulla containing smooth-walled cysts (mean tumor size, 5.5 cm).
  • MST is histologically identical to the stromal component of metanephric adenofibroma (MAF, previously termed nephrogenic adenofibroma) and is an unencapsulated spindle cell lesion that entraps native kidney.
  • Characteristic histologic features of MST include alternating cellularity that imparts a nodular low-power appearance, onion-skin cuffing around entrapped renal tubules, heterologous differentiation (glia or cartilage), and vascular alterations (angiodysplasia of entrapped arterioles, juxtaglomerular cell hyperplasia in entrapped glomeruli).
  • Recognition of this entity can spare a child potentially toxic adjuvant chemotherapy that might be used for lesions in its differential diagnosis, specifically clear cell sarcoma of the kidney.
  • [MeSH-major] Kidney Neoplasms / pathology. Neoplasms, Germ Cell and Embryonal / pathology. Nephroma, Mesoblastic / pathology. Rhabdoid Tumor / pathology
  • [MeSH-minor] Biomarkers, Tumor / analysis. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Immunoenzyme Techniques. Infant. Infant, Newborn. Male. Stromal Cells / chemistry. Stromal Cells / pathology. Treatment Outcome

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  • (PMID = 10895814.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA-42386
  • [Publication-type] Journal Article; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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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.
  • 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.
  • 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.
  • [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. 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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  • [Title] Differential apoptosis gene expression in pediatric tumors of the kidney.
  • BACKGROUND/PURPOSE: Apoptosis, or programmed cell death, is essential in maintaining normal homeostasis of tissues.
  • 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).
  • WT-II expressed greater amounts of proapoptotic receptor mRNA than CCSK or RTK. (Fas, 17.0+/-2.7% v. 2.5+/-0.5% v. 3.3+/-0.9%; P<.02; DR5, 77.0+/-8.8% v. 13.5+/-0.5% v. 27.0+/-4.8; P<.001; TNF-R, 71.3+/-17.0% v. 21.0+/-4.0% v. 29.0+/-5.0%; P<.07, respectively).
  • Surprisingly, antiapoptotic factors (e.g., bcl-2 and bcl-xl) were not overexpressed in poor prognostic tumors (CCSK, RTK) compared with those with good prognosis (WT).
  • Expression of TRAIL (a ligand for DR4 and DR5) was significantly lower in CCSK and RTK than in normal kidney (9.5+/-1.5% v. 56.1+/-10.1%; P = .01).
  • [MeSH-major] Apoptosis / genetics. Gene Expression. Kidney Neoplasms / pathology
  • [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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25. Tournade MF, Com-Nougué C, de Kraker J, Ludwig R, Rey A, Burgers JM, Sandstedt B, Godzinski J, Carli M, Potter R, Zucker JM, International Society of Pediatric Oncology Nephroblastoma Trial and Study Committee: Optimal duration of preoperative therapy in unilateral and nonmetastatic Wilms' tumor in children older than 6 months: results of the Ninth International Society of Pediatric Oncology Wilms' Tumor Trial and Study. J Clin Oncol; 2001 Jan 15;19(2):488-500
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  • [Title] Optimal duration of preoperative therapy in unilateral and nonmetastatic Wilms' tumor in children older than 6 months: results of the Ninth International Society of Pediatric Oncology Wilms' Tumor Trial and Study.
  • PURPOSE: To determine the optimal duration of preoperative chemotherapy to further increase the proportion of stage I tumors by comparison of two regimens in the treatment of patients older than 6 months who have unilateral Wilms' tumor.
  • PATIENTS AND METHODS: Eligible patients (n = 382) initially received four weekly doses of vincristine (VCR) and two courses of actinomycin D (AMD) and were randomized either to be operated on (4-week group [n = 193]) or to receive 4 more weeks of the same chemotherapy regimen (8-week group [n = 189]).
  • After surgery, patients were assigned according to tumor stage and histology to four different treatment groups: stage I and favorable histology (n = 5) were to have no further treatment (NFT); stage I and standard histology or anaplasia (n = 244), VCR and AMD for 17 weeks (AV); stages II and III and favorable or standard histology, VCR, AMD, and an anthracycline for 27 weeks (AVE) with no abdominal radiotherapy for stage II N0 disease (n = 75) or with a 15-Gy dose of abdominal irradiation (RTH) in case of stages IIN1 and III (n = 56).
  • Anaplastic tumors staged higher than I or clear-cell sarcoma of the kidney (14), AMD, VCR, an anthracycline, and ifosfamide for 36 weeks (DEVI).
  • RESULTS: No advantage was found in favor of prolonged preoperative treatment.
  • Two-year EFS and 5-year OS rates, respectively, of the different treatment groups were as follows: NFT, 100% for both EFS and OS; AV, 88% and 93%; AVE, 84% and 88%; AVE RTH, 71% and 85%; and DEVI, 71% and 71%.
  • CONCLUSION: The 4-week schedule pre-nephrectomy chemotherapy regimen should be considered the standard treatment.
  • Clinical trials should continue to improve the cure rate of high-risk patients and the quality of life of children with a more favorable prognosis.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Wilms Tumor / drug therapy
  • [MeSH-minor] Adolescent. Antibiotics, Antineoplastic / administration & dosage. Chemotherapy, Adjuvant. Child. Child, Preschool. Dactinomycin / administration & dosage. Drug Administration Schedule. Humans. Infant. Neoplasm Staging. Nephrectomy. Survival Analysis. Vincristine / administration & dosage

