Department of Oral and Maxillofacial Surgery (Head: Professor Constantinos Alexandridis), "Evangelismos" General Hospital, University of Athens Dental School, 45-47 Ipsilantou Str, Athens, GR 10676, Greece.
J Craniomaxillofac Surg. 2013 Oct;41(7):e170-4. doi: 10.1016/j.jcms.2012.11.045. Epub 2013 Jan 26.
Werner syndrome, also called progeria of the adult and pangeria is a rare autosomal recessive disorder that affects connective tissue throughout the body. It is associated with premature ageing and an increased risk of cancer and other diseases. The mean survival for patients with Werner syndrome is 47 years. Death usually occurs when patients are aged 30-65 years because of atherosclerosis or malignant tumours. The purpose of this paper is to present a patient with Werner syndrome exhibiting a chondrosarcoma of the left temporomandibular joint and ramus. To the best of our knowledge this is the first case, of a Werner syndrome patient with an associated head and neck chondrosarcoma being reported. The diagnostic procedure followed and management of the patient are outlined in the paper as well.
沃纳综合征,也称为成人早老症和进行性假性关节骨化症,是一种罕见的常染色体隐性疾病,影响全身的结缔组织。它与过早衰老以及癌症和其他疾病的风险增加有关。患有沃纳综合征的患者的平均生存时间为 47 年。由于动脉粥样硬化或恶性肿瘤,患者通常在 30-65 岁时死亡。本文的目的是介绍一例表现为左侧颞下颌关节和升支软骨肉瘤的沃纳综合征患者。据我们所知,这是首例沃纳综合征患者伴发头颈部软骨肉瘤的病例。本文还概述了所遵循的诊断程序和患者的治疗方法。