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1. Ghanem MA, van Steenbrugge GJ, Sudaryo MK, Mathoera RB, Nijman JM, van der Kwast TH: Expression and prognostic relevance of vascular endothelial growth factor (VEGF) and its receptor (FLT-1) in nephroblastoma. J Clin Pathol; 2003 Feb;56(2):107-13
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  • [Title] Expression and prognostic relevance of vascular endothelial growth factor (VEGF) and its receptor (FLT-1) in nephroblastoma.
  • AIMS: To investigate the prognostic relevance of vascular endothelial growth factor (VEGF) and its receptor Flt-1 in nephroblastoma and whether tumour microvessel density (MVD) immunoreactivity, determined by the CD31 antigen, is related to the expression of VEGF and Flt-1.
  • METHODS: The expression of VEGF and Flt-1 and MVD were investigated by means of immunohistochemical analysis in 62 Wilms's tumours.
  • Patients were treated preoperatively with chemotherapy and had a mean follow up of 5.7 years.
  • RESULTS: In general, VEGF and Flt-1 were expressed in normal kidney parenchyma and to a variable extent in the three main components of Wilms's tumour, namely: the blastemal, epithelial, and stromal cells.
  • In tumour tissue, 52% and 47% of blastemal cells were positive for VEGF and Flt-1, respectively.
  • A non-significant correlation was found between the expression of VEGF and Flt-1 in blastemal and epithelial cells and the clinicopathological stage.
  • CONCLUSIONS: These results indicate that VEGF and Flt-1 protein expression are closely related to MVD and seem to be an important predictor for poor prognosis in treated patients with Wilms's tumour.
  • Therefore, the expression of these molecules in primary Wilms's tumour may be useful in identifying those patients at high risk of tumour recurrence and in guiding antiangiogenic treatment.
  • [MeSH-major] Kidney Neoplasms / metabolism. Neoplasm Proteins / metabolism. Neovascularization, Pathologic / metabolism. Vascular Endothelial Growth Factor Receptor-1 / metabolism. Wilms Tumor / metabolism

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  • (PMID = 12560388.001).
  • [ISSN] 0021-9746
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD31; 0 / Neoplasm Proteins; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-1
  • [Other-IDs] NLM/ PMC1769871
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2. Koizumi K, Mizusawa H, Oguchi T, Domen T, Kato H: [Adult Wilms' tumor mimicking adrenal tumor: case report]. Hinyokika Kiyo; 2008 Sep;54(9):603-6
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  • [Title] [Adult Wilms' tumor mimicking adrenal tumor: case report].
  • Computed tomography, magnetic resonance image and angiograpy demonstrated non-functioning adrenal tumor.
  • Right nephrectomy was performed, because the tumor adhered firmly to the upper pole of the right kidney.
  • The pathological diagnosis was nephroblastoma (nephroblastic type, complex subtype).
  • Postoperative chemotherapy was started for stage III Wilms' tumor.
  • However, peripheral neuropathy such as numbness of the fingers developed.
  • Therefore, chemotherapy was discontinued in the fifth week.
  • In adult patients, postoperative chemotherapy for nephroblastoma is often interrupted due to side effects.
  • [MeSH-major] Kidney Neoplasms / diagnosis. Wilms Tumor / diagnosis
  • [MeSH-minor] Adrenal Gland Neoplasms. Adult. Antineoplastic Combined Chemotherapy Protocols / adverse effects. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Combined Modality Therapy. Dactinomycin / administration & dosage. Diagnosis, Differential. Diagnostic Imaging. Female. Humans. Nephrectomy. Vincristine / administration & dosage

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  • (PMID = 18975574.001).
  • [ISSN] 0018-1994
  • [Journal-full-title] Hinyokika kiyo. Acta urologica Japonica
  • [ISO-abbreviation] Hinyokika Kiyo
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine
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3. Ramburan A, Chetty R, Hadley GP, Naidoo R, Govender D: Microsatellite analysis of the DCC gene in nephroblastomas: pathologic correlations and prognostic implications. Mod Pathol; 2004 Jan;17(1):89-95
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  • Microsatellite instability has been reported in a wide variety of cancer types.
  • Normal and tumour DNA was isolated from 106 cases of nephroblastoma using the standard proteinase K digestion and phenol-chloroform extraction method from paraffin wax-embedded tissue.
  • The results were correlated with age at diagnosis, preoperative chemotherapy, clinicopathological stage, histological classification and patient outcome using chi(2) test.
  • All patients with genetic aberrations for more than one DCC marker died independent of histological classification and stage (P=0.016).
  • There was no statistically significant difference when DCC aberrations were compared with age at diagnosis, preoperative chemotherapy and clinicopathological stage.
  • [MeSH-major] Gene Expression Regulation, Neoplastic. Kidney Neoplasms / genetics. Microsatellite Instability. Microsatellite Repeats. Receptors, Cell Surface / genetics. Tumor Suppressor Proteins / genetics. Wilms Tumor / genetics
  • [MeSH-minor] Adolescent. Allelic Imbalance. Child. Child, Preschool. Follow-Up Studies. Humans. Infant. Loss of Heterozygosity. Mutation. Neoplasm Staging. Prognosis. Time Factors. Treatment Outcome

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  • (PMID = 14631365.001).
  • [ISSN] 0893-3952
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DCC protein, human; 0 / Receptors, Cell Surface; 0 / Tumor Suppressor Proteins
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4. Furtwaengler R, Reinhard H, Leuschner I, Schenk JP, Goebel U, Claviez A, Kulozik A, Zoubek A, von Schweinitz D, Graf N, Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH) Nephroblastoma Study Group: Mesoblastic nephroma--a report from the Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH). Cancer; 2006 May 15;106(10):2275-83
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  • BACKGROUND: Surgery alone is the appropriate first-line treatment for patients with mesoblastic nephroma (MN).
  • The authors evaluated the outcome of patients with MN who were enrolled in either the International Society of Pediatric Oncology (SIOP) 93-01/GPOH or the SIOP 2001/GPOH Nephroblastoma Study and Trial.
  • Eleven patients were suspected antenatally of having a renal tumor.
  • The median observation time was 4.2 years.
  • Five patients older than 6 months received preoperative chemotherapy.
  • Nine patients had a Stage III MN, 5 of those patients had tumor ruptures, and 8 had positive surgical margins.
  • After they underwent nephrectomy, 40 patients received no further treatment.
  • Patients with a cellular MN, patients with age 3 months or older, and patients with Stage III MN had lower EFS.
  • Three patients developed recurrent disease, and 2 of those patients died.
  • Nonetheless, a subgroup of patients with MN (Stage III cellular MN in patients age 3 months or older) tends to develop recurrences more often.
  • Further prospective studies will be needed to verify this finding and should help determine whether these patients may benefit from adjuvant therapy.
  • [MeSH-major] Kidney Neoplasms / diagnosis. Kidney Neoplasms / therapy. Nephroma, Mesoblastic / diagnosis. Nephroma, Mesoblastic / therapy
  • [MeSH-minor] Age Factors. Biopsy, Needle. Chemotherapy, Adjuvant. Child. Child, Preschool. Cohort Studies. Female. Germany. Humans. Immunohistochemistry. Infant. Infant, Newborn. Logistic Models. Male. Neoplasm Staging. Nephrectomy / methods. Pediatrics. Prognosis. Proportional Hazards Models. Retrospective Studies. Risk Assessment. Societies, Medical. Statistics, Nonparametric. Survival Analysis. Treatment Outcome

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  • [Copyright] Copyright 2006 American Cancer Society
  • (PMID = 16596620.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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5. Hosokawa Y, Saiki S, Hanafusa T, Meguro N, Maeda O, Kinouchi T, Kuroda M, Usami M, Kotake T: [A case of adult Wilms' tumor]. Hinyokika Kiyo; 2001 Sep;47(9):641-3
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  • [Title] [A case of adult Wilms' tumor].
  • Wilms' tumor is very rarely found in adults and there are no established treatment guidelines for such tumors in adults.
  • Computed tomography scan revealed a large right renal mass with enlarged lymph nodes.
  • Angiography showed a hypovascular tumor.
  • She underwent right nephrectomy and resection of lymph node metastasis with a diagnosis of malignant renal tumor.
  • Histopathological examination revealed nephroblastoma with lymph node metastasis.
  • The disease was classified as stage III according to the National Wilms' Tumor Study classification.
  • The patient received adjuvant chemotherapy consisting of ifosfamide, cisplatin, and etoposide.
  • This protocol was selected because of the published poor results with the standard Wilms' tumor chemotherapeutic agents when used in adults.
  • She remained without tumor recurrence as of six months after surgery.
  • Development of better therapeutic approaches to adult Wilms' tumor is awaited.
  • [MeSH-major] Kidney Neoplasms / therapy. Wilms Tumor / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Cisplatin / administration & dosage. Etoposide / administration & dosage. Female. Humans. Ifosfamide. Lymph Node Excision. Lymphatic Metastasis. Middle Aged. Neoplasm Staging. Nephrectomy. Treatment Outcome

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  • (PMID = 11692602.001).
  • [ISSN] 0018-1994
  • [Journal-full-title] Hinyokika kiyo. Acta urologica Japonica
  • [ISO-abbreviation] Hinyokika Kiyo
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide
  • [Number-of-references] 12
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6. Naguib SF, El Haddad A, El Badawy SA, Zaghloul AS: Multidisciplinary approach to wilms' tumor: a retrospective analytical study of 53 patients. J Egypt Natl Canc Inst; 2008 Dec;20(4):410-23

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Multidisciplinary approach to wilms' tumor: a retrospective analytical study of 53 patients.
  • AIM OF THE WORK: The aim of this work was to assess the epidemiologic aspects, clinico-pathological features and the results of multidisciplinary treatment of Wilms' tumor (WT) in pediatric patients treated at the National Cancer Institute (NCI), Cairo University, between January 2002 and December 2004.
  • Doppler study of the renal vein and vena cava and bone scan were done when needed.
  • Neoadjuvant chemotherapy was given to patients suffering from poor general condition, extensive tumor thrombus in the renal vein, irresectable and bilateral (stage V) nephroblastoma.
  • Otherwise, up-front nephrectomy was the standard therapeutic approach in this study.
  • Stage I and III were the most common (29.4% each), followed by stage II and IV (17.7% each), and finally by stage V (5.9%).
  • Neoadjuvant chemotherapy was given to 27 cases while up-front nephrectomy was undertaken in 26 cases.
  • Intra-operative spillage occurred in 12% of patients who had preoperative chemotherapy and 31% of those who had upfront nephrectomy.
  • Twenty five patients underwent renal bed irradiation only, while in the other 7 whole abdominal irradiation was used.
  • Complete remission (CR) was achieved in 74%, while death during neoadjuvant therapy took place in 4% of the cases.
  • Disease progression during treatment was noticed in 8%.
  • These patients were all treated with radio- and chemotherapy.
  • Therapy-related complications were mainly related to chemotherapy in 49% of patients and surgery in 5.9%.
  • Regional lymph node biopsy and accurate marking of residual disease are essential components of surgical treatment and heroic surgical attempts are unnecessary.
  • Neoadjuvant chemotherapy, which is still a fertile source of debate, could possibly help to avoid excessive post-operative radiotherapy and its potential complications.
  • Tumor stage and age of patient were found to affect the results of treatment of Wilms' tumor; but the only statistically significant determinant of prognosis was histologic differentiation.
  • Finally, further studies including molecular markers are needed to augment therapy for the blastemal predominance subtype or for favorable histology associated with loss of heterozygosity (LOA) at chromosomes 1p and 16q aiming at improved survival.
  • KEY WORDS: Wilms' tumor - Nephroblastoma.

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  • (PMID = 20571600.001).
  • [ISSN] 1110-0362
  • [Journal-full-title] Journal of the Egyptian National Cancer Institute
  • [ISO-abbreviation] J Egypt Natl Canc Inst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
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7. Singam P, Ho C, Hong GE, Mohd A, Tamil AM, Cheok LB, Zainuddin Z: Clinical characteristics of renal cancer in Malaysia : a ten year review. Asian Pac J Cancer Prev; 2010;11(2):503-6
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  • [Title] Clinical characteristics of renal cancer in Malaysia : a ten year review.
  • Renal cancer is rare and its incidence is 1.9 per 100,000 in the Malaysian population, which consists of three major ethnic groups (Malay, Chinese and Indians).
  • The study included all renal cancer patients from a single medical institution over ten years, with a total of 75 cases.
  • Seventy-three patients underwent surgery while 2 received only radiotherapy or chemotherapy.
  • There were 26 (37.4%) patients with Stage I disease, 14 (18.7%) at Stage II, 23 (30.7%) at Stage III and 12 (16%) at Stage IV.
  • The Chinese race presented at mean older age (p= 0.02) and later stage of disease (p= 0.046).
  • Patients above 40 years old had more advanced stage disease (p= 0.023).
  • Tumour histology were clear cell (72%), urothelial cell (13.3%), sarcomatoid cell and nephroblastoma each contributed 2.7%.
  • In conclusion we observed significant influences of age and race in the clinical presentation of renal cancer in our institution based population.
  • [MeSH-major] Carcinoma, Papillary / therapy. Carcinoma, Renal Cell / therapy. Carcinoma, Squamous Cell / therapy. Kidney Neoplasms / therapy. Wilms Tumor / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Combined Modality Therapy. Female. Humans. Incidence. Malaysia. Male. Middle Aged. Neoplasm Staging. Prognosis. Radiotherapy Dosage. Risk Factors. Survival Rate. Time Factors. Young Adult

