• Clinical and Experimental Pediatrics
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• 제53권11호
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• pp.975-978
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• 2010

A 7-year-old boy presented with hematochezia and abdominal pain. A 3.7-cm-sized mass was identified in the ascending colon by abdominal computed tomography and colonoscopy. The patient underwent surgical resection. Pathological examination revealed a low-grade perivascular epithelioid cell tumor (PEComa). PEComa in the colon is very rare. Only a few cases have been reported so far. An effective treatment method for this rare tumor has not been established yet. The patient received adjuvant interferon-${\alpha}$ immunotherapy for 1 year. He has been tumor-free for 26 months since the initial diagnosis. This report is the first documented case of the use of interferon-${\alpha}$ for pediatric PEComa of the colon.

• Journal of Chest Surgery
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• 제56권5호
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• pp.367-370
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• 2023

Perivascular epithelioid cell tumors (PEComas) are very rare mesenchymal neoplasms, composed of histologically and immunohistochemically distinctive cells that form a sheet-like appearance around vessel lumens. Although most are benign, a malignant subset does exist, complicating clinical diagnostic efforts. Proper evaluation and management are thus essential for long-term patient survival. Herein, we present a rare case of a benign pulmonary PEComa that was diagnosed postoperatively in a 45-year-old woman.

• 한국간담췌외과학회지
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• 제27권4호
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• pp.329-341
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• 2023

Vascular tumors of the liver are mesenchymal lesions from endothelial cells. They range from common benign lesions such as haemangioma, intermediate tumors like Kaposi sarcoma, and perivascular epithelioid cell tumor to malignant tumors such as hepatic epithelioid hemangioendothelioma and hepatic angiosarcoma in adults. Pediatric vascular tumors of the liver also include benign, locally aggressive, borderline, and malignant masses with haemangiomas being the most common benign tumors and epithelioid hemangioendothelioma being an uncommon pediatric malignancy. The list of these lesions is completed by nodular regenerative hyperplasia, solitary fibrous tumour, and hepatic small vessel neoplasms (HSVN). Some of these tumors are uncommon and rare. This review article aimed to enumerate hepatic vascular tumors along with their imaging, histopathology, molecular findings for accurate diagnosis that can result in better management.