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1. Madigan CE, Armenian SH, Malogolowkin MH, Mascarenhas L: Extracranial malignant rhabdoid tumors in childhood: the Childrens Hospital Los Angeles experience. Cancer; 2007 Nov 1;110(9):2061-6

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  • [Title] Extracranial malignant rhabdoid tumors in childhood: the Childrens Hospital Los Angeles experience.
  • BACKGROUND: Extracranial malignant rhabdoid tumor (MRT) is a rare, aggressive, pediatric malignancy with a historically poor outcome.
  • Recent efforts to intensify treatment for MRT have resulted in isolated reports of long-term survival.
  • Five patients had renal primary tumors, and 9 patients had extrarenal tumors.
  • Eleven of 14 patients had stage III or IV disease at diagnosis.
  • The time to disease progression was rapid (mean, 3.6 months).
  • There were no recurrences or deaths beyond 10 months after diagnosis.
  • All survivors received multimodal therapy, including both chemotherapy and surgery with or without radiation.
  • In addition, 2 patients received high-dose chemotherapy with hematopoietic stem cell rescue (HSCT) after neoadjuvant chemotherapy and local tumor control.
  • [MeSH-major] Rhabdoid Tumor / diagnosis. Rhabdoid Tumor / therapy
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Female. Hospitals. Humans. Infant. Infant, Newborn. Kaplan-Meier Estimate. Los Angeles. Male. Neoplasm Recurrence, Local / pathology. Prognosis. Radiotherapy. Retrospective Studies. Survival Analysis

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  • (PMID = 17828773.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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2. 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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3. Bourdeaut F, Dufour C, Delattre O: [Rhadboid tumours: hSNF/INI1 deficient cancers of early childhood with aggressive behaviour]. Bull Cancer; 2010 Jan;97(1):37-45
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  • [Transliterated title] Les tumeurs rhabdoïdes : des tumeurs hSNF5/INI1-déficientes précoces et agressives.
  • Rhabdoid tumours are rare aggressive tumours of infancy.
  • The definition classically relies on a characteristic morphology and the inactivation of the hSNF5/INI1 tumour suppressor gene.
  • This entity includes central nervous system tumours (ATRT), renal tumours (RTK) and soft-part tumours.
  • Their rarity and morphological pleomorphism make the diagnosis often challenging.
  • Deletions at the 22q11.2 locus and mutations in hSNF5/INI1 sequence must be investigated in order to confirm the diagnosis and to give insights on a presumable germline mutation.
  • The treatment is based on aggressive chemotherapy, surgery and irradiation.
  • Understanding the role of hSNF5/INI1 within the SWI-SNF complex for the epigenetic regulation of transcription might drive the future targeted therapies.
  • [MeSH-major] Chromosomal Proteins, Non-Histone / genetics. DNA-Binding Proteins / genetics. Gene Silencing. Neoplasm Proteins / genetics. Rhabdoid Tumor / genetics. Transcription Factors / genetics
  • [MeSH-minor] Central Nervous System Neoplasms / diagnosis. Central Nervous System Neoplasms / therapy. Chromosomes, Human, Pair 22 / genetics. Diagnosis, Differential. Genes, Tumor Suppressor. Germ-Line Mutation. Humans. Infant. Kidney Neoplasms / diagnosis. Kidney Neoplasms / therapy. Liver Neoplasms / diagnosis. Liver Neoplasms / therapy. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / therapy. Wilms Tumor / diagnosis. Wilms Tumor / therapy