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  • (PMID = 20843141.001).
  • [ISSN] 2476-762X
  • [Journal-full-title] Asian Pacific journal of cancer prevention : APJCP
  • [ISO-abbreviation] Asian Pac. J. Cancer Prev.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] Thailand
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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8. Blakely ML, Shamberger RC, Norkool P, Beckwith JB, Green DM, Ritchey ML, National Wilms' Tumor Study Group: Outcome of children with cystic partially differentiated nephroblastoma treated with or without chemotherapy. J Pediatr Surg; 2003 Jun;38(6):897-900
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  • [Title] Outcome of children with cystic partially differentiated nephroblastoma treated with or without chemotherapy.
  • BACKGROUND/PURPOSE: Cystic partially differentiated nephroblastoma (CPDN) is a rare variant of Wilms' tumor thought to be more favorable than standard nephroblastoma.
  • The purpose of this report is to examine the outcome of children with CPDN, after nephrectomy, treated with vincristine and dactinomycin based chemotherapy (+/- doxorubicin) or no chemotherapy.
  • METHODS: Patients were registered with the National Wilms' Tumor Study Group (NWTSG) and data were collected prospectively.
  • Thirteen patients received chemotherapy, and 8 patients did not.
  • In the chemotherapy group the stage distribution was as follows: stage I (n = 10), stage II (n = 2), stage V (n = 1).
  • In the no-chemotherapy group, all 8 patients were stage I.
  • All patients had complete tumor resection.
  • In patients receiving chemotherapy, 30% (n = 4) had toxicities causing dose reduction.
  • For stage I patients, treatment with complete tumor resection appears to be as efficacious as nephrectomy plus chemotherapy.
  • Stage II patients also have excellent outcome when treated with tumor resection and postoperative vincristine and dactinomycin.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Diseases, Cystic / drug therapy. Kidney Neoplasms / drug therapy. Wilms Tumor / drug therapy. Wilms Tumor / pathology
  • [MeSH-minor] Adolescent. Adult. Cell Differentiation / drug effects. Child. Child, Preschool. Dactinomycin / administration & dosage. Diagnosis, Differential. Doxorubicin / administration & dosage. Female. Follow-Up Studies. Humans. Male. Neoplasm Staging. Nephrectomy. Survival Analysis. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 12778388.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA-42326; United States / NICHD NIH HHS / HD / K23-HD/RR01473-01
  • [Publication-type] Journal Article; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin
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9. Pollono D, Drut R, Tomarchio S, Fontana A, Ibañez O: Fetal rhabdomyomatous nephroblastoma: report of 14 cases confirming chemotherapy resistance. J Pediatr Hematol Oncol; 2003 Aug;25(8):640-3
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  • [Title] Fetal rhabdomyomatous nephroblastoma: report of 14 cases confirming chemotherapy resistance.
  • The authors retrospectively reviewed their experience with 14 cases of fetal rhabdomyomatous nephroblastoma (15.6% of all renal tumors seen in the 1984-1998 period), analyzing the diagnostic and treatment approach as well outcome.
  • Nine patients received preoperative chemotherapy after fine-needle aspiration biopsy.
  • The response was null in five children and mild in three; in the remaining one the tumor increased in size.
  • The only patient showing greater than 50% tumor shrinkage was the one treated with radiotherapy.
  • Postoperative treatment according to staging followed the SIOP protocol.
  • Patients with fetal rhabdomyomatous nephroblastoma presenting with distant metastatic disease and high stage have unfavorable outcomes, confirming data from the literature indicating that chemotherapy is not as effective as in other variants of nephroblastoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Rhabdomyoma / drug therapy. Rhabdomyoma / pathology. Wilms Tumor / drug therapy. Wilms Tumor / pathology
  • [MeSH-minor] Age of Onset. Child, Preschool. Drug Resistance, Neoplasm. Female. Humans. Infant. Male. Prognosis. Retrospective Studies. Treatment Outcome

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  • (PMID = 12902919.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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10. Baker JM, Viero S, Kim PC, Grant RM: Stage III cystic partially differentiated nephroblastoma recurring after nephrectomy and chemotherapy. Pediatr Blood Cancer; 2008 Jan;50(1):129-31
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  • [Title] Stage III cystic partially differentiated nephroblastoma recurring after nephrectomy and chemotherapy.
  • Cystic partially differentiated nephroblastoma (CPDN) has low malignant potential.
  • We report a 1-year-old with stage III CPDN of the right kidney that recurred following radical nephrectomy and chemotherapy.
  • There was evidence of tumor spillage pre-operatively and intra-operatively.
  • During chemotherapy the disease recurred in the omentum and the peritoneum.
  • Pathology of the recurrent resected cysts revealed a more differentiated biphasic tumor without blastemal elements.
  • Chemotherapy failed to prevent recurrence but only mature elements were present following this treatment.
  • The intensity of therapy required to treat CPDN remains undefined.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / therapy. Nephrectomy. Wilms Tumor / secondary. Wilms Tumor / therapy
  • [MeSH-minor] Combined Modality Therapy. Humans. Infant. Peritoneal Neoplasms / secondary

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 16786584.001).
  • [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
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11. Ora I, van Tinteren H, Bergeron C, de Kraker J, SIOP Nephroblastoma Study Committee: Progression of localised Wilms' tumour during preoperative chemotherapy is an independent prognostic factor: a report from the SIOP 93-01 nephroblastoma trial and study. Eur J Cancer; 2007 Jan;43(1):131-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Progression of localised Wilms' tumour during preoperative chemotherapy is an independent prognostic factor: a report from the SIOP 93-01 nephroblastoma trial and study.
  • The SIOP nephroblastoma clinical trials have previously demonstrated that preoperative chemotherapy is advantageous for patients with nephroblastoma (Wilms' tumour).
  • However, some primary tumours increase in size during preoperative chemotherapy, and to investigate the clinical relevance of this progression we studied the patient cohort with increasing tumours included in the SIOP 93-01 study (June 1993 to June 2000).
  • Patients were considered eligible if they had a confirmed localised Wilms' tumour that had been measured in at least two dimensions at diagnosis and before surgery.
  • Tumour response to preoperative chemotherapy was defined according to criteria set by the World Health Organisation (WHO).
  • Tumour progression during preoperative chemotherapy was observed in 57 of 1090 patients (5%) with localised Wilms' tumours.
  • In those cases, the tumours were significantly smaller at diagnosis and were more often stage III (p=0.05) and associated with high risk histopathology (p=0.03).
  • After adjustment for stage and risk group, progression was proved to be correlated with poorer event-free and overall survival (hazard ratio 1.9, p=0.026 and 3.2, p=0.002 respectively).
  • In summary, progression of localised Wilms' tumours is rarely seen in patients during preoperative chemotherapy.
  • However, independent of stage distribution and histopathological risk group, those whose tumours do increase in size have poorer event-free and overall survival.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Wilms Tumor / drug therapy

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  • (PMID = 17084075.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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12. Rogers T, Bowley DM, Poole J, Swanepoel P, Wainwright J, Beale P, Pitcher G: Experience and outcomes of nephroblastoma in Johannesburg, 1998 - 2003. Eur J Pediatr Surg; 2007 Feb;17(1):41-4
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  • [Title] Experience and outcomes of nephroblastoma in Johannesburg, 1998 - 2003.
  • The purpose of this study is to document and analyse our experience and the outcomes of children with nephroblastoma over recent years.
  • METHODS: This is a retrospective review of all patients who underwent combined oncological and surgical treatment for nephroblastoma in the Paediatric Oncology Unit between 1998 and 2003.
  • RESULTS: Sixty-three patients were treated for Wilms' tumour; the mean age was 3 years 8 months (range 4 months to 11 years).
  • Preoperative chemotherapy was given in forty-six cases (73 %).
  • The tumour stage distribution was 11/63 stage I (17 %), 11/63 stage II (17 %), 21/63 stage III (33 %), 16/63 stage IV (25 %) and 4/63 stage V (6 %).
  • Postoperative chemotherapy and radiotherapy was given according to the SIOP protocol.
  • Children with stage I and stage II had a disease-free survival at 4 years of 89 %.
  • However, those with stage III, IV and V disease had 4-year survival of 66.75 % (p = 0.07).
  • CONCLUSION: Outcomes for children with cancer have improved dramatically over recent years; however, in the developing world, the scarcity of hospital resources and the overwhelming burden of non-cancer diseases can mean that oncological treatment is extremely challenging.
  • In our society, children tend to present with nephroblastoma at an advanced stage; however, treatment by dedicated, multidisciplinary teams can achieve good results.
  • [MeSH-major] Kidney Neoplasms. Wilms Tumor
  • [MeSH-minor] Child. Child, Preschool. Developing Countries. Female. Humans. Infant. Male. Retrospective Studies. South Africa. Survival Analysis. Treatment Outcome

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  • (PMID = 17407020.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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13. Camassei FD, Ferlini C, Jenkner A, Bosman C, Biselli R, Donfrancesco A, Boldrini R: Nephroblastoma. DNA characteristics and their modifications induced by prenephrectomy chemotherapy: a cytofluorimetric study. Pediatr Pathol Mol Med; 2002 Jan-Feb;21(1):15-23
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  • [Title] Nephroblastoma. DNA characteristics and their modifications induced by prenephrectomy chemotherapy: a cytofluorimetric study.
  • Treatment of nephroblastoma (Wilms' tumor) has presently achieved a 90% survival rate.
  • Stage and grade are considered the most reliable prognostic parameters, but other biological factors are under study in order to improve patient stratification.
  • We retrospectively studied 79 patient with nephroblastoma (58 pretreated with chemotherapy and 21 not pretreated) by means of flow cytometry.
  • DNA modifications induced by chemotherapy were investigated.
  • S-phase rate decreased after chemotherapy (p = 0.0002) and was related to survival.
  • The worse outcome of pretreated patients might be attributed to a minor sensitivity to postoperative treatment: Preoperative chemotherapy would decrease the cell proliferation and might select resistant cellular clones of (possible) neoplastic residues.
  • [MeSH-major] DNA / chemistry. Flow Cytometry / methods. Wilms Tumor / genetics. Wilms Tumor / mortality
  • [MeSH-minor] Adolescent. Aneuploidy. Cell Division. Child. Child, Preschool. Female. Humans. Infant. Male. Nephrectomy. Ploidies. Prognosis. S Phase. Time Factors

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  • (PMID = 11842975.001).
  • [ISSN] 1522-7952
  • [Journal-full-title] Pediatric pathology & molecular medicine
  • [ISO-abbreviation] Pediatr Pathol Mol Med
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 9007-49-2 / DNA
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14. Boccon-Gibod L, Rey A, Sandstedt B, Delemarre J, Harms D, Vujanic G, De Kraker J, Weirich A, Tournade MF: Complete necrosis induced by preoperative chemotherapy in Wilms tumor as an indicator of low risk: report of the international society of paediatric oncology (SIOP) nephroblastoma trial and study 9. Med Pediatr Oncol; 2000 Mar;34(3):183-90
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  • [Title] Complete necrosis induced by preoperative chemotherapy in Wilms tumor as an indicator of low risk: report of the international society of paediatric oncology (SIOP) nephroblastoma trial and study 9.
  • BACKGROUND: The SIOP Nephroblastoma therapeutic protocols include a period of preoperative chemotherapy followed by nephrectomy and a period of postoperative chemotherapy.
  • From the outset, identification of low-risk groups has been an aim of the SIOP Nephroblastoma Trials and Studies.
  • Now that 90% of children with Wilms tumor can be cured, attention is even more focused on the identification of patients who could benefit from less aggressive postoperative therapy, thus minimizing the morbidity and late effects associated with treatment.
  • The prognostic implications of total necrosis in nephroblastoma after chemotherapy have not been investigated hitherto.
  • PROCEDURE: Between November 1, 1987 and June 30, 1993, 599 patients referred to the SIOP-9 Nephroblastoma Trial and Study were preoperatively treated and classified as stages I-IV nonanaplastic Wilms tumor.
  • RESULTS: Of these 599 patients, pathologic examination of the nephrectomy specimen revealed a completely necrotic Wilms tumor (CNWT) with no viable tumor remaining in 59 (10%): these comprised 37 stages I-III and 22 stage IV.
  • The only death was related to veno-occlusive disease and occurred in a stage I patient in the month following nephrectomy.
  • Stage IV patients represented 37% of CNWT (vs. only 10% of all other cases of unilateral nonanaplastic Wilms tumor) and had a 100% rate of survival.
  • Children with CNWT were older (mean 59 months vs. 43 months); their tumor at diagnosis was larger and had regressed more significantly at subsequent ultrasound examination.
  • The data also uphold the hypothesis that Wilms tumors of blastemic pattern are most aggressive, but also are extremely responsive to chemotherapy.
  • CONCLUSIONS: Patients with unilateral nonanaplastic WT that showed total necrosis following preoperative chemotherapy had excellent outcome and should benefit from less aggressive postoperative treatment in further trials.
  • Other very responsive tumors, such as Wilms with <10% viable tumor, should also be assessed.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Wilms Tumor / drug therapy
  • [MeSH-minor] Adolescent. Child. Europe. Female. Humans. Male. Necrosis. Neoplasm Staging. Prognosis. Remission Induction. Risk Factors. Survival Analysis. Treatment Outcome

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  • (PMID = 10696124.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, Non-U.S. Gov't
  • [Publication-country] UNITED STATES
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15. Amel L, Leila BF, Lamia K, Olfa G, Abdelfattah Z, Mondher G, Faouzi M, Chakib K, Abdelatif N, Amor G, Slim BA: [Histologic and prognostic study of nephroblastoma in central Tunisia]. Ann Urol (Paris); 2003 Aug;37(4):164-9
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  • [Title] [Histologic and prognostic study of nephroblastoma in central Tunisia].
  • [Transliterated title] Etude anatomoclinique et pronostique des néphroblastomes dans le centre tunisien.
  • Nephroblastoma is a common malignant tumour in childhood that benefited from therapeutic progress.
  • This is a retrospective study of 35 nephroblastoma diagnosed and treated in the central region of Tunisia within the last 8 years (1991-1999).
  • We report and compare clinical features, therapeutic results and prognostic factors with those reported in the literature.
  • Pre-operative chemotherapy was done in 32 cases and the objective response rate was 58%.
  • Histologic analysis concluded to an anaplastic nephroblastoma in 2 cases.
  • Using the classification of the international society of pediatric oncology, 11.4% of tumours were stage I, 48.6% stage II, 5.7% stage III, 11.4% stage IV and 2.9% stage V.
  • Prognosis of nephroblastoma has been improved with chemotherapy and the pluridisciplinar treatment.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Nephrectomy. Wilms Tumor / drug therapy. Wilms Tumor / pathology

