The Spectrum of Pulmonary Perivascular Epithelioid Cell Tumors (PEComa)

The perivascular epithelioid cell family of neoplasms in the lung encompass lymphangioleiomyomatosis, angiomyolipoma, angioleiomyoma, and perivascular epithelioid cell tumor (PEComa). They share the unique attribute of immunoreactivity for both melanocytic and muscle markers. Some are primary pulmonary neoplasms, while others, such as lymphangioleiomyomatosis, are now thought to represent metastatic, indolent tumors from uterine sites of origin. The clinical and morphologic appearances vary considerably, but a genetic link with tuberous sclerosis complex is a common feature. Considerable advances in our understanding of tumor biology, management, and treatment have been made over the last 2 decades, particularly for lymphangioleiomyomatosis. This review will highlight those advances and our current understanding of pulmonary PEComa-related lesions.