Korean J Gastroenterol.  2014 Nov;64(5):302-306. 10.4166/kjg.2014.64.5.302.

A Case of Malignant Perivascular Epithelioid Cell Tumor of the Retroperitoneum with Multiple Metastases

• Oh HW
• Kim TH
• Cha RR
• Kim NY
• Kim HJ
• Jung WT
• Lee OJ
• Lee JH

• KMID: 2164435
• DOI: http://doi.org/10.4166/kjg.2014.64.5.302

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.

Keyword

Perivascular epithelioid cell tumor; Metastasis; Retroperitoneal; Liver; Bone

MeSH Terms

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
Fluorodeoxyglucose F18

Figure

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  Fig. 1. Abdominal enhanced computer tomography shows multiple hypervascular nodules in the liver (A) and 9 cm sized heterogenous enhancing mass in left para-aortic space (B).

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  Fig. 2. PET/CT scan shows 18 F-fludeoxyglucose hot uptakes in the liver (A) and left para-aortic space mass (B).

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  Fig. 3. (A) The tumor shows nested epithelioid tumor cells with round nucleus and granular eosinophilic or clear cytoplasm (H&E, ×200). The tumor cells are positive for HMB-45 (B, ×200), but negative for smooth muscle actin (C, ×200) and S-100 (D, ×200).

Reference

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