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  • (PMID = 12951705.001).
  • [ISSN] 0003-4401
  • [Journal-full-title] Annales d'urologie
  • [ISO-abbreviation] Ann Urol (Paris)
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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16. Hero B, Graf N, Simon T, Weirich A, Tröger J, Berthold F: Neuroblastoma preoperatively treated as nephroblastoma: does inadequate therapy worsen the prognosis? Klin Padiatr; 2002 Jul-Aug;214(4):157-61
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  • [Title] Neuroblastoma preoperatively treated as nephroblastoma: does inadequate therapy worsen the prognosis?
  • BACKGROUND: The current standard in the treatment of nephroblastoma is preoperative chemotherapy based on radiological appearance.
  • We asked whether initial chemotherapy according to nephroblastoma trials would change the prognosis for those neuroblastoma patients.
  • RESULTS: Out of 1603 patients registered in the German neuroblastoma trials, 29 patients (1.8 %) have preoperatively been treated according nephroblastoma protocols.
  • Advanced stages (11 stage 3, 12 stage 4) were dominant.
  • Patients with preoperative nephroblastoma treatment were older than the patients of the control group.
  • The outcome of the patients with preoperative chemotherapy according nephroblastoma trials was worse than that of the control group, but risk group adapted survival analysis revealed no disadvantage.
  • CONCLUSION: The prognosis of children with neuroblastoma tumours, which have been radiologically classified as nephroblastoma, is inferior compared to the prognosis of patients without preoperative nephroblastoma therapy.
  • The difference appears to be associated rather with more unfavourable biology than with the element "preoperative chemotherapy".
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Neoadjuvant Therapy. Neuroblastoma / drug therapy. Wilms Tumor / drug therapy

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  • (PMID = 12165895.001).
  • [ISSN] 0300-8630
  • [Journal-full-title] Klinische Pädiatrie
  • [ISO-abbreviation] Klin Padiatr
  • [Language] eng
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article
  • [Publication-country] Germany
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17. Reinhard H, Schmidt A, Furtwängler R, Leuschner I, Rübe C, Von Schweinitz D, Zoubek A, Niggli F, Graf N: Outcome of relapses of nephroblastoma in patients registered in the SIOP/GPOH trials and studies. Oncol Rep; 2008 Aug;20(2):463-7
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  • [Title] Outcome of relapses of nephroblastoma in patients registered in the SIOP/GPOH trials and studies.
  • We registered 170 relapses in 1392 children with nephroblastoma in the SIOP/GPOH trials.
  • Age, gender, initial stage, metastatic disease, local stage, histology, time to relapse and tumour volume were analysed for their prognostic relevance.
  • Patients with initial stage I and II showed a significantly better prognosis than children with stage III (57 vs. 31%, p=0.008).
  • The tumour volume initially and after preoperative chemotherapy did not have any influence on outcome.
  • In conclusion, factors for poor prognosis after relapse are early relapse, local stage III, high-risk histology and combined relapse.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Neoplasm Recurrence, Local / diagnosis. Wilms Tumor / drug therapy
  • [MeSH-minor] Child. Child, Preschool. Female. Humans. Male. Neoplasm Staging. Prognosis. Remission Induction. Retrospective Studies. Risk Factors. Treatment Outcome

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  • (PMID = 18636212.001).
  • [ISSN] 1021-335X
  • [Journal-full-title] Oncology reports
  • [ISO-abbreviation] Oncol. Rep.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
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18. Sidhom I, Hussien H, Kotb M, Anwer G, Aboul Naga S, Amin M, Ebied E, Ahmed H: Multidisciplinary Approach to Wilms' Tumor: 10 Years Experience of NCI, Egypt. J Clin Oncol; 2004 Jul 15;22(14_suppl):8544

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

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  • (PMID = 28013808.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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19. Tahri A, Benchekroun N, Karkouri M, Dahami Z, Sahraoui S, Acharki A, Benider A, Squalli S, Benjelloun S, Kahlain A: [Nephroblastoma in adults. Three case reports]. Ann Urol (Paris); 2001 Sep;35(5):257-61
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  • [Title] [Nephroblastoma in adults. Three case reports].
  • The authors report three nephroblastoma's cases occurred in adult and treated at Ibn Rochd Oncology Center at Casablanca.
  • At diagnosis, radiological exams revealed that patients presented an advanced stage and metastatic disease in one case.
  • The treatment was a total nephrectomy with ganglioma dissection when the tumor was resectable followed by chemotherapy and radiotherapy.
  • Two patients was in progressive disease despite treatment, and one patient was lost at follow-up.
  • The prognosis of adult's nephroblastoma remains very severe, due to the advanced stage at diagnosis and the mediocre reply to the treatment.
  • [MeSH-major] Kidney Neoplasms / pathology. Nephrectomy. Wilms Tumor / pathology
  • [MeSH-minor] Adult. Age of Onset. Chemotherapy, Adjuvant. Diagnosis, Differential. Disease Progression. Female. Humans. Male. Neoplasm Staging. Prognosis. Radiotherapy, Adjuvant

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  • (PMID = 11675960.001).
  • [ISSN] 0003-4401
  • [Journal-full-title] Annales d'urologie
  • [ISO-abbreviation] Ann Urol (Paris)
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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20. 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.
  • 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.
  • In almost 70% of the children the stage of advancement was low (stage I and IIN-).
  • 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%.
  • CONCLUSIONS:. 1) Mesoblastic nephroma and low risk nephroblastoma are the most common tumours in children within the first three years of life.
  • 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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21. Vujanić GM, Sandstedt B: The pathology of Wilms' tumour (nephroblastoma): the International Society of Paediatric Oncology approach. J Clin Pathol; 2010 Feb;63(2):102-9
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  • [Title] The pathology of Wilms' tumour (nephroblastoma): the International Society of Paediatric Oncology approach.
  • In the International Society of Paediatric Oncology renal tumour trials, preoperative chemotherapy has been successfully applied with resulting reduction of tumour rupture and increased favourable stage distribution of nephroblastoma.
  • Postoperative treatment includes chemotherapy and sometimes radiotherapy in a risk-adapted approach based on histological sub-classification and stage of the tumour.
  • However, preoperative chemotherapy alters the tumour's histological features and distribution of subtypes, and makes staging more difficult.
  • It emphasises the importance of a systematic, step-by-step analysis based on adequately sampled material, in order to accurately sub-classify a nephroblastoma as a low, intermediate or high risk tumour and assign its genuine stage.
  • Finally, it outlines the standard operating procedure for submission of renal tumours for rapid central pathology review which allows the treating oncologists to apply the optimal treatment protocol.
  • [MeSH-major] Kidney Neoplasms / pathology. Wilms Tumor / pathology
  • [MeSH-minor] Chemotherapy, Adjuvant. Child. Humans. Neoplasm Staging. Nephrectomy

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  • (PMID = 19687012.001).
  • [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] 10
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22. van Toorn R, Wessels G, Stefan C: Catastrophic intracerebral hemorrhage in a young infant with Wilms tumor. J Pediatr Hematol Oncol; 2007 May;29(5):298-300
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  • [Title] Catastrophic intracerebral hemorrhage in a young infant with Wilms tumor.
  • We present a 7-month-old male infant with stage I Wilms tumor who unexpectedly died from a catastrophic intracerebral hemorrhage, 4 months after completion of chemotherapy and complete surgical resection of the tumor.
  • Although extremely rare, cerebral vascular anomalies have previously been reported in children with Wilms tumor.
  • Wilms tumor diagnosed in very young infants have clinical and morphologic attributes that do not pertain in older children and the risk of associated congenital anomalies is also much higher among those discovered in the first year of life.
  • This raises the question whether routine magnetic resonance imaging should not be performed in infants less than a year with Wilms tumor, as part of the initial evaluation, to exclude cerebral metastases and underlying vascular malformations.
  • [MeSH-major] Intracranial Hemorrhages / complications. Kidney Neoplasms / complications. Wilms Tumor / complications
  • [MeSH-minor] Combined Modality Therapy. Fatal Outcome. Humans. Infant. Male. Sensitivity and Specificity. Tomography, X-Ray Computed / methods

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  • (PMID = 17483705.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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23. Camassei FD, Arancia G, Cianfriglia M, Bosman C, Francalanci P, Ravà L, Jenkner A, Donfrancesco A, Boldrini R: Nephroblastoma: multidrug-resistance P-glycoprotein expression in tumor cells and intratumoral capillary endothelial cells. Am J Clin Pathol; 2002 Mar;117(3):484-90
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  • [Title] Nephroblastoma: multidrug-resistance P-glycoprotein expression in tumor cells and intratumoral capillary endothelial cells.
  • The development of chemoresistance in a variety of cancers seems related to overexpression of the P-glycoprotein (P-gp) drug pump.
  • Nephroblastoma, the most common malignant renal tumor of childhood, usually is responsive to treatment, and prognosis is favorable in most cases.
  • However, the disease in a subset of patients is refractory to treatment, and the disease follows an aggressive course.
  • To study P-gp expression in this tumor and its correlation with outcome, tumor samples from 93 patients were examined by immunohistochemical analysis.
  • P-gp expression was determined separately in both tumor cells and intratumoral capillary endothelium.
  • The likelihood ratio test, the Kaplan-Meier method, and the log-rank test were used to evaluate its association with clinical course, grade, stage, and administration of preoperative chemotherapy.
  • While no association of P-gp expression in tumor cells with clinical course, stage, and grade could be demonstrated, positivity in endothelial cells correlated significantly with unfavorable outcome, suggesting that chemoresistance depended on an active blood-tumor barrier.
  • Previous chemotherapy induced P-gp overexpression in tumor cells.
  • [MeSH-major] Kidney Neoplasms / chemistry. Kidney Neoplasms / pathology. P-Glycoprotein / analysis. Wilms Tumor / chemistry. Wilms Tumor / pathology
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Capillaries. Child. Child, Preschool. Combined Modality Therapy. Dactinomycin / therapeutic use. Endothelium, Vascular / chemistry. Female. Humans. Immunohistochemistry. Infant. Male. Neoplasm Staging. Postoperative Care. Preoperative Care. Radiotherapy. Remission Induction. Retrospective Studies. Vincristine / therapeutic use

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  • (PMID = 11888090.001).
  • [ISSN] 0002-9173
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / P-Glycoprotein; 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; SIOP protocol
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24. Mekki M, Belghith M, Krichène I, Zakhama A, Landolsi A, Chelly S, Nouri A: [Fetal rhabomyomatous nephroblastoma. Report of 2 cases and review of the literature]. Ann Urol (Paris); 2002 Jul;36(4):245-9
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  • [Title] [Fetal rhabomyomatous nephroblastoma. Report of 2 cases and review of the literature].
  • OBJECTIVE: Fetal rhabdomyomatous nephroblastoma is a particular and very rare histologic variety of nephroblastoma.
  • The aim of this work is to study the principal clinic, therapeutic and evolutive characteristics of the fetal rhabdomyomatous nephroblastoma through two personal cases and a review of the literature.
  • PATIENTS AND METHODS: This is a retrospective study of two observations of fetal rhabdomyomatous nephroblastoma treated in the pediatric surgery departement of Monastir (Tunisia) among 47 cases of nephroblastoma.
  • The radiologic and biologic explorations load, in the two cases, to the diagnosis of left nephroblastoma.
  • After a first chemotherapy that did not induce a reduction of the tumoral volume, a widened left nephrectomy was performed for the two patients.
  • The histologic examination of the two pieces of nephrectomy concluded to a fetal rhabdomyomatous nephroblastoma with existence in the second case of an extension of the lesions to the renal pelvis and ureter in the form of a pseudo-botryoïde tumor.
  • The tumor was classified stage I in the first case and stage II N0 in the second.
  • The treatment was completed by an adapted post operative chemotherapy according to the SIOP 9 protocol.
  • CONCLUSION: The fetal rhabdomyomatous nephroblastoma is a special histologic form of nephroblastoma that is characterized by the paucity of pulmonary metastasis, the absence of response to chemotherapy and the possibility of tumoral extension in the renal pelvis and ureter.
  • His prognosis is similar to the classical nephroblastoma.
  • [MeSH-major] Kidney Neoplasms. Wilms Tumor
  • [MeSH-minor] Female. Follow-Up Studies. Humans. Infant. Kidney / pathology. Male. Nephrectomy. Prognosis. Retrospective Studies. Time Factors

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  • (PMID = 12162188.001).
  • [ISSN] 0003-4401
  • [Journal-full-title] Annales d'urologie
  • [ISO-abbreviation] Ann Urol (Paris)
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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25. Abdallah FK, Macharia WM: Clinical presentation and treatment outcome in children with nephroblastoma in Kenya. East Afr Med J; 2001 Jul;78(7 Suppl):S43-7
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  • [Title] Clinical presentation and treatment outcome in children with nephroblastoma in Kenya.
  • RESULTS: Out of 803 children with cancer, 71 (8.8%) had histologically proven nephroblastoma.
  • At presentation, 1.5% were in stage I, 13.2% stage II, 36.8% stage III, 41.2% stage IV and 7.4% stage V.
  • Eighty five per cent presented with stage III-V disease.
  • Ninety five per cent had nepherectomy and received chemotherapy.
  • Nine patients died before commencement of chemotherapy, two of whom died in the immediate post-operative period.
  • Pre-operative chemotherapy was given to 42% of the patients.
  • Approximately 25.5% of the patients received little or no induction chemotherapy due to unavailability of drugs while only 2.8% received the prescribed maintenance treatment with the remainder getting erratic or no treatment.
  • Overall, only 34.7% remained disease free two years from time of diagnosis.
  • CONCLUSION: Late presentation, poor availability of cytotoxic drugs and frequent treatment interruptions for various reasons have contributed to the poor outcome of nephroblastoma in Kenya.
  • [MeSH-major] Kidney Neoplasms / therapy. Wilms Tumor / therapy
  • [MeSH-minor] Age Distribution. Child. Child, Preschool. Humans. Kenya / epidemiology. Remission Induction. Treatment Outcome

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  • (PMID = 11957249.001).
  • [ISSN] 0012-835X
  • [Journal-full-title] East African medical journal
  • [ISO-abbreviation] East Afr Med J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Kenya
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26. Myers JB, Dall'Era J, Odom LF, McGavran L, Lovell MA, Furness P 3rd: Teratoid Wilms' tumor, an important variant of nephroblastoma. J Pediatr Urol; 2007 Aug;3(4):282-6

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

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  • (PMID = 18947756.001).
  • [ISSN] 1873-4898
  • [Journal-full-title] Journal of pediatric urology
  • [ISO-abbreviation] J Pediatr Urol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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27. Glekov IV, Lebedev VI, Belova VP, Dzhabarov FR, Danilova VS, Boĭchenko EI, Sharoev TA: [Radiotherapy in multiple modality treatment of children with nephroblastoma]. Vestn Ross Akad Med Nauk; 2002;(1):68-71
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  • [Title] [Radiotherapy in multiple modality treatment of children with nephroblastoma].
  • [Transliterated title] Luchevaia terapiia v kompleksnom lechenii nefroblastomy u deteĭ.
  • The role of radiotherapy in multiple modality treatment of Wilms' tumor is evaluated in 225 children aged 3 months to 11.5 years (mean age 3.5 years) with stage III-IV.
  • 184 (81.8%) patients presented with stage III, 93.7% with typical nephroblastoma.
  • Intervention was combined with drug and radiotherapy in 99.6% patients.
  • Exposure of the abdominal cavity in total focal doses of 10.5-50.2 Gy (mean dose 28 Gy) was carried out in 219 (97.3%) of 225 patients.
  • Special attention is paid to the incidence of relapses, remote metastases, and survival of patients in relation to prognostic factors (sex, age, stage of tumor process, terms of exposure, and total focal doses).
  • Relapses were more incident during the first year of treatment (in 65% children) and outside the exposed field (72.5% cases).
  • 33.2% patients with stage III developed metastases after 1-49 months; the lungs were involved most often.
  • The absence of relationship between the incidence of local relapses and life span after exposure to a total focal dose of up to 21.6 Gy in comparison with higher doses recommends reduced doses for therapy without notable deterioration of the survival of patients with nephroblastoma.

