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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.

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ncbi.nlm.nih.gov
jcp.bmj.com
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Authors+Show Affiliations

Lopez-Beltran A
Department of Pathology, Reina Sofia University Hospital, Cordoba, Spain.
Pérez-Seoane C
No affiliation info available
Montironi R
No affiliation info available
Hernández-Iglesias T
No affiliation info available
Mackintosh C
No affiliation info available
de Alava E
No affiliation info available

MeSH

AdultFemaleHumansNeoplasm ProteinsNeuroectodermal Tumors, PrimitiveOncogene Proteins, FusionProto-Oncogene Protein c-fli-1RNA-Binding Protein EWSUrinary Bladder Neoplasms

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 -