Cheng Jie, Wang Yanling, Cheng Andrew, Wang Lizhen, Tian Zhen, Yu Hongbo, Wang Xudong, Wu Yunong, Shen Guofang
Department of Oral and Maxillofacial Surgery, Affiliated Stomatological Hospital, Nanjing Medical University, Jiangsu, China.
J Craniofac Surg. 2011 Sep;22(5):1765-71. doi: 10.1097/SCS.0b013e31822e626a.
Liposarcoma as one of the most common soft-tissue sarcomas in adults rarely occurs in oral and maxillofacial region. Its rarity makes a systematic and in-depth understanding of these uncommon entities extremely difficult. Here we aimed to characterize and analyze the epidemiology, clinicopathologic characteristics, treatment and prognosis of primary oral and maxillofacial liposarcomas by presenting our experience from a Chinese tertiary referral hospital and literature review. All relevant information was manually collected from medical records from the authors' department (1993-2009) and literature retrieval (Jan.1940-Sep.2009). A total number of 150 patients were identified and included with mean age 49.1 years old and a slight male preponderance. These lesions mostly occurred in tongue (32.0%) followed by buccal region (28.7%) and were diagnosed as atypical lipmatous tumor/well-differentiated (58.45%) and myxoid liposarcomas (27.5%) by pathologic re-evaluation and re-categorization based on 2002 WHO classification scheme. Complete surgical excision with negative margins was the primary treatment modality, while therapeutic utilities of adjuvant radiotherapy/chemotherapy remained controversial. Our data reinforced histopathologic subtype as one of key prognostic factors irrespective of gender, age and primary sites for this malignancy. More importantly, our analysis further revealed that tumor size (especially when larger than 3.6 cm) served as another important prognostic factor suggesting higher rates of disease-related death. Taken together, these findings might for the first time provide comprehensive information regarding the epidemiology, clinicopathologic features, treatment and prognosis of oral and maxillofacial liposarcoma.
脂肪肉瘤是成人最常见的软组织肉瘤之一,很少发生于口腔颌面部。其罕见性使得对这些不常见实体进行系统深入的了解极为困难。在此,我们旨在通过展示来自中国一家三级转诊医院的经验并进行文献综述,来描述和分析原发性口腔颌面部脂肪肉瘤的流行病学、临床病理特征、治疗及预后。所有相关信息均从作者科室的病历(1993 - 2009年)及文献检索(1940年1月 - 2009年9月)中手动收集。共确定并纳入了150例患者,平均年龄49.1岁,男性略占优势。这些病变大多发生于舌部(32.0%),其次是颊部(28.7%),根据2002年世界卫生组织分类方案进行病理重新评估和重新分类后,诊断为非典型脂肪瘤性肿瘤/高分化型(58.45%)和黏液样脂肪肉瘤(27.5%)。手术切缘阴性的完整手术切除是主要治疗方式,而辅助放疗/化疗的治疗作用仍存在争议。我们的数据强化了组织病理学亚型是该恶性肿瘤关键预后因素之一,与性别、年龄和原发部位无关。更重要的是,我们的分析进一步显示肿瘤大小(尤其是大于3.6 cm时)是另一个重要的预后因素,提示疾病相关死亡发生率更高。综上所述,这些发现可能首次提供了关于口腔颌面部脂肪肉瘤流行病学、临床病理特征、治疗及预后的全面信息。