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  • (PMID = 11882978.001).
  • [ISSN] 0869-6047
  • [Journal-full-title] Vestnik Rossiiskoi akademii meditsinskikh nauk
  • [ISO-abbreviation] Vestn. Akad. Med. Nauk SSSR
  • [Language] RUS
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
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28. Hadley GP, Sheik-Gafoor MH, Buckels NJ: The management of nephroblastoma with cavo-atrial disease at presentation: experience from a developing country. Pediatr Surg Int; 2010 Dec;26(12):1169-72
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  • [Title] The management of nephroblastoma with cavo-atrial disease at presentation: experience from a developing country.
  • PURPOSE: To describe the management of cavoatrial extension of nephroblastoma in a developing country.
  • PATIENTS AND METHODS: Of 406 consecutive children with Wilms tumour, 40 (10%) presented with tumour extension into the inferior vena cava (IVC) (30), right atrium or right ventricle and form the inception cohort.
  • RESULTS: Neoadjuvant chemotherapy appropriate to the pre-operative stage caused reduction in intravascular tumour in 18 (45%) patients, allowing 3 children who had atrial tumour at presentation to be surgically managed without recourse to cardiopulmonary bypass.
  • Neoadjuvant chemotherapy also provided a window of opportunity to assess and manage co-morbidity especially malnutrition.
  • 77% of resected specimen contained viable malignant cells despite one or more cycles of neoadjuvant chemotherapy.
  • Three of these children died of chemotherapy induced neutropaenic sepsis.
  • Four patients refused surgical treatment.
  • Twenty-three patients completed the post-operative treatment protocol (1,203 characters).
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Heart Atria. Heart Diseases / drug therapy. Kidney Neoplasms / drug therapy. Neoplastic Cells, Circulating / pathology. Thrombosis / drug therapy. Vena Cava, Inferior. Wilms Tumor / drug therapy
  • [MeSH-minor] Child. Child, Preschool. Developing Countries. Female. Humans. Infant. Male. Neoadjuvant Therapy


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

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  • (PMID = 16873431.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] England
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30. Boman F, Boccon-Gibod L: [Benign epithelial cells and Tamm-Horsfall protein in lymph nodes from nephrectomy specimens with nephroblastoma: a diagnostic pitfall]. Ann Pathol; 2004 Dec;24(6):632-6
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  • [Title] [Benign epithelial cells and Tamm-Horsfall protein in lymph nodes from nephrectomy specimens with nephroblastoma: a diagnostic pitfall].
  • [Transliterated title] Cellules épithéliales bénignes et protéine de Tamm-Horsfall dans les ganglions lymphatiques des pièces de néphrectomie pour néphroblastome: un piège diagnostique.
  • When a lymph node metastasis is discovered during the pathology examination of surgical specimens with nephroblastoma, the tumour is classified as stage III, according to the classification of the International Society of Paediatric Oncology (SIOP 2001), and post-operative intensive treatment of the patient includes irradiation and chemotherapy.
  • Benign epithelial cells in perinephric lymph nodes in cases of renal tumours in childhood may be confused with metastases.
  • This case illustrates latero-aortic lymph node complexes of benign epithelial cells and Tamm-Horsfall protein in a 16-month-old girl with surgical resection of a nephroblastoma after pre-operative chemotherapy.
  • The nephroblastoma was predominantly epithelial and was classified as SIOP stage I.
  • There were accumulations of Tamm-Horsfall protein in lymph node sinuses, in lymphatic vessels, in the kidney outside the tumour, and in the renal sinus.
  • This association of epithelial cells with deposits of Tamm-Horsfall protein and their resemblance to cells of the distal convoluted tubules favour a diagnosis of benignity.
  • A definitive diagnosis is supported by the small size of the cells, their bland nuclei, and morphological features differing from those of tumour cells, but deposits of Tamm-Horsfall protein may be associated with true lymph node metastases.
  • [MeSH-major] Kidney Neoplasms / pathology. Mucoproteins / analysis. Urothelium / pathology. Wilms Tumor / pathology
  • [MeSH-minor] Blood Group Antigens / analysis. Combined Modality Therapy. Female. Humans. Infant. Lymph Nodes / pathology. Nephrectomy. Uromodulin

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  • (PMID = 15785410.001).
  • [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 / Blood Group Antigens; 0 / Mucoproteins; 0 / UMOD protein, human; 0 / Uromodulin
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31. van den Hoek J, de Krijger R, van de Ven K, Lequin M, van den Heuvel-Eibrink MM: Cystic nephroma, cystic partially differentiated nephroblastoma and cystic Wilms' tumor in children: a spectrum with therapeutic dilemmas. Urol Int; 2009;82(1):65-70
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  • [Title] Cystic nephroma, cystic partially differentiated nephroblastoma and cystic Wilms' tumor in children: a spectrum with therapeutic dilemmas.
  • BACKGROUND: Cystic renal tumors are a diagnostic and therapeutic challenge.
  • Cystic nephroma (CN), cystic partially differentiated nephroblastoma (CPDN) and cystic Wilms' tumor (CWT) are a spectrum with CN at the benign end, CWT at the malignant end and CPDN in the intermediate position.
  • CN and stage 1 CPDN are often treated with surgery alone.
  • International Society of Pediatric Oncology (SIOP) protocols for Wilms' tumor (WT) advocate preoperative chemotherapy, which may be unnecessary and potentially harmful in CN and in stage 1 CPDN.
  • There are difficulties in differentiating the three types using imaging techniques.
  • Therefore, controversies exist regarding the optimal treatment.
  • METHODS: We describe 6 children, who each had a postoperative diagnosis of CN, CPDN or CWT, and we retrospectively evaluate the treatment strategies.
  • RESULTS: The three types cannot be differentiated using imaging techniques, although the presence of solid components in the tumor is indicative of WT.
  • CONCLUSIONS: Surgery as first-line therapy should be seriously considered in children who have a cystic renal tumor, but further collaborative studies are needed since the distinction line between CPDN and CWT is not always clear.
  • [MeSH-major] Kidney Diseases, Cystic / surgery. Kidney Neoplasms / surgery. Nephrectomy. Wilms Tumor / surgery
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols. Biopsy. Cell Differentiation. Chemotherapy, Adjuvant. Child, Preschool. Diagnosis, Differential. Disease Progression. Female. Humans. Infant. Magnetic Resonance Imaging. Male. Neoadjuvant Therapy. Neoplasm Staging. Retrospective Studies. Tomography, X-Ray Computed. Treatment Outcome. Ultrasonography

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  • [Copyright] (c) 2009 S. Karger AG, Basel.
  • (PMID = 19172100.001).
  • [ISSN] 1423-0399
  • [Journal-full-title] Urologia internationalis
  • [ISO-abbreviation] Urol. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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32. Camassei FD, Jenkner A, Ravà L, Bosman C, Francalanci P, Donfrancesco A, Alò PL, Boldrini R: Expression of the lipogenic enzyme fatty acid synthase (FAS) as a predictor of poor outcome in nephroblastoma: an interinstitutional study. Med Pediatr Oncol; 2003 May;40(5):302-8
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  • [Title] Expression of the lipogenic enzyme fatty acid synthase (FAS) as a predictor of poor outcome in nephroblastoma: an interinstitutional study.
  • BACKGROUND: Treatment of nephroblastoma (Wilms tumor) has presently achieved a greater than 80% cure rate.
  • Pathologic stage and grade are considered the most reliable prognostic parameters, but other biologic factors are under study in order to improve patient stratification into risk groups.
  • PROCEDURE: To determine the predictive strength of FAS expression in Wilms tumor (with particular emphasis on intermediate risk, i.e., non anaplastic tumors, the vast majority of nephroblastomas), we evaluated immunostaining expression in archival specimens from 94 neoplasms.
  • The degree of expression was correlated with stage, grade, clinical course and administration of prenephrectomy chemotherapy.
  • The blockade of fatty acid synthesis by inhibition of FAS enzymatic function by means of metabolic analogues might prove a novel target pathway for the treatment of nephroblastoma.
  • [MeSH-major] Fatty Acid Synthases / metabolism. Kidney Neoplasms / pathology. Wilms Tumor / pathology
  • [MeSH-minor] Adolescent. Biomarkers, Tumor. Child. Child, Preschool. Female. Humans. Immunohistochemistry. Infant. Male. Multivariate Analysis. Prognosis. Proportional Hazards Models. Retrospective Studies. Survival Analysis

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  • [Copyright] Copyright 2003 Wiley-Liss, Inc.
  • (PMID = 12652618.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; EC 2.3.1.85 / Fatty Acid Synthases
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33. Ghanem MA, Van Steenbrugge GJ, Van Der Kwast TH, Sudaryo MK, Noordzij MA, Nijman RJ: Expression and prognostic value Of CD44 isoforms in nephroblastoma (Wilms tumor). J Urol; 2002 Aug;168(2):681-6
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  • [Title] Expression and prognostic value Of CD44 isoforms in nephroblastoma (Wilms tumor).
  • We evaluate the prognostic value of standard CD44 (CD44s) and some of its isoforms in treating clinical Wilms tumor.
  • MATERIALS AND METHODS: The immunohistochemical expression of CD44 isoforms was studied in paraffin material of 61 clinical Wilms tumors.
  • Patients were treated preoperatively with chemotherapy and mean followup was 5.7 years.
  • RESULTS: Generally CD44s, CD44v5 and CD44v10 were expressed in normal kidney tissues and at variable levels in the 3 cell types of Wilms tumor (blastemal, epithelial and stromal).
  • No CD44v6 expression was found neither in normal kidney or in tumor tissue.
  • CD44s, CD44v5 and CD44v10 epithelial expression gradually decreased from stage T1 to T3.
  • Blastemal expression of CD44s and CD44v5 statistically significantly correlated with clinicopathological stage.
  • CONCLUSIONS: Increased expression of CD44v5 in the blastemal part of Wilms tumor correlated with tumor stage, clinical progression and tumor related death.
  • These patients might benefit from adjuvant chemotherapy and/or radiotherapy.
  • [MeSH-major] Antigens, CD44 / analysis. Kidney Neoplasms / pathology. Wilms Tumor / pathology

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  • (PMID = 12131349.001).
  • [ISSN] 0022-5347
  • [Journal-full-title] The Journal of urology
  • [ISO-abbreviation] J. Urol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD44; 0 / CD44v5 antigen; 0 / Protein Isoforms
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34. Owens CM, Veys PA, Pritchard J, Levitt G, Imeson J, Dicks-Mireaux C: Role of chest computed tomography at diagnosis in the management of Wilms' tumor: a study by the United Kingdom Children's Cancer Study Group. J Clin Oncol; 2002 Jun 15;20(12):2768-73
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  • [Title] Role of chest computed tomography at diagnosis in the management of Wilms' tumor: a study by the United Kingdom Children's Cancer Study Group.
  • PURPOSE: This study sought to determine whether the identification of minimal pulmonary metastatic disease by chest computed tomography (CT) performed at diagnosis in patients with Wilms' tumor and normal chest x-rays (CXR) could predict a subgroup of children at increased risk of pulmonary relapse.
  • PATIENTS AND METHODS: A retrospective analysis was carried out of the records of 449 children entered onto the United Kingdom Childrens' Cancer Study Group Second Wilms' Tumor Study between July 1986 and September 1991.
  • After surgery, children with stage I Wilms' tumor received single-agent chemotherapy (vincristine), whereas children with stages II, III, and bilateral Wilms' tumor received combination chemotherapy.
  • Most children with stage III tumors were also treated with abdominal radiotherapy (20 Gy).
  • When only stage I patients were analyzed, there was a significant difference between the pulmonary relapse rate of 43% (three of seven) in the CT-positive group and 10% (five of 48) in the CT-negative group (P =.02).
  • Four of eight patients with stage I disease with pulmonary relapse died.
  • CONCLUSION: CT seemed to identify a subgroup of stage I patients who were at increased risk of pulmonary relapse.
  • These children had received only single-agent chemotherapy.
  • A prospective randomized trial is needed to clarify whether these children would benefit from combination chemotherapy.
  • [MeSH-major] Kidney Neoplasms / pathology. Lung Neoplasms / radiography. Lung Neoplasms / secondary. Neoplasm Staging. Tomography, X-Ray Computed. Wilms Tumor / radiography. Wilms Tumor / secondary
  • [MeSH-minor] Antineoplastic Agents, Phytogenic / therapeutic use. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Female. Humans. Infant. Infant, Newborn. Male. Predictive Value of Tests. Prognosis. Recurrence. Retrospective Studies. Risk Factors. Vincristine / therapeutic use

