title: Renal epithelioid angiomyolipoma with a negative premelanosome marker immunoprofile: a case report and review of the literature.
abstract: BACKGROUND
  The rare variant of renal epithelioid/pleomorphic angiomyolipoma has been reported in approximately 120 cases. One of the most important characteristics to differentiate these tumors from other renal cell neoplasms is their typical reactivity to premelanosome antigens. If such a tumor does not stain for HMB-45 or Melan-A, a specific diagnosis of epithelioid pleomorphic angiomyolipoma cannot be made with certainty.
  
  
  METHODS
  We present here what is, to the best of our knowledge, the first case of epithelioid/pleomorphic angiomyolipoma of the kidney in a 50-year-old Caucasian man with no history of tuberous sclerosis, and with a tumor marker profile negative for several premelanosome antigens. The tumor was composed of sheets of pleomorphic, round to polygonal epithelioid cells with prominent eosinophilic cytoplasm, large nuclei, many multinucleated, and very prominent nucleoli. There were prominent vessels and rare interspersed smooth muscle fibers, but adipocytes were not identified. A tumor marker profile showed tumor cell reactivity for CD68, calponin and focally for CD10. Intervening smooth muscle was reactive with smooth muscle actin. The tumor lacked reactivity for melanin-associated antigens HMB-45 and Melan-A, and for CD31, pan-cytokeratin (AE1/3) and desmin. Electron microscopic examination of tumor cells confirmed the presence of premelanosome-like granules.
  
  
  CONCLUSIONS
  Based on the characteristic microscopic appearance of this tumor, and its overall tumor marker profile, we concluded this was a renal epithelioid/pleomorphic angiomyolipoma with a negative premelanosome antigen phenotype.
authors.affiliation: Department of Pathology, University of Colorado, Anschutz Medical Campus, 12800 East 19th Avenue Mail Stop 8104, Aurora, CO 80045, USA. Francisco.LaRosa@ucdenver.edu.
authors.forename: Samantha E
authors.initials: SE
authors.lastname: Hohensee
authors.forename: Francisco G
authors.initials: FG
authors.lastname: La Rosa
authors.forename: Petra
authors.initials: P
authors.lastname: Homer
authors.forename: Thomas
authors.initials: T
authors.lastname: Suby-Long
authors.forename: Shandra
authors.initials: S
authors.lastname: Wilson
authors.forename: Scott M
authors.initials: SM
authors.lastname: Lucia
authors.forename: Kenneth A
authors.initials: KA
authors.lastname: Iczkowski