Primary vulvar Ewing sarcoma/primitive neuroectodermal tumor: a report of 2 cases and review of the literature.
Int J Gynecol Cancer
; 19(6): 1131-6, 2009 Aug.
Article
en En
| MEDLINE
| ID: mdl-19820381
ABSTRACT
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.
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Sarcoma de Ewing
/
Neoplasias de la Vulva
/
Tumores Neuroectodérmicos Periféricos Primitivos
Límite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
Int J Gynecol Cancer
Asunto de la revista:
GINECOLOGIA
/
NEOPLASIAS
Año:
2009
Tipo del documento:
Article
País de afiliación:
Estados Unidos