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  • [CommentIn] J Clin Oncol. 2002 Jun 15;20(12):2763-4 [12065550.001]
  • (PMID = 12065552.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 5J49Q6B70F / Vincristine
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35. Burgos L, Martínez Urrutia MJ, Lobato R, López Santamaría M, Aroca A, Jaureguízar E: [Strategy for high cavoatrial tumor thrombus in children with nephroblastoma]. Cir Pediatr; 2008 Apr;21(2):70-2
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  • [Title] [Strategy for high cavoatrial tumor thrombus in children with nephroblastoma].
  • [Transliterated title] Tratamiento del nefroblastoma con trombosis tumoral cavoatrial en niños.
  • INTRODUCTION: Wilms tumor surgery with intracaval-atrial extension is a challenge.
  • Nine of them had renal-caval (RC) or cavo-atrial(CA) thrombus extension.
  • RESULTS: All cases were treated with pre-postoperative chemotherapy SIOP protocols.
  • A multidisciplinary team was necessary to plan surgery and in all patients thrombus and tumor could be removed under cardiopulmonary by-pass in 3 cases (CPBP).
  • Three cases were stage III, one stage IV and 1 stage II.
  • Conclusions. Tumor size can be significantly reduced by preoperative chemotherapy.
  • [MeSH-major] Heart Diseases / etiology. Kidney Neoplasms / pathology. Neoplastic Cells, Circulating. Vena Cava, Superior. Wilms Tumor / secondary

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  • (PMID = 18624272.001).
  • [ISSN] 0214-1221
  • [Journal-full-title] Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
  • [ISO-abbreviation] Cir Pediatr
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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36. Godziński J, Weirich A, Tournade MF, Gauthier F, Buerger D, Moorman-Voestermans CG, de Kraker J, Voute P, Ludwig R, Sawicz-Birkowska K, Vujanić G, Ducourtieux M: Primary nephrectomy for emergency: a rare event in the International Society of Paediatric Oncology Nephroblastoma Trial and Study no. 9. Eur J Pediatr Surg; 2001 Feb;11(1):36-9
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  • [Title] Primary nephrectomy for emergency: a rare event in the International Society of Paediatric Oncology Nephroblastoma Trial and Study no. 9.
  • Experience of the International Society of Paediatric Oncology (SIOP) Trials and Studies indicates that the preoperative chemotherapy in Wilms' tumour improves stage distribution, decreases complication rate and reduces postoperative treatment.
  • Records of 720 patients with non-metastatic unilateral nephroblastoma who were registered in the SIOP Trial and Study 9 were reviewed.
  • Postoperative treatment included radiotherapy in 71% of cases and anthracyclines in 92%.
  • [MeSH-major] Kidney Neoplasms / surgery. Nephrectomy. Wilms Tumor / surgery

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  • (PMID = 11370981.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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37. Weirich A, Ludwig R, Graf N, Abel U, Leuschner I, Vujanic GM, Mehls O, Boos J, Beck J, Royer-Pokora B, Voûte PA: Survival in nephroblastoma treated according to the trial and study SIOP-9/GPOH with respect to relapse and morbidity. Ann Oncol; 2004 May;15(5):808-20
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  • [Title] Survival in nephroblastoma treated according to the trial and study SIOP-9/GPOH with respect to relapse and morbidity.
  • BACKGROUND: Recent Wilms' tumor (WT) trials and studies have tried to determine the minimal therapy needed for cure.
  • PATIENTS AND METHODS: From January 1989 to March 1994 the German Society of Pediatric Oncology and Hematology registered 440 patients (median age 2.9 years; 231 male, 209 female) with WTs (preoperative chemotherapy 362) for therapy according to the International Society of Pediatric Oncology Trial and Study 9.
  • Therapy for relapse depended on site of relapse and therapy already received.
  • In preoperatively treated WTs, anaplasia was a strong prognostic factor for death [relative risk (RR) 4.7], followed by poor response to preoperative therapy (RR 3.6), stage IV (RR 3.2) and abdominal stage III (RR 2.2).
  • In the 334 unilateral stage I-IV WTs (median age 3.2 years), diffuse anaplasia (21 patients) had a 5-year relapse-free survival of 38.1%, versus 58.4% in blastemal WTs (25 patients); survival was 42.9% in diffuse anaplasia versus 84% in blastemal WTs.
  • None of 46 patients (median age 1.9 years; 91.3% stages I or II) with differentiated WTs (nine epithelial, 37 stromal) relapsed despite their non-response; two died (one therapy related, one due to bilaterization).
  • In the 25 non-anaplastic bilateral WTs, differentiated cases (one epithelial, eight stromal, 33.3% abdominal stage III) were more frequent (P = 0.048) than in unilateral WTs (one stromal, abdominal stage III relapsed).
  • Three WTs developed renal failure due to Drash syndrome, but none due to tumor therapy.
  • After adriamycin 1.9% of WTs (9% of those receiving 400 mg/m(2)) required therapy for cardiac toxicity.
  • CONCLUSIONS: Initial therapy should be more individualized, taking the above risk groups (age in non-anaplastic WTs, poor response, anaplasia, etc.) into account, as morbidity even after relapse therapy with ifosfamide, carboplatin and etoposide was not high.
  • Milder therapy in low stages of differentiated and of well responding WTs should be tested.

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  • (PMID = 15111352.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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38. Stefanowicz J, Sierota D, Połczyńska K, Balcerska A, Stoba C: [Stage IV Wilms' tumour -- analysis of treatment results, according to the SIOP 93-01 protocol, at the Medical University of Gdańsk]. Med Wieku Rozwoj; 2004 Apr-Jun;8(2 Pt 1):193-6
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  • [Title] [Stage IV Wilms' tumour -- analysis of treatment results, according to the SIOP 93-01 protocol, at the Medical University of Gdańsk].
  • THE AIM OF THE STUDY: To evaluate the results of treatment in patients with Wilm's' tumor in stage IV at the Gdansk Medical University Centre.
  • MATERIAL AND METHODS: Between 1993 and 2002, 44 children with nephroblastoma were treated according to SI0P 93-01 protocol, in the Department of Paediatrics, Haematology, Oncology and Endocrinology of the Medical University of Gdansk.
  • Stage IV tumour was confirmed in 6 children: 3 boys and 3 girls.
  • RESULTS: After preoperative chemotherapy complete regression of metastases was obtained in 3 patients.
  • In l patient there was no effect of preoperative treatment.
  • In all patients nephroblastoma of intermediate histological risk was diagnosed.
  • Among the 6 analysed patients with stage IV of the disease, 5 children are alive.
  • Time of observation is from 42 to 102 months.
  • 1 child died from treatment complications: necrotising enteritis.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Wilms Tumor / drug therapy. Wilms Tumor / pathology
  • [MeSH-minor] Child. Child, Preschool. Dactinomycin / administration & dosage. Disease-Free Survival. Female. Humans. Infant. Male. Neoplasm Staging. Poland. Prognosis. Remission Induction. Retrospective Studies. Risk Factors. Time Factors. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 15738593.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; SIOP protocol
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39. Hero B, Hunneman DH, Gahr M, Berthold F: Evaluation of catecholamine metabolites, mIBG scan, and bone marrow cytology as response markers in stage 4 neuroblastoma. Med Pediatr Oncol; 2001 Jan;36(1):220-3
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  • [Title] Evaluation of catecholamine metabolites, mIBG scan, and bone marrow cytology as response markers in stage 4 neuroblastoma.
  • BACKGROUND: The early biological response has been proved an excellent predictor in acute lymphoblastic leukemia and nephroblastoma.
  • PROCEDURE: Three hundred sixty-seven unselected stage 4 neuroblastoma patients treated according the German cooperative trial NB90 were entered into the study.
  • The outcome of patients with normalized mIBG scan after four courses of chemotherapy [5 year EFS (event free survival) 0.22 +/- 0.07] was not superior to the outcome of patients with still abnormal uptake (5 year EFS 0.30 +/- 0.05).
  • Urinary catecholamine normalization after four cycles of chemotherapy (5 year EFS 0.35 +/- 0.06 versus 0.26 +/- 0.10) had no influence on outcome, whereas plasma catecholamine normalization after the first (5 year EFS 0.40 +/- 0.09 versus 0.14 +/- 0.07, P= 0.0364) or the fourth cycle (5 year EFS 0.35 +/- 0.06 versus 0.26 +/- 0.10, P = 0.0242) indicated a better outcome.
  • [MeSH-major] 3-Iodobenzylguanidine. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / analysis. Bone Marrow Examination. Homovanillic Acid / analysis. Iodine Radioisotopes. Neuroblastoma / therapy. Radiopharmaceuticals. Vanilmandelic Acid / analysis
  • [MeSH-minor] Chemotherapy, Adjuvant. Cisplatin / administration & dosage. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Dacarbazine / administration & dosage. Disease-Free Survival. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Humans. Ifosfamide / administration & dosage. Life Tables. Melphalan / administration & dosage. Neoplasm Staging. Radiotherapy, Adjuvant. Survival Analysis. Treatment Outcome. Vincristine / administration & dosage. Vindesine / administration & dosage

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  • (PMID = 11464889.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Evaluation Studies; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Iodine Radioisotopes; 0 / Radiopharmaceuticals; 35MRW7B4AD / 3-Iodobenzylguanidine; 55-10-7 / Vanilmandelic Acid; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 7GR28W0FJI / Dacarbazine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin; Q41OR9510P / Melphalan; RSA8KO39WH / Vindesine; UM20QQM95Y / Ifosfamide; X77S6GMS36 / Homovanillic Acid
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40. Sawicz-Birkowska K, Czernik J, Bagłaj M, Czauderna P, Kantorowicz-Szymik S, Poznański WA, Mańkowski P, Madziara W, Prokurat A, Osemlak J: [Renal neoplasms in children]. Przegl Lek; 2004;61 Suppl 2:20-3
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  • [Title] [Renal neoplasms in children].
  • Nephroblastoma is the most common kidney tumor in Polish children.
  • OBJECTIVE: to present clinical material and outcome of 533 children with renal tumors.
  • MATERIAL: 500 pts with nephroblastoma and 33 of non-Wilms: CMN, RCC,CSSK, RTK and others tumors were registered, mean age 4.5 years between 1993 till 2002.
  • Stage: CS I--148, CS II--191, CS III--114, CS IV--51, CS V--29 pts.
  • All pts with nephroblastoma were treated according to the first national PPGGL 01-92 protocol with pre-operative chemotherapy (ACT, VCR) for CS I-III and ACT, VCR, DOX in pts of stage IV, over the age of 6 months.
  • Pre-operative chemotherapy was done to 93.8% pts.
  • RESULTS: Radical nephrectomy post pre op chemotherapy was performed in 451 (98%) pts over 6 months and in 44 (8.2%) infants less than 6 months with nephroblastoma.
  • Partial nephrectomy for unilateral tumor post preoperative chemotherapy was made in 6 (1.2%).
  • In 26/29 (89.65%) of CS V nephroblastoma kidney sparing surgery was possible, and in 12 uni-lateral nephrectomy was performed.
  • The results of treatment of 33 pts with non-Wilms renal tumors have improved lately.
  • CONCLUSIONS: The use of systemic neoadjuvant chemotherapy in all pts over 6 months according to the recommendation of SIOP Nephroblastoma protocol (01-92) produced tumor shrinkage, facilitated complete surgical nephrectomy, and was very advantageous in the treatment of renal tumors in children.
  • The results of treatment of non-Wilms tumor have also improved thanks to introduction of new and more aggressive regimens of chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Kidney Neoplasms / surgery. Nephrectomy. Wilms Tumor / drug therapy. Wilms Tumor / surgery
  • [MeSH-minor] Adolescent. Chemotherapy, Adjuvant. Child. Child, Preschool. Disease-Free Survival. Female. Humans. Infant. Infant, Newborn. Male. Neoadjuvant Therapy. Neoplasm Staging. Poland. Prognosis. Remission Induction. Retrospective Studies. Risk Factors. Survival Analysis. Treatment Outcome

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  • (PMID = 15686041.001).
  • [ISSN] 0033-2240
  • [Journal-full-title] Przegla̧d lekarski
  • [ISO-abbreviation] Prz. Lek.
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
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41. Kremens B: [Systemic therapy in children and adolescents]. Urologe A; 2007 Oct;46(10):1404-6

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  • [Title] [Systemic therapy in children and adolescents].
  • Urologic malignancies in childhood and adolescence are mainly nephroblastomas, neuroblastomas, soft tissue sarcomas, and germ cell tumors.
  • National and supranational treatment studies are the standard of care for pediatric cancer in Germany; they yield 5-year survival rates of almost 90% for nephroblastoma and germ cell tumors and 60% for neuroblastoma (all stages) and rhabdomyosarcoma.
  • The principles of antineoplastic therapy are the same as in adult cancer medicine; the drugs used depend upon the disease.
  • In a multimodal treatment strategy, the role of chemotherapy as well as that of surgery and radiotherapy can differ, as is described for nephroblastoma, infant neuroblastoma, and stage 4 neuroblastoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Neoadjuvant Therapy. Urogenital Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Adrenal Gland Neoplasms / drug therapy. Adrenal Gland Neoplasms / mortality. Adrenal Gland Neoplasms / pathology. Adrenal Gland Neoplasms / surgery. Adrenal Medulla. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Humans. Infant. Kidney Neoplasms / drug therapy. Kidney Neoplasms / mortality. Kidney Neoplasms / pathology. Kidney Neoplasms / surgery. Neoplasm Staging. Neoplasms, Germ Cell and Embryonal / drug therapy. Neoplasms, Germ Cell and Embryonal / mortality. Neoplasms, Germ Cell and Embryonal / pathology. Neoplasms, Germ Cell and Embryonal / surgery. Neuroblastoma / drug therapy. Neuroblastoma / mortality. Neuroblastoma / pathology. Neuroblastoma / surgery. Prognosis. Radiotherapy, Adjuvant. Rhabdomyosarcoma / drug therapy. Rhabdomyosarcoma / mortality. Rhabdomyosarcoma / pathology. Rhabdomyosarcoma / surgery. Survival Rate. Wilms Tumor / drug therapy. Wilms Tumor / mortality. Wilms Tumor / pathology. Wilms Tumor / surgery

