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Primary cutaneous Ewing sarcoma/primitive neuroectodermal tumour: a clinicopathological analysis of seven cases highlighting diagnostic pitfalls and the role of FISH testing in diagnosis.
J Clin Pathol. 2009 Oct; 62(10):915-9.JC

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.

Authors+Show Affiliations

Department of Anatomical Pathology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

19783720

Citation

Shingde, M V., et al. "Primary Cutaneous Ewing Sarcoma/primitive Neuroectodermal Tumour: a Clinicopathological Analysis of Seven Cases Highlighting Diagnostic Pitfalls and the Role of FISH Testing in Diagnosis." Journal of Clinical Pathology, vol. 62, no. 10, 2009, pp. 915-9.
Shingde MV, Buckland M, Busam KJ, et al. Primary cutaneous Ewing sarcoma/primitive neuroectodermal tumour: a clinicopathological analysis of seven cases highlighting diagnostic pitfalls and the role of FISH testing in diagnosis. J Clin Pathol. 2009;62(10):915-9.
Shingde, M. V., Buckland, M., Busam, K. J., McCarthy, S. W., Wilmott, J., Thompson, J. F., & Scolyer, R. A. (2009). Primary cutaneous Ewing sarcoma/primitive neuroectodermal tumour: a clinicopathological analysis of seven cases highlighting diagnostic pitfalls and the role of FISH testing in diagnosis. Journal of Clinical Pathology, 62(10), 915-9. https://doi.org/10.1136/jcp.2008.064014
Shingde MV, et al. Primary Cutaneous Ewing Sarcoma/primitive Neuroectodermal Tumour: a Clinicopathological Analysis of Seven Cases Highlighting Diagnostic Pitfalls and the Role of FISH Testing in Diagnosis. J Clin Pathol. 2009;62(10):915-9. PubMed PMID: 19783720.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Primary cutaneous Ewing sarcoma/primitive neuroectodermal tumour: a clinicopathological analysis of seven cases highlighting diagnostic pitfalls and the role of FISH testing in diagnosis. AU - Shingde,M V, AU - Buckland,M, AU - Busam,K J, AU - McCarthy,S W, AU - Wilmott,J, AU - Thompson,J F, AU - Scolyer,R A, PY - 2009/9/29/entrez PY - 2009/9/29/pubmed PY - 2010/2/19/medline SP - 915 EP - 9 JF - Journal of clinical pathology JO - J Clin Pathol VL - 62 IS - 10 N2 - 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. SN - 1472-4146 UR - https://www.unboundmedicine.com/medline/citation/19783720/Primary_cutaneous_Ewing_sarcoma/primitive_neuroectodermal_tumour:_a_clinicopathological_analysis_of_seven_cases_highlighting_diagnostic_pitfalls_and_the_role_of_FISH_testing_in_diagnosis_ L2 - https://jcp.bmj.com/lookup/pmidlookup?view=long&pmid=19783720 DB - PRIME DP - Unbound Medicine ER -