title: Familial multilocular cystic nephroma: a variant of a unique renal neoplasm.
abstract: Multilocular cystic nephroma (MCN) is a benign renal lesion believed to be unilateral and nonfamilial. We present 2 cases of MCN in 2 brothers. The older brother had a 10-cm MCN and required radical nephrectomy, and the younger brother had bilateral lesions (6 cm and 3 cm) for which nephron-sparing surgery was possible. Both children underwent chest computed tomography to rule out pleuropulmonary blastoma. These cases suggest a genetic link to the pathogenesis of this lesion is possible and that although MCN can involve the collecting system, it can still be treated with partial nephrectomy.
authors.affiliation: Department of Urology, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Richard A
authors.initials: RA
authors.lastname: Ashley
authors.forename: Yuri E
authors.initials: YE
authors.lastname: Reinberg
mesh_terms: D002675:Child, Preschool; D006801:Humans; D007223:Infant; D052177:Kidney Diseases, Cystic / Q000473:pathology; D007680:Kidney Neoplasms / Q000235:genetics* / Q000473:pathology; D008297:Male