A Case of Malignant Perivascular Epithelioid Cell Tumor of the Retroperitoneum with Multiple Metastases.
10.4166/kjg.2014.64.5.302
- Author:
Hye Won OH
1
;
Tae Hyo KIM
;
Ra Ri CHA
;
Na Young KIM
;
Hyun Jin KIM
;
Woon Tae JUNG
;
Ok Jae LEE
;
Jeong Hee LEE
Author Information
1. Department of Internal Medicine, Gyeongsang National University School of Medicine, Jinju, Korea. kimthy@medimail.co.kr
- Publication Type:Case Reports ; English Abstract
- Keywords:
Perivascular epithelioid cell tumor;
Metastasis;
Retroperitoneal;
Liver;
Bone
- MeSH:
Aged;
Bone Neoplasms/pathology/radiography/secondary;
Female;
Fluorodeoxyglucose F18;
Humans;
Liver Neoplasms/pathology/radiography/secondary;
Perivascular Epithelioid Cell Neoplasms/*diagnosis/pathology;
Positron-Emission Tomography;
Tomography, X-Ray Computed
- From:The Korean Journal of Gastroenterology
2014;64(5):302-306
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Perivascular epithelioid cell tumors (PEComas) are unusual mesenchymal neoplasms composed of histologically and immunohistochemically 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 hypermetabolic 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 immunohistochemical 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.
