Renal Perivascular Epitheliod Cell Tumour (Pecoma) in a Child: Case Report and Review of Literature

Perivascular Epithelioid Cell Tumor (PEComa) is a rare entity with unpredictable clinical outcome. It is a group of mesenchymal tumors with presence of Perivascular Epithelioid Cells (PEC), known to stain positively with melanosome markers HMB-45. They may arise from many unusual sites including the kidneys. Renal PEComa was previously grouped as angiomyolipoma and its existence is rare too. Pediatric PEComas are even rarer with less than 40 cases described worldwide and none involving the kidney before. We report a case of a 7 year old boy who presented with incidental findings of an abdominal mass, which confirmed to be a renal PEComa of the typical epithelioid Angiomyolipoma (AAML) type. The clinical management and review of literature for this interesting entity is discussed.