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  • (PMID = 11208843.001).
  • [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] Clinical Trial; Journal Article; Randomized Controlled Trial; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine
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26. Grundy RG, Hutton C, Middleton H, Imeson J, Pritchard J, Kelsey A, Marsden HB, Vujanic GM, Taylor RE, United Kingdom Children's Cancer Study Group: Outcome of patients with stage III or inoperable WT treated on the second United Kingdom WT protocol (UKWT2); a United Kingdom Children's Cancer Study Group (UKCCSG) study. Pediatr Blood Cancer; 2004 Apr;42(4):311-9
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  • PROCEDURE: The second UK WT trial (UKWT2) ran between July 1986 and September 1991 accruing 448 patients.
  • One hundred and six patients were diagnosed and treated for stage III disease.
  • Six had clear cell sarcoma of the kidney (CCSK) and seven had rhabdoid tumours of the kidney (RTK) and are analysed separately.
  • Seventy-five received standard chemotherapy and abdominal radiotherapy according to protocol.
  • Seventeen had stage III disease at immediate nephrectomy, but radiotherapy was omitted by physician choice.
  • Thirty-three patients had inoperable disease at diagnosis and received pre-nephrectomy chemotherapy.
  • For children with stage III disease in whom RT was omitted the OS was 82% (CI: 59-97) and for inoperable disease 94% (CI: 78-98).
  • The overall and event-free survival (EFS) of children with stage III CCSK was 100% and was achieved with the majority of patients not receiving radiotherapy (CI: 48-100).
  • CONCLUSIONS: The outcome for stage III FH disease was similar to that reported for UKWT1 and NWTS-3.
  • The combination of abdominal RT together with 3-drug chemotherapy achieves a high abdominal tumour control rate.
  • The high cure rates for children in this trial with 'inoperable disease' suggests that treatment should be modified according to their post-chemotherapy stage in order to avoid over-treatment.
  • The high OS for stage III CCSK on this protocol suggests that treatment duration could be curtailed and the role of RT reviewed, though the numbers are small.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Wilms Tumor / drug therapy
  • [MeSH-minor] Adolescent. Child. Dactinomycin / therapeutic use. Doxorubicin / therapeutic use. Great Britain. Humans. Radiotherapy, Adjuvant. Sarcoma, Clear Cell / drug therapy. Sarcoma, Clear Cell / mortality. Sarcoma, Clear Cell / radiotherapy. Survival Analysis. Treatment Outcome. Vincristine / therapeutic use

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  • [Copyright] Copyright 2003 Wiley-Liss, Inc.
  • (PMID = 14966826.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin
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27. Press JZ, Kenyon JA, Xue H, Miller MA, De Luca A, Miller DM, Huntsman DG, Gilks CB, McAlpine JN, Wang YZ: Xenografts of primary human gynecological tumors grown under the renal capsule of NOD/SCID mice show genetic stability during serial transplantation and respond to cytotoxic chemotherapy. Gynecol Oncol; 2008 Aug;110(2):256-64
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  • [Title] Xenografts of primary human gynecological tumors grown under the renal capsule of NOD/SCID mice show genetic stability during serial transplantation and respond to cytotoxic chemotherapy.
  • OBJECTIVES: Human cancer tissue xenograft models may provide a more accurate reflection of tumor biology than cell lines.
  • The response to conventional chemotherapy and novel molecular targeted chemotherapy is assessed in one of the transplantable xenograft lines.
  • METHODS: Fresh tumor was transplanted beneath the renal capsule of NOD/SCID mice.
  • Transplantable tumor lines were derived from 5 tumors (4 ovarian carcinomas and 1 uterine sarcoma), and serially transplanted for 2-6 generations.
  • RESULTS: Unsupervised hierarchical cluster analysis applied to a 287 feature CGH array demonstrated a low degree of intratumoral genetic variation in 4/5 cases, with greater degree of variation in the fifth case (clear cell ovarian carcinoma derived from an omental sample).
  • BRCA mutation analysis identified germline BRCA1 mutation for further testing and this xenograft showed a significant response to carboplatin/paclitaxel chemotherapy, including a decrease in tumor volume and proliferation but did not demonstrate a response to the poly (ADP-ribose) polymerase-1 inhibitor PJ34.
  • CONCLUSIONS: Xenografts derived from gynecologic tumors can be serially transplanted and grown under renal capsule of NOD/SCID mice with minimal genetic change.
  • This model may be used to study progression of tumors, identify therapeutic targets, and test treatment modalities in tumors with well-characterized abnormalities in genes of fundamental importance in ovarian carcinogenesis, such as loss of BRCA1.
  • [MeSH-minor] Animals. Antineoplastic Combined Chemotherapy Protocols / pharmacology. Base Sequence. Carboplatin / administration & dosage. Exons. Female. Genes, BRCA1. Genes, BRCA2. Germ-Line Mutation. Humans. Immunohistochemistry. Kidney / surgery. Mice. Mice, Inbred NOD. Mice, SCID. Molecular Sequence Data. Nucleic Acid Hybridization. Paclitaxel / administration & dosage. Poly(ADP-ribose) Polymerase Inhibitors. Xenograft Model Antitumor Assays