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  • (PMID = 20080459.001).
  • [ISSN] 1769-6917
  • [Journal-full-title] Bulletin du cancer
  • [ISO-abbreviation] Bull Cancer
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / Neoplasm Proteins; 0 / SMARCB1 protein, human; 0 / Transcription Factors
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4. Chang CJ, Yeh ML, Chen CC: Rhabdoid tumor of the kidney with spontaneous rupture: case report and review of literature. Pediatr Surg Int; 2008 Apr;24(4):451-3
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  • [Title] Rhabdoid tumor of the kidney with spontaneous rupture: case report and review of literature.
  • Spontaneous rupture of the kidney is uncommon; here we report a case of spontaneous rupture of the kidney due to a rhabdoid tumor.
  • An 11-year-old boy presented with left flank pain and hematuria, was admitted to a hospital where he was found to have an abnormality of the left kidney on computed tomography (CT) scan.
  • He was referred to our department for further evaluation and treatment on the next day.
  • Spontaneous rupture of left renal tumor was suspected by a drop in hemoglobin level, hemoglobin decreased from 9.2 to 7.6 mg/dl within 72 h.
  • Urgent trans-abdominal exploration of the left kidney was performed.
  • During the operation, rupture of left renal tumor with massive bleeding was noted.
  • The surgical specimen contained a large peri-renal hemorrhage and tumor rupture into peri-pelvic soft tissue.
  • Histopathological diagnosis was rhabdoid tumor consisting of round nuclei, prominent nucleoli and eosinophic cytoplasm.
  • Two courses of adjuvant chemotherapy with actinomycin D, vincristine and epirubicin and radiotherapy (1,200 cGY) were performed post-operatively.
  • The patient died 5 months after operation due to metastasis of the tumor to the lung and intra-abdominal organs.
  • [MeSH-major] Kidney Neoplasms / complications. Rhabdoid Tumor / complications
  • [MeSH-minor] Child. Fatal Outcome. Hemorrhage / etiology. Humans. Kidney Diseases / etiology. Male. Rupture, Spontaneous / complications

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  • (PMID = 17492291.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 10
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5. Yamamoto M, Suzuki N, Hatakeyama N, Mizue N, Hori T, Kuroiwa Y, Hareyama M, Oda T, Kudoh T, Nui A, Matsuno T, Hirama T, Yokoyama S, Dome JS, Tsutsumi H: Treatment of stage IV malignant rhabdoid tumor of the kidney (MRTK) with ICE and VDCy: a case report. J Pediatr Hematol Oncol; 2006 May;28(5):286-9
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  • [Title] Treatment of stage IV malignant rhabdoid tumor of the kidney (MRTK) with ICE and VDCy: a case report.
  • The prognosis of stage IV malignant rhabdoid tumor of the kidney (MRTK) has been extremely poor.
  • However, a combination of ICE (ifosfamide, carboplatin, and etoposide) and VDCy (vincristine, doxorubicin, and cyclophosphamide) was recently reported to be effective for metastatic MRTK.
  • We describe a 21-month-old girl with stage IV MRTK who was successfully treated with ICE, VDCy, and radiotherapy.
  • She remained well, without recurrence, 24 months after diagnosis.
  • Alternating therapy with ICE and VDCy might become a standard regimen for stage IV MRTK, although further study is required to confirm its effectiveness.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Kidney Neoplasms / drug therapy. Rhabdoid Tumor / drug therapy

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  • (PMID = 16772877.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide; ICE protocol 1
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6. Alavi S, Rashidi A, Khatami AR, Arzanian MT: Rhabdoid tumor of the kidney presenting with hemiplegia: report of a case. Pediatr Hematol Oncol; 2007 Mar;24(2):123-8
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  • [Title] Rhabdoid tumor of the kidney presenting with hemiplegia: report of a case.
  • Rhabdoid tumor of the kidney (RTK) is a rare and highly malignant neoplasm of infancy, with a strong tendency for early metastasis to distant regions.
  • RTK is unique in its significant association with primary or metastatic brain tumors.
  • The authors report the first case of RTK presenting initially with hemiplegia.
  • The patient was found thereafter to have RTK concurrent with pulmonary metastases, a brain tumor, and a cerebral ischemic lesion.
  • Intensive chemotherapy consisting of carboplatin and etoposide alternating with cyclophosphamide was unsuccessful and the patient died 5 months later because of severe respiratory distress resulting from widespread pulmonary metastases.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Hemiplegia / complications. Kidney Neoplasms / pathology. Neoplasms, Multiple Primary / pathology. Rhabdoid Tumor / pathology
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Ischemia / drug therapy. Brain Ischemia / pathology. Fatal Outcome. Female. Humans. Infant. Lung Neoplasms / drug therapy. Lung Neoplasms / secondary. Magnetic Resonance Imaging. Tomography, X-Ray Computed

