Experience with six children with fetal rhabdomyomatous nephroblastoma: review of the clinical, biologic, and pathologic features

Saba, L.; Camargo, Beatriz de; Gabriel-Arana, Marcela

doi:10.1002/(sici)1096-911x(199803)30:3<152::aid-mpo4>3.0.co;2-g

Cited by 21 publications

(10 citation statements)

References 11 publications

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“…Stroma predominance on cytology correlates well with histology and may be an early indicator of chemoresistance. [11,12] Rhabdomyoblastic differentiation is easy to identify and differentiate from the exceptionally rare pediatric renal rhabdomyosarcoma. [13] Anaplastic (unfavorable) cytological features can be identified on aspirates using criteria similar to histology, i.e., variation in nuclear size of three times or more, marked hyperchromasia with bizarre nuclei, and atypical mitotic figures in a biphasic or triphasic aspirate.…”

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confidence: 99%

Role of fine needle aspiration cytology in the management of pediatric renal tumors

Iyer

2007

J Indian Assoc Pediatr Surg

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Fine needle aspiration cytology (FNAC) is a useful pre-operative procedure in pediatric renal tumors. It is a safe technique that does not upstage the tumor and permits positive diagnosis of Wilm's tumor in almost all aspirates with use of immunocytochemistry for cytokeratin and WT-1. Advanced information on diagnosis of Wilm's tumor, especially, the presence of stroma predominance or anaplastic change is a guide to selection and monitoring of chemotherapy. Early diagnosis of clear cell sarcoma of the kidney (CCSK) by FNAC helps in the evaluation of bone metastasis and selection of Doxorubicin-based chemotherapy protocols to which the tumor is responsive. Thus, the routine use of FNAC is helpful for further investigation and management of pediatric renal masses.

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confidence: 99%

Role of fine needle aspiration cytology in the management of pediatric renal tumors

Iyer

2007

J Indian Assoc Pediatr Surg

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“…Tumours with extensive rhabdomyogenesis have been termed “foetal rhabdomyomatous type”, occur in younger children and are frequently bilateral10. Rhabdomyoblastic differentiation correlates with poor response to chemotherapy10.…”

Section: Discussionmentioning

confidence: 99%

Diagnostic utility of Wilms′ tumour-1 protein (WT-1) immunostaining in paediatric renal tumours

Goyal¹,

Mishra²,

Sarkar³

et al. 2016

Indian J Med Res

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Background & objectives:Renal tumours constitute about 7 per cent of all neoplasms in children. It is important to differentiate Wilms’ tumour (commonest tumour) from non-Wilms’ tumours. The aim of this study was to evaluate the immunoexpression and diagnostic role of Wilms’ tumour-1 protein (WT1) in paediatric renal tumours.Methods:A total of 53 cases of renal tumours in children (below 18 yr) who underwent total nephrectomy were included in this retrospective study. WT1 immunostaining was done using mouse monoclonal WT1 antibody (clone: 6F-H2).Results:Of the 53 cases, 38 (72%) were of Wilms’ tumour. Non-Wilms’ group (15) included six cases of mesoblastic nephroma (MN), two each of clear cell sarcoma (CCSK), renal cell carcinoma (RCC) and peripheral neuroectodermal tumour (PNET) and one each of angiomyolipoma (AML), rhabdomyosarcoma (RMS) and malignant rhabdoid tumour (MRT). Proportion of WT1 positivity in Wilms’ tumour was 100 per cent in contrast to 26.7 per cent in non-Wilms’ tumours (P<0.001). Epithelial and blastemal components of Wilms’ tumour showed moderate (2+) nuclear and cytoplasmic staining in 80 (24/30) and 75 per cent (24/32) cases, respectively. MN, PNET, CCSK and AML were negative for WT1. RMS, RCC and MRT showed cytoplasmic staining, strongest in RMS. No significant association was seen between WT1 expression and NWTSG (National Wilms’ Tumor Study Group) stage.Interpretation & conclusions:WT1 helps to differentiate Wilms’ tumour from other paediatric renal tumours. It may help in differentiating the two subgroups of Wilms’ tumour which have distinct molecular pathogenesis and biological behaviour, however, further prospective studies are required for validation of this hypothesis.

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“…The tumour chiefly consists of fetal striated muscle with particularly distinct striations and central nuclei, and isolated regions of typical trimorphic nephroblastoma. It is a chemoresistant tumor, characterized by invading the collecting system, and having greater rates of bilateral involvement [1][2][3][4][5][6][7][8][9]. The following article brings the principal clinic, therapeutic and evolutive characteristics in a new case of this rare pathology and a review of the literature.…”

Section: Introductionmentioning

confidence: 99%

“…After cytoreduction and adjuvant therapy, only 6 patients were free of disease, and 8 had died. The same findings were reported by Saba [7], and Maes et al [8], whom noted poor survival rates in patients with FRN taken into incomplete resection of the tumor. Anderson proposed the hypothesis that a poor response to chemotherapy in patients with bilateral WT was associated with post chemotherapy presence of rhabdomyomatous histology [9].…”

Section: Introductionmentioning

confidence: 99%

Fetal rhabdomyomatous nephroblastoma -a variant of Wilms tumor: A case report and review of the literature

Bermeo¹,

Marcela²,

Zableh³

et al. 2017

PUCR

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A B S T R A C TFetal rhabdomyomatous nephroblastoma is a weird variant of Wilms tumor. It's a chemo-resistant tumor, characterized for invading the collecting system, and having greater rates of bilateral involvement. The tumor chiefly consists of fetal striated muscle with particularly distinct striations and central nuclei, and isolated regions of typical trimorphic nephroblastoma. The following article brings in a new case of this rare pathology, associated with a review of the literature

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Experience with six children with fetal rhabdomyomatous nephroblastoma: review of the clinical, biologic, and pathologic features

Cited by 21 publications

References 11 publications

Role of fine needle aspiration cytology in the management of pediatric renal tumors

Role of fine needle aspiration cytology in the management of pediatric renal tumors

Diagnostic utility of Wilms′ tumour-1 protein (WT-1) immunostaining in paediatric renal tumours

Fetal rhabdomyomatous nephroblastoma -a variant of Wilms tumor: A case report and review of the literature

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