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  • (PMID = 18547621.001).
  • [ISSN] 1095-6859
  • [Journal-full-title] Gynecologic oncology
  • [ISO-abbreviation] Gynecol. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Poly(ADP-ribose) Polymerase Inhibitors; BG3F62OND5 / Carboplatin; EC 2.4.2.30 / PARP1 protein, human; P88XT4IS4D / Paclitaxel
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28. 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.
  • 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).
  • In one case of CCSK only histology of the metastases disclosed the correct diagnosis.
  • 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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29. Reinhard H, Semler O, Bürger D, Bode U, Flentje M, Göbel U, Gutjahr P, Leuschner I, Maass E, Niggli F, Scheel-Walter HG, Stöckle M, Thüroff JW, Tröger J, Weirich A, von Schweinitz D, Zoubek A, Graf N: Results of the SIOP 93-01/GPOH trial and study for the treatment of patients with unilateral nonmetastatic Wilms Tumor. Klin Padiatr; 2004 May-Jun;216(3):132-40
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  • [Title] Results of the SIOP 93-01/GPOH trial and study for the treatment of patients with unilateral nonmetastatic Wilms Tumor.
  • BACKGROUND: The treatment of Wilms Tumor is integrated into clinical trials since the 1970's.
  • In contrast to the National Wilms Tumor Study Group (NWTSG) the SIOP trials and studies largely focus on the issue of preoperative therapy to facilitate surgery of a shrunken tumor and to treat metastasis as early as possible.
  • PATIENTS AND METHODS: In the SIOP 93-01/GPOH trial and study 1 020 patients with a newly diagnosed renal tumor were registered.
  • 847 of them had a histological proven Wilms Tumor, of whom 637 were unilateral localized, and 173 tumors had an other histology [40 congenital mesoblastic nephroma (CMN), 51 clear cell sarcoma (CCSK), 24 rhabdoid tumor (RTK) and 58 other tumors].
  • Preoperative chemotherapy in benign tumors was given to 1.3 % of the patients.
  • The main objective of the trial was the randomized question, if the postoperative two drug chemotherapy for stage I in intermediate risk or anaplasia can be reduced from conventional 3 courses to an experimental 1 course without loss of efficacy.
  • RESULTS: 519 patients with unilateral nonmetastatic Wilms did receive preoperative chemotherapy.
  • Tumor volume was measured in 487 patients before and in 402 after preoperative chemotherapy.
  • Randomisation was done in 43.7 % for stage I patients and there was no difference in EFS for both treatment arms (90 versus 91 %).
  • The tumor volume after chemotherapy is a prognostic factor for intermediate risk tumors with the exception of epithelial and stromal predominant tumors.
  • These two subtypes often present as large tumors, they do not shrink during preoperative chemotherapy but they still have an excellent prognosis.
  • On the other hand the prognosis of patients with blastemal predominant subtype after preoperative chemotherapy is worse than in any other patient group of intermediate risk tumors.
  • There are less blastemal predominant tumors compared to primary surgery, but they are chemotherapeutic resistant selected by the preoperative chemotherapy.
  • The post-operative chemotherapy in stage I can be reduced to 4 weeks without worsening treatment outcome.
  • The reduction of the tumor volume could be identified as a helpful marker for stratification of post-operative treatment.
  • Post-chemotherapy blastemal predominant subtype of Wilms tumor has to be classified as high risk tumor.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Neoadjuvant Therapy. Wilms Tumor / drug therapy
  • [MeSH-minor] Adolescent. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Female. Follow-Up Studies. Humans. Infant. Male. Neoplasm Staging. Nephrectomy. Nephroma, Mesoblastic / drug therapy. Nephroma, Mesoblastic / mortality. Nephroma, Mesoblastic / pathology. Nephroma, Mesoblastic / surgery. Prognosis. Rhabdoid Tumor / drug therapy. Rhabdoid Tumor / mortality. Rhabdoid Tumor / pathology. Rhabdoid Tumor / surgery. Sarcoma, Clear Cell / drug therapy. Sarcoma, Clear Cell / mortality. Sarcoma, Clear Cell / pathology. Sarcoma, Clear Cell / surgery

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  • (PMID = 15175957.001).
  • [ISSN] 0300-8630
  • [Journal-full-title] Klinische Pädiatrie
  • [ISO-abbreviation] Klin Padiatr
  • [Language] eng
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
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30. Wu HY, Snyder HM 3rd, D'Angio GJ: Wilms' tumor management. Curr Opin Urol; 2005 Jul;15(4):273-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • PURPOSE OF REVIEW: The management of Wilms' tumor continues to evolve with two different approaches being taken by the National Wilms Tumor Study in North America and the International Society of Pediatric Oncology in Europe in regards to preoperative chemotherapy.
  • Percutaneous biopsy for tissue diagnosis is quite accurate, but there are concerning complications with its use.
  • The surgical complication rate was similar between the National Wilms Tumor Study and the International Society of Pediatric Oncology, but intraoperative tumor spill was higher in the North American trials.
  • A longer course of chemotherapy (including doxorubicin) for clear cell sarcoma improves recurrence-free survival.
  • Patients with Wilms' tumor, aniridia, major genitourinary malformations, and mental retardation, the WAGR syndrome, have a 50% chance of unexplained end-stage renal disease 20 years after treatment.
  • SUMMARY: Less aggressive means of diagnosis and treatment for Wilms' tumor are continuing to achieve very good cure rates while lowering long term morbidity for low risk patients.
  • High-risk patients with unfavorable histology or the WAGR syndrome benefit from more intensive treatment and long-term follow-up.
  • [MeSH-major] Kidney Neoplasms / therapy. Wilms Tumor / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Child. Combined Modality Therapy. Doxorubicin / administration & dosage. Humans. Kidney Failure, Chronic / etiology. Nephrectomy. Vincristine / administration & dosage