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  • (PMID = 17454778.001).
  • [ISSN] 1521-0669
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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7. Sahdev I, James-Herry A, Scimeca P, Parker R: Concordant rhabdoid tumor of the kidney in a set of identical twins with discordant outcomes. J Pediatr Hematol Oncol; 2003 Jun;25(6):491-4
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  • [Title] Concordant rhabdoid tumor of the kidney in a set of identical twins with discordant outcomes.
  • We report identical twin boys who each had stage IV rhabdoid tumor of the left kidney at the age of 5 months and 2 years, respectively.
  • The 5-month-old boy, despite receiving chemotherapy, died of progressive disease at the age of 12 months.
  • Following resection of the tumor, his twin brother was treated with 6 cycles of combination chemotherapy consisting of cisplatinum, doxorubicin, vincristine, cyclophosphamide, and actinomycin-D alternating with ifosfamide and etoposide.
  • High-dose chemotherapy followed by autologous stem cell transplant may be an effective front-line therapeutic approach for patients with metastatic rhabdoid tumor of the kidney.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Diseases in Twins. Kidney Neoplasms / pathology. Peripheral Blood Stem Cell Transplantation. Rhabdoid Tumor / pathology
  • [MeSH-minor] Child, Preschool. Combined Modality Therapy. Fatal Outcome. Humans. Infant. Male. Transplantation, Autologous. Treatment Outcome. Twins, Monozygotic

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  • (PMID = 12794530.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 19
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8. Koga Y, Matsuzaki A, Suminoe A, Hatano M, Saito Y, Kinoshita Y, Tajiri T, Taguchi T, Kohashi K, Oda Y, Tsuneyoshi M, Hara T: Long-term survival after autologous peripheral blood stem cell transplantation in two patients with malignant rhabdoid tumor of the kidney. Pediatr Blood Cancer; 2009 Jul;52(7):888-90
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  • [Title] Long-term survival after autologous peripheral blood stem cell transplantation in two patients with malignant rhabdoid tumor of the kidney.
  • A 5-month-old male with stage II malignant rhabdoid tumor of the kidney (MRTK) and a 24-month-old male with stage III MRTK were treated with surgical resection of tumors and chemotherapy of alternating ICE (ifosfamide, carboplatin, and etoposide) and VDC (vincristine, doxorubicin, and cyclophosphamide), followed by high-dose chemotherapy using etoposide, carboplatin, and melphalan with autologous hematopoietic stem cell transplantation (SCT).
  • Two patients have been alive without any evidence of disease for 30 and 37 months after diagnosis, respectively, and require no medication.
  • Consolidation with SCT should be further studies for selected patients with high-risk MRTK.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Hematopoietic Stem Cell Transplantation. Kidney Neoplasms / mortality. Kidney Neoplasms / therapy. Rhabdoid Tumor / mortality. Rhabdoid Tumor / therapy
  • [MeSH-minor] Carboplatin / administration & dosage. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Humans. Ifosfamide / administration & dosage. Infant. Male. Melphalan / administration & dosage. Prognosis. Survival Rate. Tomography, X-Ray Computed. Transplantation, Autologous. Treatment Outcome. Vincristine / administration & dosage

