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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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  • [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.
  • Clinical responses to EGFR inhibitors have been found to be highly dependent on the presence of activating mutations, whereas gene amplification, downstream activation of Akt, and abnormalities in PTEN are also reported predictive factors.
  • 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.
  • In studying the CCSKs in more detail, we identified gene amplification in 1 of 12 (8.3%) cases and a somatic T790M EGFR mutation in a further case.
  • Downstream pathway activation, as assayed by phosphorylated Akt expression, was observed in 8 of 12 (66.7%) cases.
  • 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;.
  • 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. 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.
  • On microscopic examination of initial 17 sections, the tumor consisted of pleomorphic giant cells and spindle neoplastic cells.
  • There was no carcinomatous component.
  • Immunohistochemically, the neoplastic cells were negative for keratin and epithelial membrane antigen but positive for vimentin.
  • The giant cells were also scatteringly, weakly positive for myoglobin.
  • 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.
  • [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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5. 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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6. 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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7. 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.
  • Tumor cells showed positive vimentin staining.
  • 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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8. 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
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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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9. 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.
  • 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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10. 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.
  • 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.
  • 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.
  • 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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11. 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.
  • 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.
  • Postoperative chemotherapy and radiotherapy (21 600-30 600 cGy) was done in 10 cases.
  • CONCLUSION: CCSK remains the pediatric renal tumor most frequently misdiagnosed.
  • Its aggressiveness and its ability to give bone metastases need to recognize early this diagnosis for an adapted treatment.
  • [MeSH-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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12. 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.
  • 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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13. 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.
  • [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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14. 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.
  • [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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15. 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.
  • The most sensitive and specific immunohistochemical markers for these neoplasms are TFE3 protein and cathepsin-K.
  • Clinical outcome data are still premature at this time.
  • [MeSH-major] Carcinoma, Renal Cell / genetics. Kidney Neoplasms / genetics
  • [MeSH-minor] Chromosomes, Human, Pair 11 / genetics. Chromosomes, Human, X / genetics. Humans. Immunohistochemistry. Neoplasm Proteins / genetics. Neoplasm Proteins / metabolism. Translocation, Genetic


16. 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.
  • 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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17. 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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18. 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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19. 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).
  • In addition, gene copy number was investigated by chromogenic in situ hybridisation (CISH), and overexpressing tumours were sequenced for KIT mutations in exons 9, 11, 13 and 17.
  • 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).
  • No cases exhibited gene amplification or mutation.
  • 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.
  • CCSKs are associated with an increased expression of KIT, however, in the absence of gene amplification and/or activating mutation.
  • 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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20. 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.
  • 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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21. 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.
  • A long follow-up and more cases are necessary to evaluate and compare the results of laparoscopic approach with the open procedures.
  • [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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22. 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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23. 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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24. 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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25. 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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