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  • (PMID = 15928519.001).
  • [ISSN] 0963-0643
  • [Journal-full-title] Current opinion in urology
  • [ISO-abbreviation] Curr Opin Urol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin
  • [Number-of-references] 19
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31. Skotnicka-Klonowicz G, Bagłaj M, Sawicz-Birkowska K, Balcerska A, Dembowska B, Szymik-Kantorowicz S, Madziara W: [Nephroblastoma in children aged less than 6 months at diagnosis]. Med Wieku Rozwoj; 2003 Jul-Sep;7(3):347-57
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  • [Title] [Nephroblastoma in children aged less than 6 months at diagnosis].
  • We present the results of treatment of kidney tumours in newborns and infants aged less than 6 months, in the years 1993-2000, from the Nephroblastoma Committee of the Polish Paediatric Group of Solid Tumours (PPGGL).
  • We have analysed the diagnostic and treatment results in the group of 31 children aged 0 to 6 months.
  • Among 450 children registered between 1993 and 2000 by PPGGL and treated for kidney tumours, there were 31 (7.1%) newborns and infants aged below 6 months.
  • The accuracy of diagnosis based on imaging studies was 97%.
  • Only in one child the initial diagnosis of kidney tumour was not confirmed; cystic degeneration of kidney was finally established.
  • In 10 cases histopathology confirmed mesoblastic nephroma, in 19 cases nephroblastoma and in 2 cases sarcoma clarocellulare.
  • In 10 infants (32.2%) with nephroblastoma delayed surgery preceded by chemotherapy was performed.
  • Indications for initial preoperative chemotherapy comprised: tumour in a single kidney, tumour in a horseshoe kidney, preoperative diagnostic biopsy of the tumour and large tumour in neonates older than 3 months.
  • The tolerance of reduced chemotherapy by the infants was good. AS was 100%.
  • ESF for the 19 children registered for nephroblastoma between 1993 and 1996 for all stages of advancement and types of histology was 94.75%.
  • 2) The results of treatment of nephroblastoma in the youngest children (below 6 months of age) are the most favourable and represent world standards.3) Surgical complications in children operated primarily for nephroblastoma indicate the need of performing such operations in academic centres, specialised in newborn surgery.
  • 4) In infants with extensive kidney tumours older than 3 months, primarily considered as inoperative, individual induction chemotherapy should be taken into account.
  • [MeSH-major] Kidney Neoplasms / diagnosis. Kidney Neoplasms / therapy. Wilms Tumor / diagnosis. Wilms Tumor / therapy
  • [MeSH-minor] Disease-Free Survival. Female. Humans. Infant. Infant, Newborn. Male. Nephroma, Mesoblastic / diagnosis. Nephroma, Mesoblastic / therapy. Poland. Retrospective Studies. Sarcoma, Clear Cell / diagnosis. Sarcoma, Clear Cell / therapy. Survival Analysis

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  • (PMID = 14963342.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
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32. Vujanić GM, Kelsey A, Mitchell C, Shannon RS, Gornall P: The role of biopsy in the diagnosis of renal tumors of childhood: Results of the UKCCSG Wilms tumor study 3. Med Pediatr Oncol; 2003 Jan;40(1):18-22
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  • [Title] The role of biopsy in the diagnosis of renal tumors of childhood: Results of the UKCCSG Wilms tumor study 3.
  • BACKGROUND: The United Kingdom Children's Cancer Study Group (UKCCSG) Wilms Tumor Study 3 has adopted preoperative chemotherapy for Wilms tumors (WT), but required prechemotherapy biopsy for histologic diagnosis.
  • The aims of this review were to assess the usefulness and safety of prechemotherapy biopsy and to compare histologic features of WT before and after chemotherapy.
  • PROCEDURE: There were 286 eligible patients but only 241 biopsies and 226 nephrectomy case slides were submitted for panel review.
  • The presence of different histologic components of WT before and after chemo therapy was retrospectively assessed.
  • One child required emergency nephrectomy due to massive intratumoral bleeding, another had tumor rupture and subsequently died, and a third developed a needle track recurrence 8 months after the biopsy.
  • CONCLUSIONS: A number of renal tumors (12%) can have the correct histologic diagnosis made by PCNB.
  • Preoperative chemo therapy markedly decrease in the number of samples with preserved blastema.
  • Needle biopsy of any renal mass prior to initiation of chemotherapy is recommended.
  • [MeSH-major] Biopsy, Needle. Kidney Neoplasms / pathology. Wilms Tumor / pathology
  • [MeSH-minor] Carcinoma, Renal Cell / pathology. Child. Female. Great Britain. Humans. Male. Nephrectomy. Neuroblastoma / pathology. Predictive Value of Tests. Retrospective Studies. Rhabdoid Tumor / pathology. Sarcoma, Clear Cell / pathology. Sensitivity and Specificity. Surveys and Questionnaires

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  • [Copyright] Copyright 2003 Wiley-Liss, Inc.
  • (PMID = 12426681.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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33. Tröbs RB, Hänsel M, Friedrich T, Bennek J: A 23-year experience with malignant renal tumors in infancy and childhood. Eur J Pediatr Surg; 2001 Apr;11(2):92-8
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  • [Title] A 23-year experience with malignant renal tumors in infancy and childhood.
  • A retrospective analysis of 77 children treated between 1974 and 1996 was undertaken to evaluate morbidity and the evolution of therapy.
  • High-risk WT were diagnosed in 12 of 63 patients (19%) (NB with anaplasia 10, clear cell sarcoma 1, malignant rhabdoid tumor 1).
  • We observed 3 children of school age with renal carcinoma and one patient with an intrarenal neuroblastoma.
  • According to the SIOP/GPOH protocol in 1989, the regimen was switched from primary surgery to preoperative chemotherapy without biopsy in 1989 (11 pats.).
  • In 3 patients preoperative diagnosis by means of imaging failed.
  • During preoperative chemotherapy a venous occlusive disease of the liver occurred in 2 patients.
  • Preoperative chemotherapy led to an impressive tumor shrinkage in the majority of patients.
  • In our experience, reduction of tumor volume due to preoperative chemotherapy facilitates tumor removal by surgery and may prevent tumor spillage and the deleterious effects of radiation in young children.
  • Surgery without delay is necessary if the diagnosis is unclear or the tumor fails to respond to preoperative chemotherapy.
  • [MeSH-major] Kidney Neoplasms / surgery. Wilms Tumor / surgery
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Chemotherapy, Adjuvant. Child. Child, Preschool. Disease-Free Survival. Female. Humans. Infant. Male. Neoplasm Recurrence, Local / epidemiology. Neoplasm Staging. Prognosis. Retrospective Studies

