A 6-year-old girl with vaginal spotting who was diagnosed with perivascular epithelioid cell neoplasm after vaginoscopic resection
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Perivascular epithelioid cell neoplasm (PEComa) is a rare tumor with unknown malignant potential. We report a caseof a 6-year-old child with history of brain tumor (pineoblastoma), who presented with intermittent vaginal spottingfor 6 months. A vaginoscopy revealed a 1.5×1.0-cm mass on the vaginal wall. Pathological examination demonstratedthat the tumor was composed of clear cells with organoid patterns, which were immunohistochemically positive forHMB-45 and TFE3, and negative for CK, HNF1-B, SOX10, Melan A, and S-100 protein. These findings were consistentwith PEComa arising from the vagina. Regular follow-up with magnetic resonance imaging has shown no signs ofrecurrence. This case shows that early detection of PEComa and subsequent regular follow-ups are important becauseof the neoplasm’s unknown malignant potential.
Abstract
Perivascular epithelioid cell neoplasm (PEComa) is a rare tumor with unknown malignant potential. We report a caseof a 6-year-old child with history of brain tumor (pineoblastoma), who presented with intermittent vaginal spottingfor 6 months. A vaginoscopy revealed a 1.5×1.0-cm mass on the vaginal wall. Pathological examination demonstratedthat the tumor was composed of clear cells with organoid patterns, which were immunohistochemically positive forHMB-45 and TFE3, and negative for CK, HNF1-B, SOX10, Melan A, and S-100 protein. These findings were consistentwith PEComa arising from the vagina. Regular follow-up with magnetic resonance imaging has shown no signs ofrecurrence. This case shows that early detection of PEComa and subsequent regular follow-ups are important becauseof the neoplasm’s unknown malignant potential.
참고문헌 (13)
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