Angiomyolipoma and PEComa are rare tumors arising from perivascular epithelial cells (PECs), with distinctive morphological features.

The kidney is the most frequent site of origin.

Angiomyolipomas are mesenchymal tumors made up of abnormal blood vessels, spindle cells, and mature adipocytes, believed to be derived from perivascular epithelioid cells (PEC). PEComas are a family of mesenchymal tumors arising from PECs, including angiomyolipomas, as well as pulmonary lymphangioleiomyomatosis.