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  • (PMID = 17823786.001).
  • [ISSN] 0340-2592
  • [Journal-full-title] Der Urologe. Ausg. A
  • [ISO-abbreviation] Urologe A
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
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42. Vujanić GM, Sandstedt B, Harms D, Kelsey A, Leuschner I, de Kraker J, SIOP Nephroblastoma Scientific Committee: Revised International Society of Paediatric Oncology (SIOP) working classification of renal tumors of childhood. Med Pediatr Oncol; 2002 Feb;38(2):79-82
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  • [Title] Revised International Society of Paediatric Oncology (SIOP) working classification of renal tumors of childhood.
  • The previous International Society of Paediatric Oncology (SIOP) trials and studies recognized three prognostic groups of renal tumors of childhood: low risk, intermediate risk, and high risk tumors, which were further defined in the SIOP (Stockholm) Working Classification of Renal Tumors of Childhood (1994).
  • The results of the latest SIOP Trials and Studies showed that certain histological features which remain after preoperative chemotherapy, such as blastema, are of prognostic significance while others are not.
  • Therefore, in the next SIOP Trials and Study a revised classification of renal tumors will be followed.
  • It still recognizes the three tumor risk groups with different types in each of them, but for treatment purposes, only three major types of nephroblastoma need to be recognized: completely necrotic (low risk tumor), blastemal (high risk tumor), and others (intermediate risk tumors).
  • Patients will be treated according to tumor histology and stage.
  • Trials which include preoperative chemotherapy have shown that the presence of necrotic tumor or chemotherapy induced changes in the renal sinus or perirenal fat can be ignored for distinguishing between stage I and II, but if present at resection margins or lymph nodes, it should be regarded as stage III.
  • Prognostic significance of all histological component of Wilms tumors will be studied prospectively in the new trial.
  • [MeSH-major] Kidney Neoplasms / pathology. Wilms Tumor / pathology

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  • [Copyright] Copyright 2002 Wiley-Liss, Inc.
  • [CommentIn] Med Pediatr Oncol. 2003 Jul;41(1):102 [12764768.001]
  • [CommentIn] Med Pediatr Oncol. 2002 Feb;38(2):77-8 [11813169.001]
  • (PMID = 11813170.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Genetic Markers
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43. Ahmed R, al-Salti W, Raafat F, Morland B: Metachronous Wilms tumor associated with pulmonary embolism: how can we detect these cases early? A case report and literature review. Pediatr Hematol Oncol; 2003 Jan-Feb;20(1):55-63
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  • [Title] Metachronous Wilms tumor associated with pulmonary embolism: how can we detect these cases early? A case report and literature review.
  • A 4-year-old girl developed right metachronous Wilms tumor 2 years after completing treatment for a left-sided stage I Wilms tumor.
  • The original treatment included 7 weeks of chemotherapy, delayed nephrectomy, and another 3 weeks of chemotherapy.
  • The metachronous tumor on the right side extended into the inferior vena cava and right atrium.
  • She developed pulmonary embolism as a result.
  • She received chemotherapy and developed liquifaction of the tumor and toxic shock.
  • [MeSH-major] Neoplasms, Second Primary / complications. Pulmonary Embolism / etiology. Wilms Tumor / complications
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child, Preschool. Female. Humans. Kidney Neoplasms / pathology. Kidney Neoplasms / surgery. Nephrectomy

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  • (PMID = 12687754.001).
  • [ISSN] 0888-0018
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 5
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44. 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]).
  • The assessment criterion was the observed percentage of stage I tumors.
  • 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).
  • RESULTS: No advantage was found in favor of prolonged preoperative treatment.
  • The percentages obtained for the 4-week and the 8-week groups, respectively, were as follows: stage I, 64% versus 62%; intraoperative tumor rupture rate, 1% versus 3%; 2-year EFS, 84% versus 83%; and 5-year OS, 92% versus 87%.
  • 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%.
  • The rate of abdominal recurrences in stage II N0 nonirradiated patients was 6.6%.
  • 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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45. Uba AF, Chirdan LB: Childhood Wilms' tumour: prognostic factors in North Central Nigeria. West Afr J Med; 2007 Jul-Sep;26(3):222-5
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  • [Title] Childhood Wilms' tumour: prognostic factors in North Central Nigeria.
  • BACKGROUND: Childhood Wilms' tumour or nephroblastoma represents one of the challenges for paediatric oncologists in developing countries.
  • OBJECTIVE: To review the clinical characteristics and outcome of management of childhood nephroblastoma in North- Central Nigeria.
  • METHODS: The clinical, operative and histological records of children aged 15 years and below, that were managed for Wilms' tumour at the Jos University Teaching Hospital between 1998 and 2005 were retrospectively reviewed.
  • RESULTS: There were 32 children (M:F=1.9:1) with histologically confirmed nephroblastoma seen over the 7-year period.
  • At presentation, 1 (3.1%) patient was in stage I, 8 (25%) stage II, 11 (34.4%) stage III and 12 (37.5%) stage IV.
  • About 72% of the patients presented with stage III-IV disease.
  • Nephrectomy and chemotherapy were the modality of treatment.
  • Fifteen (46.9%) of the patients received little or no induction chemotherapy due to unavailability of drugs while only 12 (37.5%) received the prescribed maintenance treatment with the remainder getting erratic or no treatment.
  • Overall, only 43.8% were alive between 1 and 9 months (median: 6 months) of follow-up period, but there was no survivor at two years after treatment.
  • CONCLUSION: Childhood nephroblastoma has a high mortality rate in north central Nigeria because of late clinical presentation with advanced disease, poor availability of cytotoxic drugs and frequent interruptions in treatment and inadequate follow-up.
  • [MeSH-major] Treatment Outcome. Wilms Tumor / diagnosis
  • [MeSH-minor] Adolescent. Antibiotics, Antineoplastic / therapeutic use. Child. Child, Preschool. Disease Progression. Doxorubicin / therapeutic use. Female. Humans. Infant. Male. Nephrectomy. Nigeria / epidemiology. Prognosis. Retrospective Studies. Time Factors. Vincristine / therapeutic use

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  • (PMID = 18399339.001).
  • [ISSN] 0189-160X
  • [Journal-full-title] West African journal of medicine
  • [ISO-abbreviation] West Afr J Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Nigeria
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin
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46. Calvo Rodríguez M, Fírvida Pérez JL, Vega Vázquez F, Salgado Fernández M, García Mata J, Rubén Rodríguez M, Barreiro Mouro A: [Wilms tumor in the adult]. Arch Esp Urol; 2001 May;54(4):370-4
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  • [Title] [Wilms tumor in the adult].
  • [Transliterated title] Tumor de Wilms del adulto.
  • OBJECTIVE: To report a case of Wilms' tumor in an adult patient.
  • METHODS: The records of an adult patient with renal tumor is reviewed.
  • Physical examination and patient assessment by ultrasound and CT showed a solid tumor in the right kidney.
  • Pathological analysis demonstrated a biphasic nephroblastoma (Wilms' tumor) with infiltration of renal hilar fat (stage II).
  • After surgery, adjuvant chemotherapy with vincristine-actinomycin D was administered for 60 weeks.
  • CONCLUSIONS: Although rare in adults, Wilms' tumor should be included in the differential diagnosis of all renal tumors.
  • Treatment is usually by surgery and chemotherapy with or without radiotherapy, depending on tumor stage.
  • [MeSH-major] Kidney Neoplasms / pathology. Wilms Tumor / pathology

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  • (PMID = 11455774.001).
  • [ISSN] 0004-0614
  • [Journal-full-title] Archivos españoles de urología
  • [ISO-abbreviation] Arch. Esp. Urol.
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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47. Lucas S, Reindl T, Henze G, Kurtz A, Sakuma S, Driever PH: Increased midkine serum levels in pediatric embryonal tumor patients. J Pediatr Hematol Oncol; 2009 Oct;31(10):713-7

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  • [Title] Increased midkine serum levels in pediatric embryonal tumor patients.
  • We analyzed sera of pediatric tumor patients in comparison to a large number of children and adolescents without malignant disease.
  • MK was studied in sera of 152 noncancer patients and 29 embryonal tumor patients (14 nephroblastoma, 10 neuroblastoma, and 5 rhabdomyosarcoma) using an enzyme-linked immunosorbent assay.
  • MK serum levels were significantly higher in tumor patients (median 0.621 ng/mL) than in noncancer patients.
  • About 86% of tumor patients were identified using a cut-off value of 0.176 ng/mL.
  • MK values did neither correlate with tumor size nor with stage or histology, but decreased in half of the nephroblastoma patients after chemotherapy and surgery.
  • MK may serve as an additional marker for the detection of pediatric embryonal tumors, but its clinical relevance for the evaluation of response to therapy needs further study.
  • [MeSH-minor] Adolescent. Biomarkers / blood. Case-Control Studies. Child. Child, Preschool. Cytokines / blood. Enzyme-Linked Immunosorbent Assay. Female. Humans. Infant. Male. Neuroblastoma / blood. Neuroblastoma / diagnosis. Neuroblastoma / therapy. Rhabdomyosarcoma / blood. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / therapy. Wilms Tumor / blood. Wilms Tumor / diagnosis. Wilms Tumor / therapy. Young Adult

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  • (PMID = 19727009.001).
  • [ISSN] 1536-3678
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers; 0 / Cytokines; 0 / MDK protein, human; 0 / Nerve Growth Factors; 137497-38-2 / midkine
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48. Watanabe R, Takahashi A, Suzuki M, Toki F, Kanazawa T, Hirato J, Morikawa A, Kuwano H: Adolescent wilms tumor with intraspinal and bone metastases: a case report and the review of literature. J Pediatr Hematol Oncol; 2009 Jan;31(1):45-8
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  • [Title] Adolescent wilms tumor with intraspinal and bone metastases: a case report and the review of literature.
  • A 14-year-old girl was referred for a large tumor of the left kidney, with intraspinal and vertebral metastases.
  • Left nephrectomy and intraspinal tumor resection were performed.
  • The histology of both tumors was nephroblastoma with no anaplasia and favorable histology, and they were diagnosed as stage IV.
  • The tumor bed and vertebras were irradiated.
  • We started chemotherapy according to the DD-4A regimen of Japanese Wilms' Tumor Study Group.
  • Intensive multimodality therapy including DD-4A regimen of National Wilms' Tumor Study can result in long-term disease-free remission.
  • [MeSH-major] Bone Neoplasms / secondary. Kidney Neoplasms / pathology. Spinal Neoplasms / secondary. Wilms Tumor / secondary
  • [MeSH-minor] Adolescent. Combined Modality Therapy. Female. Humans. Magnetic Resonance Imaging. Neoplasm Staging

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  • (PMID = 19125087.001).
  • [ISSN] 1536-3678
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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49. Hadley GP, Govender D, Landers G: Malignant solid tumours in neonates: an African perspective. Pediatr Surg Int; 2002 Dec;18(8):653-7

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  • Neuroblastoma (NB) was the commonest tumour seen (11), but the soft-tissue sarcomas were the dominant group (14).
  • Chemotherapy, despite appropriate dose reduction, had significant morbidity and mortality.
  • Stage I disease was associated with a good prognosis, whilst stage IV disease was uniformly fatal.
  • Stage IVs disease had only 50% early survival.
  • Patients with renal tumours, whether nephroblastoma or mesoblastic nephroma, did well.
  • [MeSH-minor] Female. Humans. Infant, Newborn. Male. Neoplasm Staging. South Africa / epidemiology. Survival Analysis. Treatment Outcome

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  • (PMID = 12598957.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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50. 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.
  • Among patients with Wilms' tumors (WT), nephroblastoma (NB) of intermediate risk predominated (73%; 46 of 63 pats.).
  • 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.
  • Comparing relapse-free survival of stages I, II and III, respectively, there was a reduced survival rate for stage III (p=0.019).
  • According to the SIOP/GPOH protocol in 1989, the regimen was switched from primary surgery to preoperative chemotherapy without biopsy in 1989 (11 pats.).
  • 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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51. Lamalmi N, Rouas L, Cherradi N, Malihy A, Khattab M, Alhamany Z: [Botryoid Wilms tumor extending into the duodenum]. Arch Pediatr; 2010 Dec;17(12):1664-6
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  • [Title] [Botryoid Wilms tumor extending into the duodenum].
  • We report on a rare case of botryoid Wilms tumor extending into the duodenum.
  • This uncommon macroscopic form of nephroblastoma consists entirely of a polypoid renal intrapelvic mass.
  • The main differential diagnosis of this unusual tumor is botryoid rhabdomyosarcoma.
  • Radiology revealed a large heterogeneous mass in the renal calyx, protruding into the ureter.
  • The tumor was found to extend into the 2nd portion of the duodenum.
  • The pathologic diagnosis was mixed type nephroblastoma, SIOP 2001 stage III.
  • The patient was given a course of postoperative chemotherapy.
  • To our knowledge, botryoid nephroblastoma has not previously been described as extending into the digestive system.
  • [MeSH-major] Duodenal Neoplasms / diagnosis. Kidney Neoplasms / diagnosis. Neoplasms, Multiple Primary / diagnosis. Ureteral Neoplasms / diagnosis. Wilms Tumor / diagnosis
  • [MeSH-minor] Chemotherapy, Adjuvant / methods. Diagnosis, Differential. Humans. Infant. Male. Neoplasm Invasiveness. Neoplasm Staging. Nephrectomy. Rhabdomyosarcoma / diagnosis. Treatment Outcome

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  • [Copyright] Copyright © 2010 Elsevier Masson SAS. All rights reserved.
  • (PMID = 20943355.001).
  • [ISSN] 1769-664X
  • [Journal-full-title] Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
  • [ISO-abbreviation] Arch Pediatr
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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52. Cozzi DA, Schiavetti A, Morini F, Castello MA, Cozzi F: Nephron-sparing surgery for unilateral primary renal tumor in children. J Pediatr Surg; 2001 Feb;36(2):362-5
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  • [Title] Nephron-sparing surgery for unilateral primary renal tumor in children.
  • PURPOSE: Definition of the role of nephron-sparing surgery (NSS) in the treatment of children with primary unilateral renal tumor (URT).
  • Criteria for selection of patients eligible for NSS were at least 50% of affected kidney preservable and stage I at surgery (frozen section biopsies from regional lymph nodes, perirenal fat, and surrounding renal parenchyma).
  • Preoperative 2-drug chemotherapy was given to all patients more than 6 months of age.
  • Between 1992 and 1995, 3-drug chemotherapy was used after NSS.
  • Thereafter, following NSS, 2-drug chemotherapy was given if no microscopic residual disease was found on final histologic examination.
  • Seven children had standard histology nephroblastoma, 1 highly differentiated epithelial type nephroblastoma, 1 oncocytoma, and 1 cystic nephroma.
  • CONCLUSION: NSS should be considered in selected children with URT, especially in patients with increased risk for metachronous tumor or renal disease, and in patients with benign or low-grade malignant URT.

