title: Intrapelvic Bulky Tumor as an Unusual Presentation of Erdheim-Chester Disease.
abstract: Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis, which is known to affect various organs; however, there have been no reports of its intrapelvic involvement. We herein describe the case of 69-year-old man who died of a rapidly-growing intrapelvic tumor, which was finally diagnosed as ECD at autopsy. Immunohistochemically, the tumor cells were positive for CD68 and BRAF V600E, and negative for CD1a. Since BRAF V600E has recently been reported to be specific to ECD, it can be a useful biomarker for diagnosis, especially in atypical cases.
authors.affiliation: Department of Urology, Graduate School of Medicine, The University of Tokyo, Japan.
authors.forename: Satoru
authors.initials: S
authors.lastname: Taguchi
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Kishida
authors.forename: Koichi
authors.initials: K
authors.lastname: Tamura
authors.forename: Yorito
authors.initials: Y
authors.lastname: Nose
authors.forename: Toshikazu
authors.initials: T
authors.lastname: Sato
authors.forename: Akira
authors.initials: A
authors.lastname: Ishikawa
authors.forename: Yukio
authors.initials: Y
authors.lastname: Homma
authors.forename: Mitsunori
authors.initials: M
authors.lastname: Yamakawa
mesh_terms: D000368:Aged; D015703:Antigens, CD / Q000276:immunology; D015214:Antigens, Differentiation, Myelomonocytic / Q000276:immunology; D014408:Biomarkers, Tumor; D031249:Erdheim-Chester Disease / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D006801:Humans; D008297:Male; D010386:Pelvic Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology; D000097382:CD68 Molecule
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D014408:Biomarkers, Tumor; C067980:CD68 antigen, human; D000097382:CD68 Molecule