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Vaginal Epithelioid Angiosarcoma: A Potential Pitfall in Gynecologic Pathology

Richer, Maxime MD, PhD1; Barkati, Maroie MD, FRCPC2; Meunier, Caroline MD, FRCPC3; Fortin, Suzanne MD, FRCPC4; Provencher, Diane MD, FRCSC, FACOG5; Rahimi, Kurosh MD, MSc, FRCPC1

Journal of Lower Genital Tract Disease: April 2014 - Volume 18 - Issue 2 - p E38–E42
doi: 10.1097/LGT.0b013e31829863b3
Case Reports

Objective Epithelioid angiosarcoma of the vagina is a rare variant that can easily be misdiagnosed considering the much higher frequency of epithelial neoplasms at that particular site.

Material and Methods We report the case of a 41-year-old gravida 2, para 1, aborta 1, with no prior history of irradiation, who consulted after the discovery of 3 lesions at the lower right portion of the vagina.

Result The lesion consisted of epithelioid cells with high-grade nuclei and prominent nucleoli. These cells expressed CD31, CD34, factor VIII, Fli-1, vimentin, smooth muscle actin, and WT-1. Keratin 8/18 was focally positive. They were immunonegative for keratin AE1/AE3, keratin 34βE12, keratin 7, keratin 20, S100, HMB-45, myogenin, desmin, and human herpesvirus type 8. Polymerase chain reaction–based HPV viral search was also negative.

Conclusions A broad immunohistochemical panel including antibodies against vascular differentiation markers as well as various cytokeratins allows proper diagnosis of this unusual and aggressive entity.

The epithelioid variant of the vaginal angiosarcoma is a very rare entity posing a real diagnostic challenge to general and gynecopathologists.

Departments of 1Pathology and 2Radiation Oncology, Centre hospitalier de l’Université de Montréal, Hôpital Notre-Dame; Departments of 3Pathology and 4Obstetrics and Gynecology, Hôpital Maisonneuve-Rosemont; and 5Division of Gynecologic Oncology, Centre hospitalier de l’Université de Montréal, Hôpital Notre-Dame, Montréal, Québec, Canada

Reprint requests to: Maxime Richer, MD, PhD, Department of Pathology, Hôpital Notre-Dame du Centre hospitalier de l’Université de Montréal, 1560 Sherbrooke St East, Montréal, Québec, Canada H2L 4M1. E-mail:

The authors have declared they have no conflicts of interest.

Copyright © 2014 by the American Society for Colposcopy and Cervical Pathology