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  • (PMID = 11371043.001).
  • [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] 0 / Antineoplastic Agents
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34. Ross H, Argani P: Xp11 translocation renal cell carcinoma. Pathology; 2010 Jun;42(4):369-73
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  • [Title] Xp11 translocation renal cell carcinoma.
  • Xp11 translocation renal cell carcinoma (RCC) is a group of neoplasms characterised by translocations involving a breakpoint at Xp11.2.
  • The resulting gene fusions involve the TFE3 transcription factor gene and multiple reported genes, including the same one (ASPL) found in the characteristic gene fusion of alveolar soft part sarcoma.
  • The only known risk factor for its development is prior exposure to chemotherapy.
  • The most distinctive histological pattern is a neoplasm with both clear cells and papillary architecture, often with abundant psammoma bodies.
  • Clinical outcome data are still premature at this time.
  • [MeSH-major] Carcinoma, Renal Cell / genetics. Kidney Neoplasms / genetics


35. Perlman EJ: Pediatric renal tumors: practical updates for the pathologist. Pediatr Dev Pathol; 2005 May-Jun;8(3):320-38
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  • [Title] Pediatric renal tumors: practical updates for the pathologist.
  • Pediatric renal tumors were targeted by the National Wilms Tumor Study Group for 4 decades with extraordinary success.
  • Within this historic context, this review provides a summary of the new Children's Oncology Group renal tumor protocols that will be opening in the very near future, focusing on their pathologic requirements.
  • All renal tumors must first be registered on the Renal Tumor Classification and Banking Protocol, followed by registration on 1 of 4 primary therapeutic protocols based on histology, stage, and molecular analysis.
  • Changes in staging criteria include classification of all tumor spillage as stage III, and requirement of regional lymph node evaluation for eligibility for stage I Wilms tumors (WTs) weighing less than 550 g in infants younger than 24 months and for stage I clear cell sarcoma.
  • Patients with unilateral favorable histology WT with loss of heterozygosity for chromosomes 1p and 16q will receive more aggressive chemotherapy at each stage.
  • Patients with bilateral WT and patients with diffuse hyperplastic perilobar nephroblastomatosis will be eligible for a novel therapeutic protocol requiring pathologic classification based on response of tumor to previous therapy.
  • Stage I anaplastic WT will be targeted with more aggressive chemotherapy than in the past.
  • For the first time, pediatric renal cell carcinoma will be eligible for a cooperative group protocol.
  • In conclusion, these new protocols bring considerable change in their overall organization, in eligibility, and in therapy.
  • [MeSH-major] Antineoplastic Protocols. Kidney Neoplasms / therapy

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  • (PMID = 16010493.001).
  • [ISSN] 1093-5266
  • [Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • [ISO-abbreviation] Pediatr. Dev. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 75
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36. 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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  • [Title] The survivin:fas ratio in pediatric renal tumors.
  • BACKGROUND/PURPOSE: Apoptosis factors inducing or preventing cell death may govern the behavior of certain 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.
  • Survivin is a novel inhibitor of apoptosis that is expressed in a cell cycle-dependent manner and is abundantly expressed in several tumors of unfavorable histology.
  • 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.
  • Follow-up data were obtained on patient outcomes, and antiapoptotic to proapoptotic ratios were calculated and correlated with clinical recurrence of disease.
  • RESULTS: Pediatric renal tumors express greater levels of both pro- and antiapoptotic factors than normal kidney.
  • 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).
  • 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.
  • [MeSH-major] Antigens, CD95 / metabolism. Kidney Neoplasms / metabolism. Microtubule-Associated Proteins. Proteins / metabolism. RNA, Messenger / metabolism
  • [MeSH-minor] Apoptosis. Blotting, Western. Child. Humans. Immunoenzyme Techniques. Inhibitor of Apoptosis Proteins. Neoplasm Proteins. Neoplasm Recurrence, Local / diagnosis. Predictive Value of Tests. Prognosis. Statistics, Nonparametric

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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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37. Varlet F, Stephan JL, Guye E, Allary R, Berger C, Lopez M: Laparoscopic radical nephrectomy for unilateral renal cancer in children. Surg Laparosc Endosc Percutan Tech; 2009 Apr;19(2):148-52
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  • [Title] Laparoscopic radical nephrectomy for unilateral renal cancer in children.
  • PURPOSE: At the present time, the standard approach for renal cancer in children is open surgery, and the role of laparoscopic approach remains to be defined.
  • We report our preliminary experience in the treatment by laparoscopic radical nephrectomy (LRN) for unilateral renal cancer in children.
  • METHODS: Five children, whose mean age was 4 years old, were operated for unilateral renal malignant tumors by laparoscopic approach in our unit from October 2005 to June 2007.
  • They were preoperatively treated with chemotherapy according to the International Society of Pediatric Oncology 2001 protocol: vincristine and actinomicyn D for 4 weeks.
  • The fifth case was a 10-year-old child, treated 8 years before with chemotherapy for a cerebellar vermis medulloblastoma history.
  • A percutaneous biopsy was performed preoperatively and the histology showed a juvenile renal-cell carcinoma.
  • The mean operative time was 90 minutes (60 to 117 min).
  • The histology was 3 unilateral Wilms tumor, 1 clear-cell sarcoma and 1 juvenile renal-cell carcinoma with t(X;17).
  • CONCLUSIONS: LRN for renal cancer in children is feasible after preoperative chemotherapy, with the same oncologic strategies as open surgery.
  • [MeSH-major] Kidney Neoplasms / surgery. Laparoscopy. Minimally Invasive Surgical Procedures. Nephrectomy / methods
  • [MeSH-minor] Age Factors. Antibiotics, Antineoplastic / therapeutic use. Antineoplastic Agents, Phytogenic / therapeutic use. Child. Child, Preschool. Dactinomycin / therapeutic use. Feasibility Studies. Female. Humans. Infant. Male. Vincristine / therapeutic use

