Renal hypertrophic infundibular stenosis.
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.
Craver
Randall
R
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
Boyd
Rebecca
R
Ward
Kenneth
K
Harmon
Edwin
E
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
D019009:Proto-Oncogene Proteins c-kit