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KMID : 0614620140640050302
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Korean Journal of Gastroenterology
2014 Volume.64 No. 5 p.302 ~ p.306
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A Case of Malignant Perivascular Epithelioid Cell Tumor of the Retroperitoneum with Multiple Metastases
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Oh Hye-Won
Kim Tae-Hyo
Cha Ra-Ri
Kim Na-Young
Kim Hyun-Jin
Jung Woon-Tae
Lee Ok-Jae
Lee Jeong-Hee
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Abstract
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Perivascular epithelioid cell tumors (PEComas) are unusual mesenchymal neoplasms composed of histologically and im-munohistochemically distinct perivascular epithelioid cells (PECs). Although PEComas have the potential to behave in a malignant fashion, malignant PEComas arising from the retroperitoneum are extremely rare. A 68-year-old woman presented with a painful palpable mass in her left upper abdomen. Computed tomography of the abdomen showed a 9 cm sized heterogeneous mass in left para-aortic space and multiple hypervascular nodules in the liver. 18F-fludeoxyglucose-PET/CT showed multifocal hyper-metabolic lesions in retroperitoneum, liver, and skeletal bones. Percutaneous needle biopsies were done on the retroperitoneal and hepatic mass. Both specimens were positive for human melanoma black-45 (HMB-45) on histological and im-munohistochemical staining which was compatible with PEComas. Herein, we report a rare case of retroperitoneal PEComa with multiple metastases involving liver and bone at initial diagnosis that exhibited aggressive behavior and resulted in a devastating prognosis.
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KEYWORD
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Perivascular epithelioid cell tumor, Metastasis, Retroperitoneal, Liver, Bone
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