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Primary primitive neuroectodermal tumour of the urinary bladder: a clinico-pathological study emphasising immunohistochemical, ultrastructural and molecular analyses.
J Clin Pathol. 2006 Jul; 59(7):775-8.JC

Abstract

Primary primitive neuroectodermal tumours (PNETs) of the bladder are extremely rare and aggressive neoplasms, and only six examples have been reported in the literature. The case of a 21-year-old woman, who remains disease free 3 years after tumour resection, is reported here. Morphological features were found to correspond to a small round blue cell tumour without rosette formation and with extensive areas of necrosis. Strong expression of CD99, vimentin and CD117 (c-kit), and focal reactivity to cytokeratin and S-100 protein was observed in tumour cells. Ultrastructurally, sparse neurosecretory granules were observed. Diagnosis of PNET was supported by molecular genetic analysis, showing the EWS-FLI-1 fusion transcript type 2 by RT-PCR and EWS gene rearrangement by fluorescence in situ hybridisation. A normal genetically balanced genotype was shown by comparative genomic hybridisation, which, together with the expression of c-kit, a known therapeutic target for imatinib, may have prognostic and therapeutic implications.

Authors+Show Affiliations

Department of Pathology, Reina Sofia University Hospital, Cordoba, Spain.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

16803953

Citation

Lopez-Beltran, A, et al. "Primary Primitive Neuroectodermal Tumour of the Urinary Bladder: a Clinico-pathological Study Emphasising Immunohistochemical, Ultrastructural and Molecular Analyses." Journal of Clinical Pathology, vol. 59, no. 7, 2006, pp. 775-8.
Lopez-Beltran A, Pérez-Seoane C, Montironi R, et al. Primary primitive neuroectodermal tumour of the urinary bladder: a clinico-pathological study emphasising immunohistochemical, ultrastructural and molecular analyses. J Clin Pathol. 2006;59(7):775-8.
Lopez-Beltran, A., Pérez-Seoane, C., Montironi, R., Hernández-Iglesias, T., Mackintosh, C., & de Alava, E. (2006). Primary primitive neuroectodermal tumour of the urinary bladder: a clinico-pathological study emphasising immunohistochemical, ultrastructural and molecular analyses. Journal of Clinical Pathology, 59(7), 775-8.
Lopez-Beltran A, et al. Primary Primitive Neuroectodermal Tumour of the Urinary Bladder: a Clinico-pathological Study Emphasising Immunohistochemical, Ultrastructural and Molecular Analyses. J Clin Pathol. 2006;59(7):775-8. PubMed PMID: 16803953.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Primary primitive neuroectodermal tumour of the urinary bladder: a clinico-pathological study emphasising immunohistochemical, ultrastructural and molecular analyses. AU - Lopez-Beltran,A, AU - Pérez-Seoane,C, AU - Montironi,R, AU - Hernández-Iglesias,T, AU - Mackintosh,C, AU - de Alava,E, PY - 2006/6/29/pubmed PY - 2006/9/13/medline PY - 2006/6/29/entrez SP - 775 EP - 8 JF - Journal of clinical pathology JO - J Clin Pathol VL - 59 IS - 7 N2 - Primary primitive neuroectodermal tumours (PNETs) of the bladder are extremely rare and aggressive neoplasms, and only six examples have been reported in the literature. The case of a 21-year-old woman, who remains disease free 3 years after tumour resection, is reported here. Morphological features were found to correspond to a small round blue cell tumour without rosette formation and with extensive areas of necrosis. Strong expression of CD99, vimentin and CD117 (c-kit), and focal reactivity to cytokeratin and S-100 protein was observed in tumour cells. Ultrastructurally, sparse neurosecretory granules were observed. Diagnosis of PNET was supported by molecular genetic analysis, showing the EWS-FLI-1 fusion transcript type 2 by RT-PCR and EWS gene rearrangement by fluorescence in situ hybridisation. A normal genetically balanced genotype was shown by comparative genomic hybridisation, which, together with the expression of c-kit, a known therapeutic target for imatinib, may have prognostic and therapeutic implications. SN - 0021-9746 UR - https://www.unboundmedicine.com/medline/citation/16803953/Primary_primitive_neuroectodermal_tumour_of_the_urinary_bladder:_a_clinico_pathological_study_emphasising_immunohistochemical_ultrastructural_and_molecular_analyses_ L2 - https://jcp.bmj.com/lookup/pmidlookup?view=long&pmid=16803953 DB - PRIME DP - Unbound Medicine ER -