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A proximal type epithelioid sarcoma of the vulva with multiple distant metastases: A case report and review of the literature

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Author(s)
Hyewon ChungTae-Kyu JangSun Young KwonJinkyeong HaSo-Jin Shin
Keimyung Author(s)
Chung, Hye WonJang, Tae KyuKwon, Sun YoungShin, So Jin
Department
Dept. of Obstetrics & Gynecology (산부인과학)
Dept. of Pathology (병리학)
Journal Title
Gynecol Oncol Rep
Issued Date
2021
Volume
37
Keyword
Epithelioid sarcomaProximal typeVulvaMetastasis
Abstract
Epithelioid sarcomas (ESs) of the vulva are extremely rare soft tissue tumors characterized by an aggressive clinical course and a poor prognosis. The proximal type of ES occurs in the trunk and external genital area and has higher recurrence and distant metastasis rates than the distal type, which is found in the upper and lower extremities. We describe a case of a vulvar ES in a 24-year-old patient who was referred from the department of plastic surgery with protruding mushroom-like lesions in multiple areas, including the lower abdomen, whole vulva, anus, and both inguinal lesions. A biopsy of the lesions confirmed a proximal-type ES. Computed tomography and magnetic resonance imaging revealed multiple metastatic lesions in several regions, including the perineum, vagina, and inguinal regions; nodal metastases in the left external iliac and right inguinal region; and distant metastases in the lungs, pleura of the left lung, bones, and soft tissue. The patient underwent active palliative radiotherapy, followed by chemotherapy, and showed a partial response to treatment. Nineteen months after the initial diagnosis, the patient expired due to cancer progression and pneumothorax.
Keimyung Author(s)(Kor)
정혜원
장태규
권선영
하진경
신소진
Publisher
School of Medicine (의과대학)
Citation
Hyewon Chung et al. (2021). A proximal type epithelioid sarcoma of the vulva with multiple distant metastases: A case report and review of the literature. Gynecol Oncol Rep, 37, 100835. doi: 10.1016/j.gore.2021.100835
Type
Article
ISSN
2352-5789
Source
https://www.clinicalkey.com/#!/content/playContent/1-s2.0-S2352578921001399
DOI
10.1016/j.gore.2021.100835
URI
https://kumel.medlib.dsmc.or.kr/handle/2015.oak/43942
Appears in Collections:
1. School of Medicine (의과대학) > Dept. of Obstetrics & Gynecology (산부인과학)
1. School of Medicine (의과대학) > Dept. of Pathology (병리학)
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