title: Familial gigantiform cementoma: distinctive clinical features of a large Chinese pedigree.
abstract: Familial gigantiform cementoma is a rare benign fibrocemento-osseous lesion of the jaws that can cause severe facial deformity. It has an autosomal dominant mode of inheritance, but varies in its phenotype. It is more common in white, African, and East-Asian patients. Here we report what is to our knowledge the first distinctive Chinese family with familial gigantiform cementoma involving 4 generations and 13 patients, and which suggests that the tumour presents with 3 distinctive growth phrases.
authors.affiliation: Department of Oral and Maxillofacial-Head and Neck Oncology, Ninth People's Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine, No. 639, Zhizaoju Road, Shanghai, China. Electronic address: xnngo-go@163.com.
authors.forename: H W
authors.initials: HW
authors.lastname: Wang
authors.affiliation: Department of Oral and Maxillofacial-Head and Neck Oncology, Ninth People's Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine, No. 639, Zhizaoju Road, Shanghai, China. Electronic address: feifei09033@126.com.
authors.forename: M
authors.initials: M
authors.lastname: Yu
authors.affiliation: Department of Oral and Maxillofacial-Head and Neck Oncology, Ninth People's Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine, No. 639, Zhizaoju Road, Shanghai, China. Electronic address: qinxj1989@sina.cn.
authors.forename: X J
authors.initials: XJ
authors.lastname: Qin
authors.affiliation: Department of Oral and Maxillofacial-Head and Neck Oncology, Ninth People's Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine, No. 639, Zhizaoju Road, Shanghai, China. Electronic address: chenping_zhang2009@163.com.
authors.forename: C P
authors.initials: CP
authors.lastname: Zhang
mesh_terms: D000293:Adolescent; D015519:Bone Density / Q000502:physiology; D002485:Cementoma / Q000235:genetics*; D005264:Femoral Fractures / Q000235:genetics; D005799:Genes, Dominant / Q000235:genetics; D006801:Humans; D008297:Male; D008339:Mandibular Neoplasms / Q000235:genetics*; D010375:Pedigree
keywords: Chinese pedigree; Facial deformity; Familial gigantiform cementoma (FGC); Long bone fracture