Wilms' Tumour and Genitourinary Rhabdomyosarcoma

Gough, D. C. S.

doi:10.1111/j.1464-410x.1989.tb05143.x

Cited by 5 publications

(2 citation statements)

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“…The most intriguing finding of this analysis was the detection of WT1 protein in the cytoplasm of rhabdomyosarcoma cells supporting a possible linkage between Wilms' tumors, myogenic differentiation, and rhabdomyosarcomas (42)(43)(44)(45)(46)(47)(48)(49). Interestingly, rhabdomyogenesis is not uncommon in Wilms' tumors and correlates with a younger age and more favorable outcome.…”

Section: Discussionmentioning

confidence: 82%

The Expression of WT1 in the Differentiation of Rhabdomyosarcoma from Other Pediatric Small Round Blue Cell Tumors

et al. 2002

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The WT1 gene encodes a transcription factor implicated in normal and neoplastic development. The purpose of this study was to evaluate the diagnostic utility of a commercial WT1 antibody on a variety of pediatric small round blue cell tumors (SRBCT). A mouse monoclonal antibody (clone: 6F-H2, DAKO) raised against the N-terminal amino acids 1-181 of the human WT1 protein was tested. Microscopic sections from 66 specimens were stained using an antigen retrieval protocol with trypsin. The tumors included peripheral neuroectodermal tumors (PNET/Ewing's), neuroblastomas, desmoplastic small round cell tumors (DSRCT), lymphomas, Wilms' tumors, and rhabdomyosarcomas (RMS). One RMS case was investigated by Western blot analysis and RT-PCR to confirm the antibody specificity. A strong cytoplasmic staining was demonstrated in all RMS (11/11). The Western blot analysis confirmed the WT1 protein in the tissue, and the RT-PCR confirmed the presence of WT1 mRNA in the peripheral blood and tissue of one RMS patient. The Wilms' tumors had a variable nuclear and/or cytoplasmic positivity in most (17/24) cases. All PNET/Ewing's were negative. The nuclei of two lymphoblastic lymphomas stained strongly. A weak nuclear or cytoplasmic staining was reported in a few DSRCT (3/5), lymphomas (2/10), and neuroblastomas (2/8). This is a useful antibody in the differentiation of RMS from other SRBCTs. A strong cytoplasmic staining favors an RMS, and a strong nuclear staining is suggestive of a Wilms' tumor. A role for WT1 in the pathogenesis of rhabdomyosarcomas is raised. The limited sampling precludes any conclusions regarding the value of tissue or peripheral blood analysis for WT1 mRNA in patients with rhabdomyosarcoma. The WT1 gene (1) encodes a protein with four zinc fingers of the Kruppel-type in the C-terminal region that recognizes a guanidine-cytidine (GC)-rich "EGR1" consensus sequence (2) required in tissue differentiation and proliferation (2-4). The N-terminal half contains a large proline-glutaminerich domain important for inhibition of transcriptional activation (5, 6). There are at least eight protein isoforms ranging between 52 and 62 kDa in mammals produced by a combination of alternative splicing and RNA editing (4, 7). The WT1 proteins are normally expressed in the nuclei of glomerular podocytes and mesothelial cells. It has also been demonstrated in stem cells bearing the CD34ϩ phenotype (8). The role of WT1 in normal human development also extends to a diversity of mammalian mesodermal tissues (9), including the body-wall musculature in a 13.5-days postconception (dpc) mouse embryo (43-49 dpc human). Embryologic studies of wt1-null mice reveal a failure to develop kidney and gonads (10). Mutations and splicing disruptions of WT1 have been described in , WAGR (15), and Frasier (13,16) (17-22), mesothelial-derived neoplasms (23-27), breast cancer (28, 29), genitourinary tumors (30, 31), and small round blue cell tumors (SRBCT;24,27,(32)(33)(34). Recent studies have evaluated the possible role of peripheral blood RNA ...

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Section: Discussionmentioning

confidence: 82%

The Expression of WT1 in the Differentiation of Rhabdomyosarcoma from Other Pediatric Small Round Blue Cell Tumors

et al. 2002

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“…Those who make this a routine practice are the only ones who will find tumors there at initial nephrectomy. Since the site of a common abdominal recurrence in stage I and stage I1 disease is the remaining kidney, it is possible that some patients relapsing in this way were initially stage V at presentation but the disease was undetected [12]. In any case, the survival for metachronous bilateral Wilms' tumor is not good [ 13,141.…”

Section: Maria Do Carmo Assunqao MD (Pathologist)mentioning

confidence: 99%