Renal angiomyolipoma resembling gastrointestinal stromal tumor with skenoid fibers.

We report an unusual case of renal angiomyolipoma occurring in 68-year-old man. The tumor lacked well-developed vascular and adipose components and was composed almost exclusively of smooth muscle cells. Numerous skenoid-like periodic acid-Schiff-positive globules were interspersed between the tumor cells; the lesion therefore closely resembled a low-grade stromal tumor of the gastrointestinal tract. The HMB45-positive/CD34-negative immunophenotype was essential for the diagnosis of angiomyolipoma. Neither gastrointestinal tumor nor any signs of tuberous sclerosis were found. This lesion should be included in the list of morphologic variations of angiomyolipoma, which may cause diagnostic difficulties.