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  • (PMID = 19390283.001).
  • [ISSN] 1534-4908
  • [Journal-full-title] Surgical laparoscopy, endoscopy & percutaneous techniques
  • [ISO-abbreviation] Surg Laparosc Endosc Percutan Tech
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Antineoplastic Agents, Phytogenic; 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine
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38. Shet T, Viswanathan S: The cytological diagnosis of paediatric renal tumours. J Clin Pathol; 2009 Nov;62(11):961-9
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  • [Title] The cytological diagnosis of paediatric renal tumours.
  • Fine needle aspiration cytology (FNAC) is used for preoperative diagnosis of paediatric renal tumours, especially in centres where preoperative chemotherapy is advocated in Wilms' tumour.
  • This review focuses on salient cytological features in specific paediatric renal tumours, the approach to resolving a differential diagnosis and the role of ancillary methods in diagnosis of paediatric renal tumours.
  • Crucial differential diagnoses include distinguishing: Wilms' tumour from benign tumours in the kidney like multicystic nephroma or congenital mesoblastic nephroma; aggressive non-Wilms' tumours of kidney like rhabdoid tumour of kidney; and Wilms' tumour from other paediatric round cell sarcomas like neuroblastoma, non-Hodgkin lymphoma etc.
  • An approach based on classifying smears according to their cellular patterns as triphasic, round cell, spindle cell or epithelioid cell type assists in classifying paediatric renal tumours on cytology.
  • Immunocytochemistry for WT1, cytokeratin, synaptophysin, leucocyte common antigen and MIC2 will aid in evaluating round cell tumours in the renal region, while WT1, bcl2, vimentin and desmin will be useful for spindle cell tumours in that region.
  • A checklist of common tumours in a particular age group, relevant clinical information, awareness of distinctive and overlapping cytological features, and appropriate use of immunocytochemistry with cytogenetics go a long way in ensuring an accurate cytological diagnosis.
  • Used judiciously, FNAC is as effective a tool as a core biopsy for preoperative diagnosis of paediatric renal tumours, and with experience a 92% accuracy rate can be achieved.
  • [MeSH-major] Kidney Neoplasms / pathology. Wilms Tumor / pathology
  • [MeSH-minor] Adolescent. Age Distribution. Biopsy, Fine-Needle / methods. Carcinoma, Renal Cell / pathology. Child. Child, Preschool. Diagnosis, Differential. Humans. Infant. Infant, Newborn. Nephroma, Mesoblastic / pathology. Rhabdoid Tumor / pathology. Sarcoma, Clear Cell / pathology. Sarcoma, Ewing / pathology. Young Adult

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  • (PMID = 19700411.001).
  • [ISSN] 1472-4146
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 36
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39. Miniati D, Gay AN, Parks KV, Naik-Mathuria BJ, Hicks J, Nuchtern JG, Cass DL, Olutoye OO: Imaging accuracy and incidence of Wilms' and non-Wilms' renal tumors in children. J Pediatr Surg; 2008 Jul;43(7):1301-7
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  • [Title] Imaging accuracy and incidence of Wilms' and non-Wilms' renal tumors in children.
  • PURPOSE: The purpose of this study is to determine the actual incidence, age distribution, and preoperative imaging accuracy of non-Wilms' tumors (nWT) in children with renal masses.
  • METHODS: Pathologic reports from all tumor nephrectomies or open renal biopsies performed at a single institution from September 1999 to June 2005 were analyzed.
  • The nWT group included congenital mesoblastic nephroma (5), clear cell sarcoma (4), neuroblastoma (4), renal cell carcinoma (4), lymphoma (2), angiomyolipoma (2), teratoma (1), hemangioma (1), and renal epithelial tumor (1).
  • Sensitivity, specificity, positive predictive value, and negative predictive value for computed tomography (CT) determining a diagnosis of WT were 0.92, 0.55, 0.84, and 0.73, respectively.
  • The CT reports explicitly stated a potential diagnosis in 89% of cases, with a diagnostic accuracy of 82%.
  • CONCLUSIONS: Non-Wilms' tumors may represent a significant proportion of renal tumors in children, especially in children aged less than 6 months or greater than 12 years.
  • These data have significant implications for parental counseling, surgical plan, and the choice of neoadjuvant chemotherapy and argue in favor of obtaining a tissue diagnosis before instituting therapy.
  • [MeSH-major] Kidney / pathology. Kidney Neoplasms / diagnosis. Wilms Tumor / diagnosis

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  • (PMID = 18639686.001).
  • [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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40. Luo CC, Lin JN, Jaing TH, Yang CP, Hsueh C: Malignant rhabdoid tumour of the kidney occurring simultaneously with a brain tumour: a report of two cases and review of the literature. Eur J Pediatr; 2002 Jun;161(6):340-2
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  • [Title] Malignant rhabdoid tumour of the kidney occurring simultaneously with a brain tumour: a report of two cases and review of the literature.
  • Malignant rhabdoid tumour of the kidney (MRTK), an uncommon aggressive neoplasm of children, is now recognised as a separate entity from Wilms' tumour with distinct clinical and pathological features.
  • We report two cases, aged 2 and 6 months, of MRTK occurring concurrently with a brain tumour.
  • Both patients expired 2 and 6 months later despite receiving aggressive post-operative chemotherapy and radiotherapy.
  • CONCLUSION: malignant rhabdoid tumour of the kidney is an uncommon neoplasm of early childhood with a poor prognosis.
  • Due to its significant association with brain tumours or early brain metastases, concurrent brain computer tomographic examination is essential for all patients with this disease.
  • [MeSH-major] Brain Neoplasms / pathology. Kidney Neoplasms / pathology. Neoplasms, Multiple Primary. Rhabdoid Tumor / pathology. Sarcoma, Clear Cell / pathology
  • [MeSH-minor] Humans. Infant. Male. Tomography, X-Ray Computed