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  • (PMID = 11172435.001).
  • [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
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53. Stefanowicz J, Sierota D, Balcerska A, Stoba C: [Wilms' tumour of unfavorable histology--results of treatment with the SIOP 93-01 protocol at the Gdańsk centre. Preliminary report]. Med Wieku Rozwoj; 2004 Apr-Jun;8(2 Pt 1):197-200
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  • [Title] [Wilms' tumour of unfavorable histology--results of treatment with the SIOP 93-01 protocol at the Gdańsk centre. Preliminary report].
  • BACKGROUND: Wilms' tumour is the most common primary renal tumour of childhood.
  • The aim or the study was to evaluate the results of treatment in patients with unfavourable histology Wilms' tumour.
  • MATERIAL AND METHODS: 44 children were treated for nephroblastoma (Wilms' tumour) according to the SIOP 93-01 protocol in the Department of Paediatrics. Haematology.
  • Among these 2 cases of nephroblastoma with diffuse anaplasia, 6 with sarcomatous stroma and 2 cases with clear cell sarcoma of the kidney were diagnosed.
  • In one patient file tumour structure was undefined due to complete necrosis of the tumour tissue.
  • The staging stratification was as follows: stage I-l, II N (-)-l, II N (+) -3, III- 6, including a neoplasmic occluding thrombus in the inferior vena cava, IV - 0, V-0.
  • Fatal outcome was attributed in one case to cancer progression (primary resistance to chemotherapy after 14 months of treatment) and in the other, to the treatment complications (fungal sepsis after undergoing the third course of chemotherapy Carbo+ VP-16).
  • In the analysed group there were no patients with stage IV disease, no relapses were observed.
  • CONCLUSIONS: our observations support the opinion, that high-grade malignancy histology is the basic unfavourable prognostic factor in nephroblastoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Wilms Tumor / drug therapy. Wilms Tumor / pathology
  • [MeSH-minor] Carcinosarcoma / drug therapy. Carcinosarcoma / pathology. Child. Child, Preschool. Dactinomycin / administration & dosage. Female. Humans. Infant. Male. Neoplasm Staging. Poland. Prognosis. Remission Induction. Retrospective Studies. Risk Factors. Sarcoma, Clear Cell / drug therapy. Sarcoma, Clear Cell / pathology. Survival Analysis. Time Factors. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 15738594.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Poland
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; SIOP protocol
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54. Mekki M, Landolsi A, Belghith M, Krichene I, Chelly S, Zakhama A, Jouini R, Golli M, Gahbiche M, Nouri A: [Malignant renal tumors before one year of age. Experience of a North African pediatric surgery service]. Ann Urol (Paris); 2003 Oct;37(5):239-43
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  • [Title] [Malignant renal tumors before one year of age. Experience of a North African pediatric surgery service].
  • The malignant tumours of the kidney are not very frequent during the first year of life and pose diagnostic and therapeutic problems.
  • The aim of this work is to make an analysis of the epidemiologic, clinical and anatomo-pathological characteristics of these tumours during the first year of life and a development on the therapeutic methods and their results.
  • First chemotherapy was prescribed to five patients.
  • The tumour corresponded to a nephroblastoma in seven cases and a rhabdoide tumour in the last case.
  • Mortality was high (50%) caused by the toxicity of chemotherapy in three cases and an advanced stage of cancer in one case.
  • CONCLUSION: Renal tumoral pathology occurring in infants less than one year of age poses true etiologic and therapeutic problems.
  • The high frequency of the nephroblastoma and the absence of benign tumour in our series encourage us to evocate more often the malignant renal tumours and to practice per cutaneous biopsies in case of diagnostic doubt.

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  • (PMID = 14606310.001).
  • [ISSN] 0003-4401
  • [Journal-full-title] Annales d'urologie
  • [ISO-abbreviation] Ann Urol (Paris)
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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55. 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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56. Reinhard H, Furtwängler R, Siemer S, Wullich B, Graf N: [Wilms' tumor in adults]. Urologe A; 2007 Jul;46(7):748-53
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  • [Title] [Wilms' tumor in adults].
  • [Transliterated title] Wilms-Tumor bei Erwachsenen.
  • BACKGROUND: Wilms' tumor (nephroblastoma) is the most frequent renal tumor in childhood.
  • In contrast nephroblastoma in adults is rare, and the disease used to have a poor prognosis.
  • PATIENTS AND METHODS: Of 1,300 registered patients, a total of 41 patients older than 16 years were enrolled in the pediatric nephroblastoma trial from 1994 to 2005.
  • Treatment was given according to the pediatric protocol.
  • RESULTS: The adults had higher local stages, more frequent metastasis, and developed more toxicity due to therapy.
  • Patients with local stage I and II have an event-free survival of 84%.
  • CONCLUSION: Adults with nephroblastoma have a very good prognosis if treated according to a pediatric protocol.
  • [MeSH-major] Kidney Neoplasms / drug therapy. Kidney Neoplasms / epidemiology. Risk Assessment / methods. Vincristine / therapeutic use. Wilms Tumor / drug therapy. Wilms Tumor / epidemiology
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents, Phytogenic / therapeutic use. Female. Germany / epidemiology. Humans. Male. Middle Aged. Prevalence. Risk Factors. Treatment Outcome

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  • (PMID = 17505815.001).
  • [ISSN] 0340-2592
  • [Journal-full-title] Der Urologe. Ausg. A
  • [ISO-abbreviation] Urologe A
  • [Language] ger
  • [Publication-type] Clinical Trial; English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 5J49Q6B70F / Vincristine
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57. Pascual Samaniego M, Calleja Escudero J, Alvarez Gago T, Gonzalo Rodríguez V, Müller Arteaga C, Fernández del Busto E: [Adult Wilms' tumor]. Actas Urol Esp; 2004 Jul-Aug;28(7):544-8
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  • [Title] [Adult Wilms' tumor].
  • [Transliterated title] Tumor de Wilms del adulto.
  • Wilms' tumor is a malignant embryonic renal neoplasm that is exceptional in adults.
  • There are not clinical data or radiographic investigations that can distinguish it from renal cell carcinoma.
  • The prognosis of Wilms' tumor in adults is worse than in children because of the high recurrence, the lower response rate to chemotherapy regimens and the advanced stage at the time of clinical presentation, like an asymptomatic abdominal mass in 75% of the cases.
  • We report a new case of nephroblastoma in a 29 years old woman presenting like a renal colic, with a cystic configuration by abdominal ultrasound initially, that changed into a solid renal mass later.
  • There is not a definitive treatment protocol currently but some authors suggest a combination chemotherapy with carboplatin and etoposide because it is very effective in recurrent or refractory adult Wilms' tumor.
  • [MeSH-major] Kidney / pathology. Kidney Neoplasms / pathology. Wilms Tumor / pathology
  • [MeSH-minor] Adult. Female. Humans. Nephrectomy / methods. Tomography, X-Ray Computed. Treatment Outcome. Urography

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  • (PMID = 15384282.001).
  • [ISSN] 0210-4806
  • [Journal-full-title] Actas urologicas espanolas
  • [ISO-abbreviation] Actas Urol Esp
  • [Language] spa
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Spain
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58. Paulino AC, Wen BC, Brown CK, Tannous R, Mayr NA, Zhen WK, Weidner GJ, Hussey DH: Late effects in children treated with radiation therapy for Wilms' tumor. Int J Radiat Oncol Biol Phys; 2000 Mar 15;46(5):1239-46
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  • [Title] Late effects in children treated with radiation therapy for Wilms' tumor.
  • PURPOSE: To determine the frequency and types of late effects in children receiving radiation therapy (RT) for Wilms' tumor.
  • MATERIALS AND METHODS: From 1968 to 1994, 55 children received megavoltage RT at our institution as part of treatment for Wilms' tumor.
  • There were 12 Stage I, eight Stage II, 15 Stage III, six Stage IV, and one Stage V patient.
  • All patients received chemotherapy; the most common agents were actinomycin-D/vincristine/adriamycin in 13 and actinomycin-D/vincristine in 18.
  • RESULTS: Of 42 patients, 13 (31.0%) did not have late effects of treatment.
  • The number of patients who developed muscular hypoplasia, limb length inequality, kyphosis, and iliac wing hypoplasia were seven (16.7%), five (11.9%), three (7.1%), and three (7.1%), respectively.
  • Median time to development of scoliosis was 102 months, with a range of 16-146 months.
  • Only one of 12 Group A patients developed scoliosis.
  • Of 23 patients, five irradiated within 10 days of surgery and one of 19 irradiated after 10 days developed bowel obstruction (p = 0.09, log rank test).
  • Three patients developed hypertension with normal blood urea nitrogen (BUN) and creatinine levels; another patient had chronic renal insufficiency in a nonirradiated kidney.
  • One patient developed diffuse interstitial pneumonitis.
  • Four patients developed benign neoplasms; three were in the RT field (two osteochondroma, one lipoma) and one outside (cervical intraepithelial neoplasia II).
  • There were three second malignancies (chronic myelogenous leukemia at 9 years, osteosarcoma at 11 years, and breast cancer at 25 years after initial diagnosis of nephroblastoma); both solid malignancies occurred in the RT field.
  • CONCLUSIONS: Late effects of therapy were seen in more than two thirds of children treated for Wilms' tumor.
  • [MeSH-major] Kidney Neoplasms / radiotherapy. Radiation Injuries / complications. Wilms Tumor / radiotherapy
  • [MeSH-minor] Child. Child, Preschool. Dose-Response Relationship, Radiation. Female. Fertility / radiation effects. Follow-Up Studies. Humans. Infant. Intestinal Obstruction / etiology. Intestine, Small / radiation effects. Kidney Diseases / etiology. Kyphosis / etiology. Male. Muscles / radiation effects. Neoplasm Staging. Neoplasms, Second Primary / etiology. Puberty, Delayed / etiology. Scoliosis / etiology. Time Factors

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  • (PMID = 10725637.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] UNITED STATES
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59. Efferth T, Bode ME, Schulten HG, Thelen P, Granzen B, Beniers AJ, Mertens R, Gefeller O, Ringert RH, Jakse G, Fuzesi L: Differential expression of the lung resistance-related protein/major vault protein in the histological compartments of nephroblastomas. Int J Oncol; 2001 Jul;19(1):163-8
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  • Nephroblastomas (Wilms' tumors) are curable with survival rates above 80%.
  • Some tumors, however, fail to respond to therapy and those patients have a poor prognosis.
  • In a search for molecular markers of drug resistance, we investigated the expression of lung resistance protein (LRP) in tissue samples from 32 children with nephroblastoma by means of immunohistochemistry.
  • LRP/MVP expression was found in the blastemal and epithelial compartments but to a significantly lesser extent in the stromal compartment of Wilms' tumors.
  • We found significant relationships between LRP/MVP expression and chemotherapeutic pre-treatment of tumors and tumor stage.
  • The immunohistochemical results were validated with a real-time RT-PCR technique and a significant association between protein and mRNA expression was observed.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Kidney Neoplasms / metabolism. Neoplasm Proteins / metabolism. Vault Ribonucleoprotein Particles / metabolism. Wilms Tumor / metabolism
  • [MeSH-minor] Antibiotics, Antineoplastic / therapeutic use. Antineoplastic Agents, Phytogenic / therapeutic use. Child. Child, Preschool. DNA Primers / chemistry. Dactinomycin / therapeutic use. Drug Resistance, Multiple. Humans. Immunoenzyme Techniques. RNA, Messenger / metabolism. Reverse Transcriptase Polymerase Chain Reaction. Vincristine / therapeutic use. beta 2-Microglobulin / metabolism

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  • (PMID = 11408938.001).
  • [ISSN] 1019-6439
  • [Journal-full-title] International journal of oncology
  • [ISO-abbreviation] Int. J. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Antineoplastic Agents, Phytogenic; 0 / Biomarkers, Tumor; 0 / DNA Primers; 0 / Neoplasm Proteins; 0 / RNA, Messenger; 0 / Vault Ribonucleoprotein Particles; 0 / beta 2-Microglobulin; 0 / major vault protein; 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine
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60. Jayasinghe C, Siegler N, Leuschner I, Fleischhack G, Born M, Müller AM: Renal cell carcinoma with Xp11.2 translocation in a 7-year-old boy. Klin Padiatr; 2010 May;222(3):187-9
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  • [Title] Renal cell carcinoma with Xp11.2 translocation in a 7-year-old boy.
  • BACKGROUND: More than 90% of pediatric renal tumors are nephroblastomas while renal cell carcinomas (RCC) are rare in children (< 5%).
  • PATIENT: According to the clinical diagnoses of a nephroblastoma stage IV a 7-year-old boy with a kidney tumor and peripheral pulmonary lesion was preoperatively treated for 8 weeks with Vincristine, Actinomycin D and Adriamycin.
  • There was no tumor regression and the pulmonary lesion was no longer detectable.
  • Hence chemotherapy was put to a halt.
  • CONCLUSION: Fine needle aspiration biopsy (FNA) would have allowed to adjust the tumor subtype.
  • Prognosis of pediatric RCC with translocation seems more favourable than without translocation though definitive evidence will only be possible by documentation in a clinical diagnose-related register.
  • [MeSH-major] Carcinoma, Renal Cell / genetics. Carcinoma, Renal Cell / secondary. Chromosomes, Human, X / genetics. Kidney Neoplasms / genetics. Lung Neoplasms / genetics. Lung Neoplasms / secondary. Sex Chromosome Aberrations. Translocation, Genetic / genetics
  • [MeSH-minor] Child. Humans. Kidney / pathology. Male. Neoplasm Staging. Nephrectomy. Prognosis. Tomography, X-Ray Computed

