Abstract:
AIMS:To perform a clinicopathological analysis of a series of primary cutaneous Ewing sarcomas/primitive neuroectodermal tumours (ES/PNET) to highlight the pathological features, discuss the differential diagnosis, emphasise the role of molecular testing (particularly fluorescence in situ hybridisation, FISH) in diagnosis and outline the patients' clinical course. METHODS:Seven cases of primary cutaneous ES/PNET were identified from the authors' consultation files. RESULTS:The patients were aged 16-61 years (median 25). Five were female and two were male. Five cases involved the limbs and two the trunk. Five were initially misdiagnosed (three as carcinoma and two as melanoma). All cases were characterised histologically by sheet-like growth of small round cells with little cytoplasm and showed strong membranous staining for CD99 and positive but variable staining for FLI-1. Six patients showed an EWS rearrangement (five on FISH analysis and one on RT-PCR). All tumours were completely excised. Three patients received adjuvant chemotherapy, one of whom also received radiotherapy. Follow-up was available in all cases (range 11-57 months; median 41). No recurrences or metastases occurred. CONCLUSIONS:Although rare, primary cutaneous ES/PNET should be considered in the differential diagnosis of cutaneous "small blue cell tumours". Immunostaining for FLI-1 and molecular testing for evidence of an EWS rearrangement are useful ancillary investigations to confirm the diagnosis. The prognosis of primary cutaneous ES/PNET appears to be more favourable than extracutaneous ES/PNET.
journal_name
J Clin Patholjournal_title
Journal of clinical pathologyauthors
Shingde MV,Buckland M,Busam KJ,McCarthy SW,Wilmott J,Thompson JF,Scolyer RAdoi
10.1136/jcp.2008.064014subject
Has Abstractpub_date
2009-10-01 00:00:00pages
915-9issue
10eissn
0021-9746issn
1472-4146pii
62/10/915journal_volume
62pub_type
杂志文章abstract:AIMS:To institute recommendations from a laboratory turnround time study; to evaluate audit methods; and to quantify improvements achieved. METHODS:Changes to result report distribution and specimen delivery were affected by posting results directly from the laboratory followed by the introduction of a twice daily cou...
journal_title:Journal of clinical pathology
pub_type: 杂志文章
doi:10.1136/jcp.48.4.372
更新日期:1995-04-01 00:00:00
abstract::A 45-year-old man is described in whom there is currently ERCP and histological evidence of primary sclerosing cholangitis (PSC). A liver biopsy obtained 29 years ago shows similar histological features confirming that he had PSC at that time. This case indicates that PSC may follow a relatively benign course. ...
journal_title:Journal of clinical pathology
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pub_type: 杂志文章,多中心研究
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journal_title:Journal of clinical pathology
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journal_title:Journal of clinical pathology
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更新日期:1997-03-01 00:00:00
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pub_type: 杂志文章
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更新日期:1984-06-01 00:00:00
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更新日期:1983-09-01 00:00:00
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