A Case of Malignant Perivascular Epithelioid Cell Tumor of the Retroperitoneum with Multiple Metastases / 대한소화기학회지
The Korean Journal of Gastroenterology
; : 302-306, 2014.
Article
in Korean
| WPRIM (Western Pacific)
| ID: wpr-190503
Responsible library:
WPRO
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.
Full text:
Available
Database:
WPRIM (Western Pacific)
Main subject:
Bone Neoplasms
/
Tomography, X-Ray Computed
/
Fluorodeoxyglucose F18
/
Positron-Emission Tomography
/
Perivascular Epithelioid Cell Neoplasms
/
Liver Neoplasms
Type of study:
Prognostic study
Limits:
Aged
/
Female
/
Humans
Language:
Korean
Journal:
The Korean Journal of Gastroenterology
Year:
2014
Document type:
Article