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  • (PMID = 20514625.001).
  • [ISSN] 1439-3824
  • [Journal-full-title] Klinische Pädiatrie
  • [ISO-abbreviation] Klin Padiatr
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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61. Inoue M, Uchida K, Kohei O, Nashida Y, Deguchi T, Komada Y, Kusunoki M: Teratoid Wilms' tumor: a case report with literature review. J Pediatr Surg; 2006 Oct;41(10):1759-63
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  • [Title] Teratoid Wilms' tumor: a case report with literature review.
  • Teratoid Wilms' tumor is an unusual histologic variant of nephroblastoma in which the heterologous tissue predominates.
  • The treatment for this tumor has not been established because of its rarity and varying tumor components.
  • The authors report a case of localized teratoid Wilms' tumor in a 4-month-old male infant successfully treated with nephrectomy only.
  • The tumor originated from the right kidney with multiple cysts and calcifications; renal teratoma was suspected preoperatively, and the operation was performed.
  • The tumor was excised completely, and the specimen weighed 340 g.
  • Histopathologic examination showed that various teratoid elements occupied most of the tumor with sparse islands of triphasic nephroblastoma, which was consistent with teratoid Wilms' tumor.
  • Anaplastic elements, tumor capsule invasion, renal sinus soft tissue invasion, or tumors in intrarenal vessels were not observed.
  • The patient was disease free for 3 years after surgery without receiving chemotherapy.
  • We selected this therapeutic approach because of the chemoresistance of this tumor and the excellent prognosis in small (<550 g) stage I/favorable-histology classical Wilms' tumor diagnosed at less than 24 months of age.
  • Therefore, nephrectomy alone may be an acceptable treatment of this stage of tumor in patients with teratoid Wilms' tumor.
  • [MeSH-major] Kidney Neoplasms / pathology. Kidney Neoplasms / surgery. Nephrectomy. Teratoma / pathology. Teratoma / surgery. Wilms Tumor / pathology. Wilms Tumor / surgery
  • [MeSH-minor] Humans. Infant. Male. Radiography, Abdominal. Tomography, X-Ray Computed. Ultrasonography

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  • (PMID = 17011284.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 18
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62. Das DK: Fine-needle aspiration (FNA) cytology diagnosis of small round cell tumors: value and limitations. Indian J Pathol Microbiol; 2004 Jul;47(3):309-18

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Small round cell tumors (SRCTs) are a group of malignancies (non-Hodgkin lymphoma, neuroblastoma, retinoblastoma, hepatoblastoma, nephroblastoma, rhabdomyosarcoma, small cell anaplastic carcinoma, Ewing sarcomal peripheral neuroectodermal tumor, and desmoplastic small round cell tumor), characterized both cytologically and histologically by a predominantly small round to oval, and relatively undifferentiated cells.
  • The patients may present in later (inoperable) stage with huge intrathoracic and intraabdominal mass, when chemotherapy and/or radiation therapy may be the first or only line of treatment.
  • As a less invasive procedure fine needle aspiration (FNA) cytology has definite advantage over surgical excision biopsy to arrive at a tissue diagnosis before initiation of therapy.
  • Important cytomorphological features, which help in the identification of various SRCTs include completely dissociated cell population and lymphoglandular bodies (cytoplasmic fragments) in non-Hodgkin lymphoma (NHL), eosinophilicfibrillar material and Homer-Wright rosettes along with cellular processes in neuroblastoma, acinar formation in hepatoblastoma, blastema cells with tubular differentiation in nephroblastoma, tadpole shaped cells in embryonal rhabdomyosarcoma, extreme nuclear molding and perinuclear blue inclusion in small cell anaplastic carcinoma (SCAC), irregular, punched out and large cytoplasmic vacuolations due to glycogen in Ewing sarcoma, and sheets of undifferentiated small round cells surrounded by collageneous stroma in desmoplastic small round cell tumor (DSRCT).
  • Some of these features such as nuclear molding, rosette, and acinar formation are noticed in more than one type of SRCTs.
  • It is suggested that cytomorphological features along with one or more of the parameters such as special stains (cytochemistry), immunocytochemistry (ICC), electron microscopy (EM), tissue culture, DNA ploidy, karyotype and molecular analysis can increase the diagnostic accuracy of SRCTs.
  • However, these facilities may not be available in all the laboratories, especially in the developing countries, and even if available in a limited form, a tissue diagnosis has to be offered often by FNA cytology based on morphological features, as a life saving measure in seriously ill patients before the results of ancillary studies are finalized.

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  • (PMID = 16295413.001).
  • [ISSN] 0377-4929
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] India
  • [Number-of-references] 67
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63. Jenkner A, Camassei FD, Boldrini R, de Sio L, Ravà L, Bosman C, Boglino C, Donfrancesco A: 111 renal neoplasms of childhood: a clinicopathologic study. J Pediatr Surg; 2001 Oct;36(10):1522-7
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  • [Title] 111 renal neoplasms of childhood: a clinicopathologic study.
  • PURPOSE: The aim of this study was to perform a clinicopathologic evaluation of a single pediatric institution renal tumor series.
  • Most patients were treated within the frame of 3 consecutive SIOP trials, which included preoperative chemotherapy as their main feature.
  • RESULTS: In 98 patients (88%), nephroblastoma was diagnosed, followed by 6 adult-type renal tumors, 3 cystic nephromas, 2 mesoblastic nephromas, and 2 clear cell sarcomas.
  • For nephroblastoma, a statistically significant correlation between grade and both disease-free survival rate and 5-year survival rate, and between stage and overall survival rate was shown.
  • Tumor spillage was very sensitive to chemo or radiotherapy.
  • CONCLUSIONS: Wilms' tumor was the most frequent neoplasm and resulted in a 5-year cure rate of 90%.
  • [MeSH-major] Kidney Neoplasms / pathology. Wilms Tumor / pathology

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  • [Copyright] Copyright 2001 by W.B. Saunders Company.
  • (PMID = 11584401.001).
  • [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
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64. Michiels JJ, Budde U, van der Planken M, van Vliet HH, Schroyens W, Berneman Z: Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. Best Pract Res Clin Haematol; 2001 Jun;14(2):401-36

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  • Acquired von Willebrand syndrome (AVWS) associated with hypothyroidism is of type I, results from a decreased synthesis of factor VIII and von Willebrand factor (VWF), responds to desmopressin with normal half-life times for factor VIII and VWF parameters, and disappears after treatment with I-thyroxine.
  • AVWS type I or III, which occurs in a minority of patients with Wilms' tumour in the complete absence of an inhibitor against VWF and no absorption of factor VIII or VWF onto nephroblastoma cells, responds to chemotherapy and/or tumour resection.
  • Hyaluronic acid produced by nephroblastoma cells may be the causative factor in atypical AVWS in Wilms' tumour.
  • AVWS type II in thrombocythaemia is caused by a platelet-dependent proteolysis of large VWF multimers, given the inverse relationship between platelet count and large VWF multimers in plasma and specific increases in the number of proteolytic VWF fragments in plasma.
  • The laboratory findings of AVWS associated with systemic lupus erythematosus or IgG benign monoclonal gammopathy are characterized by a prolonged bleeding time and activated partial thromboplastin time, decreased or absent ristocetin-induced platelet activity, low to very low levels of factor VIII coagulant activity (mean 15%), VWF: Ag (mean 10.7%) and VWF: RCo (mean 6.2%), and a type II multimeric pattern of VWF.
  • High-dose intravenous immunoglobulin transiently corrects the factor VIII coagulant and VWF levels, lasting for a few weeks in AVWS type II associated with systemic lupus erythematosus or IgG benign monoclonal gammopathy.
  • Prednisolone and chemotherapy will not affect AVWS associated with IgG benign monoclonal gammopathy because the monoclonal IgG protein remains to act as an anti-VWF autoantibody.
  • The clinical picture of AVWS associated with early-stage IgG multiple myeloma, chronic lymphocytic leukaemia or non-Hodgkin's lymphoma without a paraprotein or no detectable underlying disorder is similar to that of AVWS type II in IgG benign monoclonal gammopathy but poorly documented with regard to the underlying immune mechanism of AVWS.
  • Drug-induced AVWS has been described in association with the use of pesticides valproic acid, ciprofloxacin, griseofulvin, tetracycline, thrombolytic agents and hydroxyethyl starch.
  • [MeSH-minor] Hematologic Tests. Humans. Kidney Neoplasms / complications. Lupus Erythematosus, Systemic / complications. Lymphoproliferative Disorders / complications. Paraproteinemias / complications. Syndrome. Wilms Tumor / complications

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  • (PMID = 11686107.001).
  • [ISSN] 1521-6926
  • [Journal-full-title] Best practice & research. Clinical haematology
  • [ISO-abbreviation] Best Pract Res Clin Haematol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 148
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65. Linni K, Urban C, Lackner H, Höllwarth ME: Nephron-sparing procedures in 11 patients with Wilms' tumor. Pediatr Surg Int; 2003 Aug;19(6):457-62
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  • [Title] Nephron-sparing procedures in 11 patients with Wilms' tumor.
  • PURPOSE: In unilateral Wilms' tumor (WT), tumor nephrectomy is the standard surgical approach, whereas partial nephrectomy (PN) is controversially discussed.
  • The aim of our retrospective study was to show that in selected cases of unilateral WT kidney-sparing operations could be a reasonable alternative to nephrectomy and to discuss the results of patients with bilateral WT treated by tumor enucleation.
  • MATERIALS AND METHODS: From 1981 to 1998, seven patients with unilateral nephroblastoma (four stage I, one stage III and two stage IV) had tumor resection by PN (five right side, two left side), which was planned when the tumor volume was reduced after 4 to 6 weeks of chemotherapy by at least 50%, when the tumor occupied one pole or was easily resectable, when 50% or more of the kidney tissue remained and when paraaortic lymph nodes were free by intraoperative histological examination.
  • In four patients with bilateral WT (stage V) bilateral tumor enucleation was carried out-except in one patient in whom the contralateral kidney had to been removed because of extension of the tumor via the inferior vena cava to the right atrium.
  • All patients ( n = 11) received pre- and postoperative chemotherapy followed by radiotherapy in four patients.
  • RESULTS: All patients with unilateral WT ( n = 7) are still alive and disease free (follow-up time: mean 6.6 years, range: 28 months to 11 years) with normal renal function, although two patients with secondary nephrectomy revealed creatinine clearance levels at the lower range.
  • In a stage III tumor patient (intraperitoneal metastasis, free lymph nodes), secondary nephrectomy was necessary due to renal arterial thrombosis 2 days after PN.
  • In one stage IV tumor patient (lung metastasis, free lymph nodes), the primary resection was not far enough away from the tumor margin so that an additional slice of tissue with then tumor-free margins had to be resected.
  • In the group of bilateral WT patients ( n = 4), one child died 2 months after surgery during chemotherapy because of central venous line sepsis.
  • One patient who additionally suffered from inferior vena cava tumor thrombosis extending to the right atrium making nephrectomy of the right kidney necessary developed chronic renal failure 4.7 years postoperatively.
  • The other two stage V tumor patients have creatinine clearance levels within the normal range.
  • CONCLUSIONS: Kidney-sparing procedures remain the operative approach of choice in patients with bilateral WT, but bear the risk of chronic renal failure when one kidney has to be removed.
  • PN in children with unilateral WT, carried out by an experienced surgeon, is a reasonable alternative to nephrectomy if strict guidelines such as excellent tumor response to preoperative chemotherapy and easy resectability far away from the tumor margins through healthy kidney tissue are followed.
  • Paraaortic lymph nodes must be free of tumor invasion in order to avoid local radiotherapy.
  • PN prevents the patient from having to have dialysis in cases of contralateral nephrectomy resulting from metachronous WT or subsequent renal trauma.
  • [MeSH-major] Kidney Neoplasms / surgery. Nephrectomy / methods. Wilms Tumor / surgery
  • [MeSH-minor] Algorithms. Child. Child, Preschool. Female. Humans. Infant. Male. Postoperative Complications. Retrospective Studies. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 12756594.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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66. Reinhard H, Aliani S, Ruebe C, Stöckle M, Leuschner I, Graf N: Wilms' tumor in adults: results of the Society of Pediatric Oncology (SIOP) 93-01/Society for Pediatric Oncology and Hematology (GPOH) Study. J Clin Oncol; 2004 Nov 15;22(22):4500-6
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  • [Title] Wilms' tumor in adults: results of the Society of Pediatric Oncology (SIOP) 93-01/Society for Pediatric Oncology and Hematology (GPOH) Study.
  • PURPOSE: In the Society of Pediatric Oncology (SIOP) 93-01 study, 30 patients older than 16 years were found to have Wilms' tumor.
  • They were treated according to the pediatric protocol and were analyzed for clinical presentation, stage distribution, and prognosis.
  • Tumor stages were defined according to SIOP, and treatment was risk-adapted according to SIOP 93-01/Society for Pediatric Oncology and Hematology (GPOH) protocol.
  • RESULTS: Ten patients (33%) had metastatic disease at the time of diagnosis (liver, four patients; lung, three patients; liver and lung, three patients).
  • The local stage distribution showed a predominance of higher stages (stage I, eight patients; stage IIN-, three patients; stage IIN+, four patients; stage III, 15 patients).
  • Two of 30 patients showed a nephroblastoma and a renal cell carcinoma simultaneously in the same kidney.
  • A complete remission was achieved in 24 patients; four patients relapsed after complete remission; and three of them reached a second remission with further treatment.
  • Event-free survival was 57%, with an overall survival of 83% (median observation time, 4 years).
  • CONCLUSION: Adults can be cured in a high percentage by a multimodal treatment according to pediatric protocols.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Wilms Tumor / drug therapy. Wilms Tumor / pathology
  • [MeSH-minor] Adolescent. Adult. Age Factors. Disease-Free Survival. Female. Humans. Male. Middle Aged. Neoplasm Staging. Prognosis. Retrospective Studies. Treatment Outcome

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  • (PMID = 15542800.001).
  • [ISSN] 0732-183X
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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