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  • [Copyright] (c) 2009 Wiley-Liss, Inc.
  • (PMID = 19260106.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; BG3F62OND5 / Carboplatin; Q41OR9510P / Melphalan; UM20QQM95Y / Ifosfamide
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9. 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.
  • Aspirates from Wilms' tumor, which are mesenchyme predominant, show clusters of spindle cells associated with the matrix material.
  • 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.
  • Intrarenal yolk sac tumor is a rare neoplasm and shows severely pleomorphic cells on aspiration.
  • 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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10. Naraghi S, Khoshyomn S, DeMattia JA, Vane DW: Receptor tyrosine kinase inhibition suppresses growth of pediatric renal tumor cells in vitro. J Pediatr Surg; 2000 Jun;35(6):884-90
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  • [Title] Receptor tyrosine kinase inhibition suppresses growth of pediatric renal tumor cells in vitro.
  • PURPOSE: Children who undergo standard therapy for renal tumors are at an increased risk for treatment sequelae such as congestive heart failure, abnormal trunk development, and secondary malignancies.
  • Recent experimental evidence suggests that growth factor receptors such as epidermal growth factor receptor (EGFR) and platelet-derived growth factor receptor (PDGFR) play an important role in growth and development of pediatric renal tumors especially that of Wilms' tumor.
  • In this study we investigated the effects of genistein, AG1478, and AG1295, from the class of growth factor receptor tyrosine kinase (GFR-TK) inhibitors, on proliferation and colonigenic growth of 2 pediatric renal tumor cell lines.
  • METHODS: The authors studied the effect of genistein (broad-spectrum GFR-TK inhibitor), AG1478 (EGFR-specific GFR-TK inhibitor), and AG1295 (PDGFR-specific GFR-TK inhibitor) on proliferation and colonigenic growth of rhabdoid tumor of the kidney and Wilms' tumor cell lines: G-401 and SK-NEP-1, respectively.
  • Viable cell counts at each concentration were obtained by hemocytometer and trypan blue exclusion, and percent growth inhibition was calculated based on control cultures at the same time-point.
  • As a measure of colonigenic survival, the percent inhibition of colony formation in drug-treated dishes was calculated based on the number of colonies (>50 cells) in control dishes.
  • CONCLUSIONS: This is the first experimental study on the use of GFR-TK inhibitors as a potential treatment for pediatric renal tumors.
  • GFR-TK inhibitors such as genistein occur naturally in soybean foods and have been shown to reach therapeutic levels in blood after consuming a soybean-based diet.
  • Considering the significance of growth factor receptor activity in Wilms' tumor development, inhibition of GFR-TKs should be investigated as effective and potentially nontoxic adjunctive treatment for this childhood tumor.
  • Furthermore, GFR-TK inhibitors may offer an effective alternative to the treatment of commonly fatal rhabdoid tumor of the kidney in children.
  • [MeSH-major] Kidney Neoplasms / drug therapy. Receptor Protein-Tyrosine Kinases / antagonists & inhibitors. Tyrphostins / therapeutic use
  • [MeSH-minor] Adult. Cell Division / drug effects. Drug Screening Assays, Antitumor. Enzyme Inhibitors / therapeutic use. Female. Genistein / therapeutic use. Humans. Infant. Male. Platelet-Derived Growth Factor / antagonists & inhibitors. Protein Tyrosine Phosphatases / antagonists & inhibitors. Quinazolines. Rhabdoid Tumor / drug therapy. Rhabdoid Tumor / pathology. Tumor Cells, Cultured / drug effects. Tumor Cells, Cultured / pathology. Tumor Stem Cell Assay. Wilms Tumor / drug therapy. Wilms Tumor / pathology

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  • (PMID = 10873031.001).
  • [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 / 6,7-dimethoxy-2-phenylquinoxaline; 0 / Enzyme Inhibitors; 0 / Platelet-Derived Growth Factor; 0 / Quinazolines; 0 / Tyrphostins; 170449-18-0 / tyrphostin AG 1478; DH2M523P0H / Genistein; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 3.1.3.48 / Protein Tyrosine Phosphatases
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11. Soffer SZ, Kim E, Huang J, McCrudden K, Yokoi A, Moore JT, Manley C, O'Toole K, Middlesworth W, Stolar C, Yamashiro DJ, Kandel JJ: Resistance of a VEGF-producing tumor to anti-VEGF antibody: unimpeded growth of human rhabdoid tumor xenografts. J Pediatr Surg; 2002 Mar;37(3):528-32
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  • [Title] Resistance of a VEGF-producing tumor to anti-VEGF antibody: unimpeded growth of human rhabdoid tumor xenografts.
  • BACKGROUND/PURPOSE: Rhabdoid tumor of the kidney (RTK) is a lethal malignancy of childhood for which there currently are no effective therapies.
  • Because vascular endothelial growth factor (VEGF) is nearly ubiquitous in human tumors, the authors hypothesized that a xenograft model of RTK would (1) express VEGF and (2) respond to anti-VEGF intervention.
  • METHODS: A total of 2 x 10(6) cultured RTK cells were implanted intrarenally (G-401) in athymic mice.
  • Apoptosis was assessed by TdT-mediated dUTP nick end labeling (TUNEL) assay, VEGF expression examined by reverse transcription polymerase chain reaction, and tumor weights compared by Kruskal-Wallis analysis.
  • RESULTS: Mean tumor weights were not altered significantly by anti-VEGF (0.78-g, controls v 0.56-g treated tumors; P value, not significant).
  • Grossly, xenografts grew in a novel manner, encasing rather than invading the kidney, and did not metastasize.
  • CONCLUSIONS: Unexpectedly, growth of RTK xenografts was not inhibited by specific anti-VEGF antibody, although these tumors express significant amounts of VEGF.
  • In addition, RTK vasculature, apoptosis, and VEGF expression were not substantially altered by anti-VEGF antibody.
  • These results suggest that tumor-derived VEGF is of highly variable importance in different malignancies.
  • [MeSH-major] Antibodies, Monoclonal / pharmacology. Antineoplastic Agents / pharmacology. Endothelial Growth Factors / immunology. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Lymphokines / immunology. Rhabdoid Tumor / drug therapy. Rhabdoid Tumor / pathology. Transplantation, Heterologous / pathology
  • [MeSH-minor] Animals. Antigens, CD31 / immunology. Drug Resistance, Neoplasm. Female. Humans. Immunohistochemistry. Lung Neoplasms / drug therapy. Lung Neoplasms / metabolism. Lung Neoplasms / pathology. Mice. Mice, Nude. Paraffin Embedding. Vascular Endothelial Growth Factor A. Vascular Endothelial Growth Factors