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  • (PMID = 12029454.001).
  • [ISSN] 0340-6199
  • [Journal-full-title] European journal of pediatrics
  • [ISO-abbreviation] Eur. J. Pediatr.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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41. Rebhandl W, Handisurya A, Memaran N, Felberbauer FX, Aberle J, Paya K, Strobl B, Horcher E: Expression of cytokeratin-18-related tissue polypeptide-specific (TPS) antigen in Wilms tumor. Med Pediatr Oncol; 2001 Oct;37(4):357-64
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  • [Title] Expression of cytokeratin-18-related tissue polypeptide-specific (TPS) antigen in Wilms tumor.
  • BACKGROUND: So far, there is no approved tumour marker for diagnosis or follow-up in Wilms tumour (WT).
  • Tissue polypeptide-specific antigen (TPS), a cytokeratin 18 proteolytic fragment, has been suggested to be of value in the clinical management of WT patients.
  • Cytokeratin 18 fragments are an early indicator of apoptosis and cytokeratin 18 might influence tumour cell behaviour.
  • We investigated TPS expression in specimens of WT and other paediatric renal malignancies PROCEDURE: Immunoreactivity of WT sections (n = 9), clear cell sarcomas (CCSK, n = 3), and a renal cell carcinoma (RCC), and two pediatric kidney tumour cell lines (WT: SK-NEP-1 and rhabdoid tumour of the kidney: G-401) were investigated using the monoclonal antibody M3.
  • Cell culture supernatants were evaluated for TPS release.
  • Serum TPS was measured in five patients at diagnosis, during chemotherapy and after surgical resection.
  • The supernatant of G-401 but not of SK-NEP-1 showed a time- and cell number-dependent increase of TPS release.
  • CONCLUSIONS: This is the first study demonstrating the synthesis and release of TPS by WTs and other paediatric renal malignancies.
  • [MeSH-major] Biomarkers, Tumor / analysis. Keratins / metabolism. Kidney Neoplasms / metabolism. Peptides / analysis. RNA, Messenger / analysis. Wilms Tumor / metabolism
  • [MeSH-minor] Adolescent. Base Sequence. Biopsy, Needle. Blotting, Western. Cell Line. Child. Child, Preschool. Female. Fluorescent Antibody Technique. Humans. Immunohistochemistry. Infant. Infant, Newborn. Male. Molecular Sequence Data. Prognosis. Reverse Transcriptase Polymerase Chain Reaction. Sensitivity and Specificity

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  • [Copyright] Copyright 2001 Wiley-Liss, Inc.
  • (PMID = 11568899.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Peptides; 0 / RNA, Messenger; 0 / tissue polypeptide specific antigen; 68238-35-7 / Keratins
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42. Jimenez RE, Folpe AL, Lapham RL, Ro JY, O'Shea PA, Weiss SW, Amin MB: Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases. Am J Surg Pathol; 2002 Mar;26(3):320-7
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  • [Title] Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases.
  • Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is an extraordinarily rare primary tumor in the kidney and can be mistaken for a variety of other round cell tumors, including blastema-predominant Wilms' tumor (WT).
  • Immunohistochemistry for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET.
  • No study has examined FLI-1 or WT-1 expression in renal ES/PNET.
  • The clinicopathologic features of 11 renal ES/PNET were studied along with immunohistochemistry for cytokeratin, desmin, CD99, FLI-1, and WT-1.
  • Immunohistochemical results on the renal ES/PNET were cytokeratin (2/8 focal), desmin (0/9), CD99 (8/8), FLI-1 (5/8), and WT-1 (0/8).
  • Follow-up on 8 cases (mean, 28 mo; range, 6-64 mo) showed 4 lung and pleural metastases, 1 bone metastasis, liver metastasis, 2 local recurrences, and 5 deaths from disease (median time to death, 16.8 mo).
  • No case had distant metastatic disease at presentation.
  • Adjuvant therapy included chemotherapy (8 cases), radiation (3 cases), and bone marrow transplantation (1 case).
  • Our study affirms a unique proclivity of renal ES/PNET for young adults and that it is a highly aggressive neoplasm, with rapid death in many cases, usually after the development of treatment-resistant lung metastases.
  • These tumors must be distinguished from blastema-predominant WT and other primitive renal tumors that require different therapy.
  • FLI-1 and WT-1 immunohistochemistry may be valuable in this differential diagnosis, given the known immunophenotypic overlap between ES/PNET and blastema-predominant WT with regard to CD99, cytokeratin, and desmin.
  • The accurate distinction between these two entities has clear prognostic and therapeutic implications.
  • [MeSH-major] Kidney Neoplasms / pathology. Neuroectodermal Tumors, Primitive / pathology. Proto-Oncogene Proteins. Sarcoma, Ewing / pathology
  • [MeSH-minor] Adult. Aged. Antigens, CD / analysis. Cell Adhesion Molecules / analysis. Child. Combined Modality Therapy. DNA-Binding Proteins / analysis. Desmin / analysis. Diagnosis, Differential. Female. Genes, Tumor Suppressor. Humans. Immunohistochemistry. Keratins / analysis. Male. Middle Aged. Neoplasm Metastasis. Proto-Oncogene Protein c-fli-1. Trans-Activators / analysis. WT1 Proteins / analysis. Wilms Tumor / chemistry. Wilms Tumor / genetics. Wilms Tumor / pathology

