Definition: A group of mesenchymal tumors composed of epithelioid and spindle cells displaying perivascular epithelioid (PEC) differentiation. The PEComa family of tumors includes angiomyolipoma, clear-cell “sugar” tumor of lung and gastrointestinal tract and other sites, lymphangioleimyomatosis, clear-cell myomelanocytic tumor of the falciform ligament.
Synonym(s): extrapulmonary sugar tumor, monotypic epithelioid angiomyolipoma, perivascular epithelioid cell tumor
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