복막전이를 동반한 그물막의 악성 혈관주위 상피모양세포 종양 1예
Malignant Perivascular Epithelioid Cell Tumor (PEComa) Arising in the Omentum with Metastatic Peritoneal Seeding: A Case Report
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초록
The World Health Organization (WHO) defines a perivascular epithelioid cell tumor (PEComa) as a mesenchymal neoplasia composed of perivascular epithelioid cells with characteristic morphological and immunohistochemical features. They are rarely malignant. Indeed, only a few cases have been reported in the English literature, and this is the first case of PEComa reported in Korea. A 64-year-old man presented with an abdominal mass and peritoneal seeding. The patient underwent mass excision with descending colon resection and anastomosis. The primary mass and peritoneal seeding was diagnosed as a PEComa histopathologically. Seven months later, he developed liver metastasis and aggravated peritoneal seeding. Here, we report a case of malignant PEComa of the omentum.
Abstract
The World Health Organization (WHO) defines a perivascular epithelioid cell tumor (PEComa) as a mesenchymal neoplasia composed of perivascular epithelioid cells with characteristic morphological and immunohistochemical features. They are rarely malignant. Indeed, only a few cases have been reported in the English literature, and this is the first case of PEComa reported in Korea. A 64-year-old man presented with an abdominal mass and peritoneal seeding. The patient underwent mass excision with descending colon resection and anastomosis. The primary mass and peritoneal seeding was diagnosed as a PEComa histopathologically. Seven months later, he developed liver metastasis and aggravated peritoneal seeding. Here, we report a case of malignant PEComa of the omentum.
참고문헌 (10)
- Bonetti F, "PEC and sugar", Am J Surg Pathol, 16, pp.307~308 (1992)
- Vang R, "Perivascular epithelioid cell tumor ('PEComa') of the uterus: a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors", Am J Surg Pathol, 26, pp.1~13 (2002)
- Bleeker JS, "Malignant" perivascular epithelioid cell neoplasm: risk stratification and treatment strategies", Sarcoma, 2012, pp.541626~ (2012)
- 김국현, "대장에서 발생한 혈관주위 상피모양세포 종양 1예", 대한내과학회지, 72(5), pp.540~545 (2007), 대한내과학회
- 조영환, "대장암으로 오인된 혈관주위상피모양세포종양 1예", 대한소화기학회지, 60(6), pp.377~381 (2012), 대한소화기학회
- Prasad ML, "Pleomorphic angiomyolipoma of digestive tract: a heretofore unrecognized entity", Int J Surg Pathol, 8, pp.67~72 (2000)
- Folpe AL, "Perivascular epithelioid cell neoplasms: pathology and pathogenesis", Hum Pathol, 41, pp.1~15 (2010)
- Scheppach W, "PEComa of the colon resistant to sirolimus but responsive to doxorubicin", ifosfamide, 2013, pp.19, 1657~1660
- Shitara K, "Dramatic tumor response to everolimus for malignant epithelioid angiomyolipoma", Jpn J Clin Oncol, 41, pp.814~816 (2011)
- Dickson MA, "Extrarenal perivascular epithelioid cell tumors (PEComas) respond to mTOR inhibition: clinical and molecular correlates", Int J Cancer, 132, pp.1711~1717 (2013)
