Perivascular epithelioid cell tumor of the heart in a child.

We report a case of cardiac perivascular epithelioid cell tumor (PEComa) that occurred in a 10-year-old girl. The patient presented with cardiac murmur and increasing dyspnea. A solid mass was detected in the left atrioventricular groove by sonography and chest computed tomography. Histologic examination revealed a tumor composed of spindle and epithelioid cells, exhibiting either a clear or a slightly eosinophilic cytoplasm and a bland nucleus. Intranuclear pseudoinclusions and calcifications could be found occasionally. Immunohistochemically, tumor cells were positive for HMB45, melan-A, and smooth muscle actin. We regard this case as a PEComa of uncertain malignant potential. The patient remains disease free at her 18-month follow up. To the best of our knowledge, this is the 3rd report of cardiac PEComa and the 1st report of cardiac PEComa affecting a child.