Korean Journal of Radiology  2013;14(4):692-696

doi:10.3348/kjr.2013.14.4.692

Uncommon of the Uncommon: Malignant Perivascular Epithelioid Cell Tumor of the Lung.

Hyun Ju LIM 1 ; Ho Yun LEE ; Joungho HAN ; Yong Soo CHOI ; Kyung Soo LEE

Affiliations

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Keywords

Perivascular epithelioid cell; Pecoma; Clear cell sugar tumor; Lung tumor

Country

Republic of Korea

Language

English

Abstract

A perivascular epithelioid cell (PEC) tumor is a rare mesenchymal tumor characterized by abundant cytoplasmic Periodic acid-Schiff positive glycogen (also called sugar tumor or clear cell tumor of the lung for this characteristic) and is mostly benign. We report a case of a 63-year-old man who presented with an enlarging mass on chest radiograph. After a thorough workup, diagnosis of malignant pulmonary PEC tumor with lung to lung metastases was established. Herein, the difficulties of diagnosis and management we confronted are described.