title: Multifocal and microscopic chromophobe renal cell carcinomatous lesions associated with 'capsulomas' without FCLN gene abnormality.
abstract: Chromophobe renal cell carcinoma (RCC) accounts for approximately 5% of renal epithelial neoplasms. Multiple and/or bilateral chromophobe RCCs in an individual are generally rare but frequently occur in patients with Birt-Hogg-Dubé syndrome (BHDS) and in patients with tuberous sclerosis complex (TSC). The responsible genes in both BHDS and TSC act as tumor suppressors. Therefore, it seems that some genetic backgrounds are required for the generation and progression of multiple chromophobe RCCs. Here, we report a case of multiple and bilateral chromophobe RCCs along with several small-sized capsular angiomyolipomas known as 'capsulomas' in a 39-year-old woman who had neither a particular medical history nor specific gene mutation. There has been no report of sporadic multiple chromophobe RCCs and 'capsulomas' developing in a patient without genetic features, having potential for novel genetic variation.
authors.affiliation: Department of Pathology, Sapporo Medical University School of Medicine, Sapporo, Japan.
authors.forename: Kotaro
authors.initials: K
authors.lastname: Sugimoto
authors.forename: Akira
authors.initials: A
authors.lastname: Takasawa
authors.forename: Shingo
authors.initials: S
authors.lastname: Ichimiya
authors.forename: Masaki
authors.initials: M
authors.lastname: Murata
authors.forename: Hiromichi
authors.initials: H
authors.lastname: Kimura
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Aoyama
authors.forename: Johan J P
authors.initials: JJ
authors.lastname: Gille
authors.forename: Naoto
authors.initials: N
authors.lastname: Kuroda
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Shimizu
authors.forename: Tadashi
authors.initials: T
authors.lastname: Hasegawa
authors.forename: Norimasa
authors.initials: N
authors.lastname: Sawada
authors.forename: Mitsuko
authors.initials: M
authors.lastname: Furuya
authors.forename: Yoji
authors.initials: Y
authors.lastname: Nagashima
mesh_terms: D000328:Adult; D018207:Angiomyolipoma / Q000235:genetics / Q000473:pathology*; D002292:Carcinoma, Renal Cell / Q000235:genetics / Q000473:pathology*; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000235:genetics / Q000473:pathology*; D009378:Neoplasms, Multiple Primary / Q000235:genetics / Q000473:pathology*; D011518:Proto-Oncogene Proteins / Q000235:genetics; D025521:Tumor Suppressor Proteins / Q000235:genetics
chemical_list: C476078:FLCN protein, human; D011518:Proto-Oncogene Proteins; D025521:Tumor Suppressor Proteins
keywords: angiomyolipoma; chromophobe renal cell carcinoma; kidney