Primary Vulvar and Vaginal Extraosseous Ewing's Sarcoma/Peripheral Neuroectodermal Tumor: Diagnostic Confirmation with CD99 Immunostaining and Reverse Transcriptase-Polymerase Chain Reaction

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 characteri...

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Veröffentlicht in:International journal of gynecological pathology 2000-04, Vol.19 (2), p.103-109
Hauptverfasser: Vang, Russell, Taubenberger, Jeffery K, Mannion, Ciaran M, Bijwaard, Karen, Malpica, Anais, Ordonez, Nelson G, Tavassoli, Fattaneh A, Silver, Susan A
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container_issue 2
container_start_page 103
container_title International journal of gynecological pathology
container_volume 19
creator Vang, Russell
Taubenberger, Jeffery K
Mannion, Ciaran M
Bijwaard, Karen
Malpica, Anais
Ordonez, Nelson G
Tavassoli, Fattaneh A
Silver, Susan A
description 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.
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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.</abstract><cop>Philadelphia, PA</cop><cop>Hagerstown, MD</cop><pub>Lippincott Williams &amp; Wilkins, Inc</pub><pmid>10782405</pmid><doi>10.1097/00004347-200004000-00002</doi><tpages>7</tpages></addata></record>
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subjects 12E7 Antigen
Adult
Antigens, CD - analysis
Antineoplastic Agents - therapeutic use
Biological and medical sciences
Cell Adhesion Molecules - analysis
Chromosomes, Human, Pair 11
Chromosomes, Human, Pair 22
Female
Genital system. Mammary gland
Humans
Immunohistochemistry
Investigative techniques, diagnostic techniques (general aspects)
Medical sciences
Neuroectodermal Tumors - diagnosis
Neuroectodermal Tumors - genetics
Neuroectodermal Tumors - therapy
Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques
Radiotherapy
Reverse Transcriptase Polymerase Chain Reaction
Sarcoma, Ewing - diagnosis
Sarcoma, Ewing - genetics
Sarcoma, Ewing - therapy
Surgical Procedures, Operative
Translocation, Genetic
Vaginal Neoplasms - diagnosis
Vaginal Neoplasms - genetics
Vaginal Neoplasms - therapy
Vulvar Neoplasms - diagnosis
Vulvar Neoplasms - genetics
Vulvar Neoplasms - therapy
title Primary Vulvar and Vaginal Extraosseous Ewing's Sarcoma/Peripheral Neuroectodermal Tumor: Diagnostic Confirmation with CD99 Immunostaining and Reverse Transcriptase-Polymerase Chain Reaction
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