PEComa of the lung

Perivascular epithelioid cell tumor (PEComa), also called clear cell ′sugar′ tumor of the lung, is a rare benign tumor arising from perivascular epithelioid cells (PECs). We report a case of a 15-year-old boy who presented with right lower lobe lesion which turned out to be a clear cell tumor of the lung. An [18F]-fluoro-2-deoxy-D-glucose (FDG) - positron emission tomography (PET) scan revealed mild FDG uptake in the lung lesion (SUV< 1) with no active uptake elsewhere in the body. We discuss the clinical, radiologic and immunohistochemical features of clear cell ′sugar′ tumor of lung and compare them with published literature.