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  • [Copyright] Copyright 2002 by W.B. Saunders Company.
  • (PMID = 11877682.001).
  • [ISSN] 1531-5037
  • [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 / Antibodies, Monoclonal; 0 / Antigens, CD31; 0 / Antineoplastic Agents; 0 / Endothelial Growth Factors; 0 / Lymphokines; 0 / Vascular Endothelial Growth Factor A; 0 / Vascular Endothelial Growth Factors
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12. 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.
  • Fas is a pro-apoptotic receptor that induces cell death when bound by its ligand and is expressed at greater levels in pediatric renal tumors of good prognosis.
  • This study evaluates the expression of survivin, as well as the prognostic value of the survivin:fas ratio in various types and stages of pediatric renal tumors.
  • METHODS: Multiple apoptosis mRNA species were quantified by Rnase protection assay (RPA) in 32 pediatric renal tumors and adjacent normal kidney specimens before chemotherapy: Wilms' tumor (WT), n = 9; clear cell sarcoma (CCS), n = 4; rhabdoid tumor of the kidney (RTK), n = 5; mesoblastic nephroma (MN), n = 3 and normal kidney, n = 11.
  • RESULTS: Pediatric renal tumors express greater levels of both pro- and antiapoptotic factors than normal kidney.
  • Survivin and fas appeared to be expressed differentially in the tumor specimens sampled.
  • The mean survivin:fas ratio was significantly greater in the 10 tumors that went on to recur after treatment (4 RTK, 3 CCS, 3 WT), than in tumors not recurring (2.16+/-1.4 v 1.0+/-1.07; P =.01, Kruskal-Wallis test).
  • The positive predictive value of tumor recurrence was 85.7% (CI: 42.1%, 99.6%) and the negative predictive value was 71.4% (CI: 41.9%, 91.6%) when a cutoff ratio of 1.6 was considered.
  • CONCLUSIONS: The survivin:fas mRNA ratio is of prognostic value in its ability to predict recurrent disease in children undergoing treatment for pediatric renal tumors.
  • In this series, a ratio of greater than 1.6 predicted recurrent disease with a high probability irrespective of clinical stage or pathologic type.
  • Determining the survivin:fas ratio may guide treatment, follow-up and counseling of patients with pediatric renal tumors.
  • [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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13. Powis M: Neonatal renal tumours. Early Hum Dev; 2010 Oct;86(10):607-12
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  • [Title] Neonatal renal tumours.
  • Neonatal renal tumours are rare, with only 7% of all neonatal tumours arising from the kidney.
  • Mesoblastic nephroma is the most common tumour to be found at this age, but Wilms' tumour and other malignant and benign tumours occur.
  • Given the low malignant potential of these tumours, treatment is by radical nephroureterctomy, except in cases with bilateral disease or syndromic patients with a high incidence of metachronous tumours.
  • Chemotherapy is rarely indicated.
  • Survival is generally excellent for all tumour types in this age group, the exception being malignant rhabdoid tumour of the kidney which may have metastases at presentation.
  • [MeSH-major] Kidney Neoplasms / diagnosis. Nephroma, Mesoblastic / diagnosis. Wilms Tumor / diagnosis
  • [MeSH-minor] Anatomy, Cross-Sectional. Diagnosis, Differential. Genetic Predisposition to Disease. Humans. Imaging, Three-Dimensional. Infant, Newborn. Neuroblastoma / diagnosis. Practice Guidelines as Topic. Risk Factors

