Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
6
pubmed:dateCreated
2009-10-12
pubmed:abstractText
Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) family of tumor is a very aggressive malignant round cell tumor characterized by translocations involving EWS-FLI1 genes. They are increasingly recognized in extraosseous sites as a result of improvements in diagnostic tools. In this paper, we report 2 additional cases arising in vulva of young adults who have been treated aggressively and have survived fore more than 7 and 4 years successively. Histologic examination showed small round (blue) cell morphology in both cases. The tumor cells contained glycogen and were positive for CD99 and vimentin and negative for keratins, lymphoid markers, S-100, synaptophysin, chromogranin, and desmin. Reverse transcriptase polymerase chain reaction analysis from paraffin-embedded tissue revealed EWS-FLI1 fusion product in 1 case. Collectively, 13 cases of vulvar ES/PNET have been reported in the literature. Only 8 cases have detailed follow-up information with an average follow-up data of 28 months. Ewing sarcoma/PNET should be considered in the differential diagnosis of any undifferentiated tumors involving the lower gynecologic tract and all axillary tests including molecular tests should be performed for correct diagnosis because prolonged survival is possible for this dreadful disease after complete surgical resection, followed by adjuvant therapy.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Aug
pubmed:issn
1525-1438
pubmed:author
pubmed:issnType
Electronic
pubmed:volume
19
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1131-6
pubmed:dateRevised
2011-11-17
pubmed:meshHeading
pubmed:year
2009
pubmed:articleTitle
Primary vulvar Ewing sarcoma/primitive neuroectodermal tumor: a report of 2 cases and review of the literature.
pubmed:affiliation
Department of Pathology, Lombardi Comprehensive Cancer Center, Georgetown University Hospital, 3900 Reservoir Road NW, Washington, DC 20007, USA.
pubmed:publicationType
Journal Article, Review, Case Reports