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  • [CommentIn] Am J Surg Pathol. 2003 Aug;27(8):1177 [12883255.001]
  • (PMID = 11859203.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / DNA-Binding Proteins; 0 / Desmin; 0 / Proto-Oncogene Protein c-fli-1; 0 / Proto-Oncogene Proteins; 0 / Trans-Activators; 0 / WT1 Proteins; 68238-35-7 / Keratins
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43. D'Antiga L, Baker A, Pritchard J, Pryor D, Mieli-Vergani G: Veno-occlusive disease with multi-organ involvement following actinomycin-D. Eur J Cancer; 2001 Jun;37(9):1141-8
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  • [Title] Veno-occlusive disease with multi-organ involvement following actinomycin-D.
  • Actinomycin-D (Act-D) is a rare cause of veno-occlusive disease (VOD).
  • Between 1993 and 1998, we managed 6 patients, all male, median age 19 months (range 6-48 months) who received Act-D for Wilms' tumour (n=4), clear cell sarcoma (n=1) or rhabdomyosarcoma (n=1).
  • VOD presented with a median platelet count of 12 x 10(9)/l, INR 3.8, fibrinogen 16 mg/l, fibrinogen degradation products (FDPs) > or =80 microg/l, aspartate aminotransferase (AST) 6922 IU/l, bilirubin 47 micromol/l.
  • All six children developed encephalopathy, hepatomegaly, ascites, reversed portal flow and renal impairment.
  • The treatment was supportive.
  • Severe Adult Respiratory Distress Syndrome developed in 3 patients, all of whom died.
  • Intravascular coagulopathy precedes and characterises severe VOD during Act-D treatment.
  • [MeSH-minor] Child, Preschool. Constriction, Pathologic. Humans. Infant. Kidney Neoplasms / drug therapy. Male. Rhabdomyosarcoma / drug therapy. Testicular Neoplasms / drug therapy. Wilms Tumor / drug therapy

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  • (PMID = 11378345.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 1CC1JFE158 / Dactinomycin
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44. Porta C, Zimatore M, Imarisio I, Natalizi A, Sartore-Bianchi A, Danova M, Riccardi A: Gemcitabine and oxaliplatin in the treatment of patients with immunotherapy-resistant advanced renal cell carcinoma: final results of a single-institution Phase II study. Cancer; 2004 May 15;100(10):2132-8
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  • [Title] Gemcitabine and oxaliplatin in the treatment of patients with immunotherapy-resistant advanced renal cell carcinoma: final results of a single-institution Phase II study.
  • BACKGROUND: Currently, there is no standard treatment for patients with advanced renal cell carcinoma (RCC) who do not experience a response to first-line immunotherapy.
  • METHODS: Forty-two patients with RCC who had progressive disease following immunotherapy received gemcitabine (1000 mg/m2 intravenously on Days 1 and 8 every 21 days) and L-OHP (90 mg/m2 intravenously on Day 1 every 21 days) for a minimum of 2 cycles before responses were evaluated.
  • Responses to treatment and toxicity were recorded according to the Response Evaluation Criteria in Solid Tumors and the National Cancer Institute Common Toxicity Criteria, respectively.
  • RESULTS: No complete responses were recorded; however, 6 patients experienced a partial response (14.28%; 95% confidence interval, 5.43-28.5%), 11 patients (26.19%) had temporary stable disease as a best response, and the remaining 25 patients (59.52%) experienced progression despite receiving treatment.
  • The median time to disease progression was 2.5 months (mean, 3.86 months; range, 1.5-11.0 months), whereas the median overall survival was 9.5 months (mean, 10.46 months; range, 4.0-22.5 months).
  • With regard to toxicity, treatment generally was well tolerated, with only one episode of Grade 4 toxicity and expected episodes of Grade 3 toxicity, including myelosuppression and neuropathy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Carcinoma, Renal Cell / drug therapy. Deoxycytidine / analogs & derivatives. Drug Resistance, Neoplasm. Immunotherapy. Kidney Neoplasms / drug therapy
  • [MeSH-minor] Adenocarcinoma, Clear Cell / drug therapy. Adenocarcinoma, Clear Cell / pathology. Aged. Carcinoma, Papillary / drug therapy. Carcinoma, Papillary / pathology. Female. Humans. Male. Middle Aged. Organoplatinum Compounds / administration & dosage. Prognosis. Salvage Therapy. Sarcoma / pathology. Survival Rate. Therapeutics. Treatment Outcome

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  • [Copyright] Copyright 2004 American Cancer Society.
  • (PMID = 15139055.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase II; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Organoplatinum Compounds; 04ZR38536J / oxaliplatin; 0W860991D6 / Deoxycytidine; B76N6SBZ8R / gemcitabine
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45. Lekili M, Muezzinoglu T, Nese N, Temeltas G: Sorafenib in metastatic renal cell carcinoma with sarcomatoid differentiation. J Chin Med Assoc; 2010 May;73(5):262-4
Hazardous Substances Data Bank. NICOTINAMIDE .

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  • [Title] Sorafenib in metastatic renal cell carcinoma with sarcomatoid differentiation.
  • Targeted therapy in the management of metastatic renal cell cancer has been recently introduced to urology practice.
  • The drugs used for management are used in a very limited number of patients and only for clear cell histology.
  • We present a case where we administered sorafenib, a multikinase inhibitor of tumor-cell proliferation and angiogenesis, to a patient with metastatic renal cell carcinoma of clear cell histology.
  • This case report might provide evidence that antiangiogenic agents may be active in any histological type of renal cell carcinoma.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Benzenesulfonates / therapeutic use. Carcinoma, Renal Cell / drug therapy. Kidney Neoplasms / drug therapy. Pyridines / therapeutic use. Sarcoma / pathology

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  • [Copyright] Copyright 2010 Elsevier. Published by Elsevier B.V. All rights reserved.
  • (PMID = 20685594.001).
  • [ISSN] 1728-7731
  • [Journal-full-title] Journal of the Chinese Medical Association : JCMA
  • [ISO-abbreviation] J Chin Med Assoc
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China (Republic : 1949- )
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzenesulfonates; 0 / Phenylurea Compounds; 0 / Pyridines; 25X51I8RD4 / Niacinamide; 9ZOQ3TZI87 / sorafenib
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