title: Renal hypertrophic infundibular stenosis.
abstract: Bilateral hydrocalycosis secondary to radiographically demonstrated bilateral infundibular stenosis required a left nephrectomy in an 18-year-old young man. The infundibular narrowing was due to marked hyperplasia of the submucosal smooth muscle. Scattered within the hypertrophic smooth muscle were rare spindled CD117-positive interstitial cells, similar to the cells responsible for peristaltic pacemaker activity within the gastrointestinal tract. The admixture of CD117 cells suggests this is a true muscular hyperplasia rather than a leiomyomatosis. The stimulus to the hyperplasia is unknown.
authors.affiliation: Department of Pathology, Louisiana State University Health Sciences Center, Children's Hospital of New Orleans, 200 Henry Clay Ave., New Orleans, LA 70118, USA. rcrave@lsuhsc.edu
authors.forename: Randall
authors.initials: R
authors.lastname: Craver
authors.forename: Rebecca
authors.initials: R
authors.lastname: Boyd
authors.forename: Kenneth
authors.initials: K
authors.lastname: Ward
authors.forename: Edwin
authors.initials: E
authors.lastname: Harmon
mesh_terms: D000293:Adolescent; D002648:Child; D003251:Constriction, Pathologic / Q000473:pathology*; D006801:Humans; D006965:Hyperplasia / Q000378:metabolism / Q000473:pathology*; D007150:Immunohistochemistry; D007674:Kidney Diseases / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D008297:Male; D009130:Muscle, Smooth / Q000378:metabolism / Q000473:pathology*; D009392:Nephrectomy; D019009:Proto-Oncogene Proteins c-kit / Q000378:metabolism
chemical_list: D019009:Proto-Oncogene Proteins c-kit