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A 6-year-old girl with vaginal spotting who was diagnosed with perivascular epithelioid cell neoplasm after vaginoscopic resection

대한산부인과학회지 2014년 57권 5호 p.409 ~ 411
 ( Cho Hyun-Jung ) - University of Ulsan College of Medicine Asan Medical Center Department of Obstetrics and Gynecology

 ( Lee Mi-Kyung ) - University of Ulsan College of Medicine Asan Medical Center Department of Obstetrics and Gynecology
 ( Kang Byung-Moon ) - University of Ulsan College of Medicine Asan Medical Center Department of Obstetrics and Gynecology
 ( Kim Sung-Hoon ) - University of Ulsan College of Medicine Asan Medical Center Department of Obstetrics and Gynecology
 ( Chae Hee-Dong ) - University of Ulsan College of Medicine Asan Medical Center Department of Obstetrics and Gynecology
 ( Kim Chung-Hoon ) - University of Ulsan College of Medicine Asan Medical Center Department of Obstetrics and Gynecology

Abstract


Perivascular epithelioid cell neoplasm (PEComa) is a rare tumor with unknown malignant potential. We report a case of a 6-year-old child with history of brain tumor (pineoblastoma), who presented with intermittent vaginal spotting for 6 months. A vaginoscopy revealed a 1.5×1.0-cm mass on the vaginal wall. Pathological examination demonstrated that the tumor was composed of clear cells with organoid patterns, which were immunohistochemically positive for HMB-45 and TFE3, and negative for CK, HNF1-B, SOX10, Melan A, and S-100 protein. These findings were consistent with PEComa arising from the vagina. Regular follow-up with magnetic resonance imaging has shown no signs of recurrence. This case shows that early detection of PEComa and subsequent regular follow-ups are important because of the neoplasm’s unknown malignant potential.

키워드

Perivascular epithelioid cell neoplasms; Resection; Vagina; Vaginoscopy
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