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  • [Copyright] Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.
  • (PMID = 20888153.001).
  • [ISSN] 1872-6232
  • [Journal-full-title] Early human development
  • [ISO-abbreviation] Early Hum. Dev.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Ireland
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14. 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.
  • MRTK is unique in its significant association with primary brain tumours or brain metastases.
  • 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.
  • [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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15. 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.
  • Variations in expression of such factors may account for some variations in tumor behavior.
  • This study evaluates the expression of apoptotic mRNA species in pediatric renal tumors to determine whether a pattern of differential apoptosis gene expression correlates with tumor grade and type.
  • METHODS: Twenty-five frozen tissue specimens were obtained from patients undergoing biopsy or resection of pediatric renal tumors before chemotherapy: Wilms' tumor stage II (WT-II, n = 4); Wilms' tumor stage III/IV (WT-III/IV, n = 4); clear cell sarcoma of the kidney stage III (CCSK, n = 2); rhabdoid tumor of the kidney stage III/IV (RTK, n = 4); and normal kidney (NK, n = 11).
  • 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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16. 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.
  • In 17 (3 %) patients the tumor stage remained unclear.
  • Tumor volume was measured in 487 patients before and in 402 after preoperative chemotherapy.
  • The median tumor volume did shrink from 353 to 126 ml.
  • The event free survival (EFS) after 5 years was 91 % for all patients with unilateral Wilms tumor without distant metastasis.
  • 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.
  • CONCLUSION: Patients with unilateral Wilms tumor without metastasis have an excellent prognosis.
  • 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.
  • Focal anaplasia has a better prognosis than diffuse anaplasia and will be classified as intermediate 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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17. 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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18. 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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19. Leath CA 3rd, Huh WK, Conner M, Barnes MN 3rd: Primary extrarenal rhabdoid tumor of the ovary. A case report. J Reprod Med; 2003 Apr;48(4):283-6
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  • [Title] Primary extrarenal rhabdoid tumor of the ovary. A case report.
  • BACKGROUND: Malignant rhabdoid tumors are rare, aggressive neoplasms that consist of both renal and extrarenal subtypes.
  • Although extrarenal rhabdoid tumors have been documented at multiple extrarenal sites, to our knowledge no primary ovarian cases have been reported.
  • CASE: An 18-year-old, Caucasian woman was diagnosed with a pure primary extrarenal rhabdoid tumor of the ovary following diagnostic laparoscopy for pelvic pain.
  • The tumor exhibited rapid growth, failed to respond to chemotherapy and led rapidly to death.
  • CONCLUSION: Although no other reports on primary ovarian extrarenal rhabdoid tumor have been published, the aggressive behavior of the tumor in this patient was similar to that seen in patients with metastatic disease.
  • [MeSH-major] Ovarian Neoplasms / pathology. Rhabdoid Tumor / pathology
  • [MeSH-minor] Adolescent. Biopsy, Needle. Chemotherapy, Adjuvant. Combined Modality Therapy. Disease Progression. Fatal Outcome. Female. Humans. Immunohistochemistry. Laparoscopy / methods. Neoplasm Staging. Pelvic Pain / diagnosis. Pelvic Pain / etiology. Rare Diseases

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  • (PMID = 12746993.001).
  • [ISSN] 0024-7758
  • [Journal-full-title] The Journal of reproductive medicine
  • [ISO-abbreviation] J Reprod Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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20. 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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21. 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.
  • 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

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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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22. Anatol T, Mohammed W, Nunez J: A childhood malignant rhabdoid tumour of the kidney. West Indian Med J; 2002 Sep;51(3):191-3
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  • [Title] A childhood malignant rhabdoid tumour of the kidney.
  • A case report is presented of a malignant rhabdoid tumour occurring in the kidney of a 23-month-old boy.
  • Important differences between this and the conventional Wilms' tumour include the histological demonstration of sheets or cords of large cells resembling myoblasts, a tendency to frequent clinical relapse, and a high mortality rate despite multimodal therapy.
  • In this child, an encouraging initial response to pre-operative chemotherapy, followed by surgical excision and postoperative triple chemotherapy, was not sustained.
  • Recurrence of pulmonary metastases did not respond to further chemotherapy and whole lung irradiation.
  • [MeSH-major] Kidney Neoplasms / diagnosis. Rhabdoid Tumor / diagnosis

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  • (PMID = 12501552.001).
  • [ISSN] 0043-3144
  • [Journal-full-title] The West Indian medical journal
  • [ISO-abbreviation] West Indian Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Jamaica
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23. 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.
  • A Wilms' tumor (WT) was present in 73 children.
  • 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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24. Perlman EJ: Pediatric renal tumors: practical updates for the pathologist. Pediatr Dev Pathol; 2005 May-Jun;8(3):320-38
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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.
  • All rhabdoid tumors outside the central nervous system will be eligible for a single 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
  • [MeSH-minor] Child. Humans. Neoplasm Staging

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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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