title: Hepatic Angiosarcoma with Dyskeratosis Congenita.
abstract: Nail dystrophy, oral leukoplakia and abnormal skin pigmentation are the defining features of dyskeratosis congenita. Dyskeratosis congenita is a disorder of poor telomere maintenance and is known to increase the risk of developing multiple types of malignancy. However, there are few reports of liver tumors arising in dyskeratosis congenita patients. We herein report the second case of hepatic angiosarcoma arising from dyskeratosis congenita: a 23-year-old man was introduced to our hospital due to the detection of multiple tumors in the liver. A histological analysis showed angiosarcoma that stained positive for antibodies to both CD31 and blood coagulation factor VIII.
authors.affiliation: Department of Medicine and Molecular Science, Gunma University Graduate School of Medicine, Japan.
authors.forename: Norio
authors.initials: N
authors.lastname: Horiguchi
authors.forename: Satoru
authors.initials: S
authors.lastname: Kakizaki
authors.forename: Keisuke
authors.initials: K
authors.lastname: Iizuka
authors.forename: Satoshi
authors.initials: S
authors.lastname: Hagiwara
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Ohyama
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Yamazaki
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Shibusawa
authors.forename: Ken
authors.initials: K
authors.lastname: Sato
authors.forename: Motoyasu
authors.initials: M
authors.lastname: Kusano
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Nakazato
authors.forename: Masanobu
authors.initials: M
authors.lastname: Yamada
mesh_terms: D000328:Adult; D019871:Dyskeratosis Congenita / Q000150:complications* / Q000473:pathology; D005169:Factor VIII / Q000378:metabolism; D017809:Fatal Outcome; D006394:Hemangiosarcoma / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007972:Leukoplakia, Oral / Q000209:etiology* / Q000473:pathology; D008113:Liver Neoplasms / Q000209:etiology* / Q000473:pathology; D008297:Male; D019408:Platelet Endothelial Cell Adhesion Molecule-1 / Q000097:blood
chemical_list: D019408:Platelet Endothelial Cell Adhesion Molecule-1; D005169:Factor VIII