@article {Lopez-Beltran775, author = {A Lopez-Beltran and C P{\'e}rez-Seoane and R Montironi and T Hern{\'a}ndez-Iglesias and C Mackintosh and E de Alava}, title = {Primary primitive neuroectodermal tumour of the urinary bladder: a clinico-pathological study emphasising immunohistochemical, ultrastructural and molecular analyses}, volume = {59}, number = {7}, pages = {775--778}, year = {2006}, doi = {10.1136/jcp.2005.029199}, publisher = {BMJ Publishing Group}, 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{\textendash}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.}, issn = {0021-9746}, URL = {https://jcp.bmj.com/content/59/7/775}, eprint = {https://jcp.bmj.com/content/59/7/775.full.pdf}, journal = {Journal of Clinical Pathology} }