TY - JOUR
T1 - Primary vulvar and vaginal extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor
T2 - Diagnostic confirmation with CD99 immunostaining and reverse transcriptase-polymerase chain reaction
AU - Vang, Russell
AU - Taubenberger, Jeffery K.
AU - Mannion, Ciaran M.
AU - Bijwaard, Karen
AU - Malpica, Anais
AU - Ordonez, Nelson G.
AU - Tavassoli, Fattaneh A.
AU - Silver, Susan A.
PY - 2000/4
Y1 - 2000/4
N2 - Two cases of extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor arising in unusual, superficial sites are reported. One tumor involved the vaginal wall of a 35-year-old woman, and the other neoplasm arose in the dermis of the vulva in a 28-year-old woman. The tumors showed Characteristic microscopic features of Ewing's sarcoma/peripheral neuroectodermal tumor with nodular monotonous proliferations of undifferentiated, small, round, hyperchromatic cells with a low mitotic index. Rare rosette-like formations were apparent only in the vulvar neoplasm. The tumors displayed intense immunoreactivity in a membranous pattern for CD99, the cell surface glycoprotein encoded by the MIC2 gene. Genetically, the tumors expressed the EWS/FLI-1 chimeric transcript, derived from the t(11;22)(q24;q12) chromosomal translocation. Both patients had localized disease treated with wide local excision; one received postoperative chemotherapy, and the other received chemotherapy and radiotherapy. To date, 18 and 19 months after diagnosis, neither patient has had clinical evidence of local recurrence or metastasis. To our knowledge, these are the first reported cases of vaginal and vulvar Ewing's sarcoma/peripheral neuroectodermal tumor, confirmed with molecular genetic analysis, in the English literature.
AB - Two cases of extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor arising in unusual, superficial sites are reported. One tumor involved the vaginal wall of a 35-year-old woman, and the other neoplasm arose in the dermis of the vulva in a 28-year-old woman. The tumors showed Characteristic microscopic features of Ewing's sarcoma/peripheral neuroectodermal tumor with nodular monotonous proliferations of undifferentiated, small, round, hyperchromatic cells with a low mitotic index. Rare rosette-like formations were apparent only in the vulvar neoplasm. The tumors displayed intense immunoreactivity in a membranous pattern for CD99, the cell surface glycoprotein encoded by the MIC2 gene. Genetically, the tumors expressed the EWS/FLI-1 chimeric transcript, derived from the t(11;22)(q24;q12) chromosomal translocation. Both patients had localized disease treated with wide local excision; one received postoperative chemotherapy, and the other received chemotherapy and radiotherapy. To date, 18 and 19 months after diagnosis, neither patient has had clinical evidence of local recurrence or metastasis. To our knowledge, these are the first reported cases of vaginal and vulvar Ewing's sarcoma/peripheral neuroectodermal tumor, confirmed with molecular genetic analysis, in the English literature.
KW - Chromosomal translocation
KW - Ewing's sarcoma
KW - Peripheral neuroectodermal tumor
KW - Reverse transcriptase polymerase chain reaction
KW - Vagina
KW - Vulva
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U2 - 10.1097/00004347-200004000-00002
DO - 10.1097/00004347-200004000-00002
M3 - Article
C2 - 10782405
AN - SCOPUS:0033998249
SN - 0277-1691
VL - 19
SP - 103
EP - 109
JO - International Journal of Gynecological Pathology
JF - International Journal of Gynecological Pathology
IS - 2
ER -