Brady Jacob S, Chung Sei Y, Marchiano Emily, Eloy Jean Anderson, Baredes Soly, Park Richard Chan Woo
Department of Otolaryngology - Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, NJ, USA.
Department of Otolaryngology - Head and Neck Surgery, University of Michigan Medical School, Ann Arbor, MI, USA.
Int J Pediatr Otorhinolaryngol. 2017 Sep;100:71-76. doi: 10.1016/j.ijporl.2017.06.003. Epub 2017 Jun 15.
To analyze the demographics, survival, and treatment efficacy of pediatric sarcomas of the facial skeleton and skull.
Retrospective study of cases from the US National Cancer Institute's Surveillance, Epidemiology, and End Results database. Pediatric patients between the ages of 0 and 18 diagnosed with a malignant sarcoma of either the mandible or the bones of skull, face, and associated joints from 1973 to 2013 were studied.
In total, 204 patients were included in the analysis. The average age at diagnosis was 11.39 (±5.15) years with a male-to-female ratio of 1.4:1. Whites were the most commonly affected race (76.0%). Malignant mandible sarcomas accounted for 29.9% of the cohort (n = 61). The most common pathology was osteosarcoma, which accounted for 43.6% of the cohort (n = 89). Among patients with known histologic grade (n = 95), 26.0% were AJCC stage III or IV. Overall, 5-year disease-specific survival (DSS) was 80.6%. When stratified by treatment modality, 5-year DSS was 86.0% for surgery alone, 67.9% for radiation alone, and 75.3% for surgery with adjuvant radiotherapy (p = 0.041).
Osteosarcoma, Ewing's sarcoma, and chondrosarcoma are the most common subtypes of pediatric head and neck bone sarcoma. Such sarcomas more commonly affect whites and males during pubertal ages. Disease-specific survival is not affected by primary site. Surgery alone is the mainstay of treatment, and demonstrates higher 5-year disease-specific survival compared to radiotherapy alone. Adjuvant radiotherapy does not seem to increase survival, but further investigation is warranted.
分析面部骨骼和颅骨小儿肉瘤的人口统计学特征、生存率及治疗效果。
对美国国立癌症研究所监测、流行病学和最终结果数据库中的病例进行回顾性研究。研究对象为1973年至2013年间诊断为下颌骨或颅骨、面部及相关关节恶性肉瘤的0至18岁小儿患者。
共有204例患者纳入分析。诊断时的平均年龄为11.39（±5.15）岁，男女比例为1.4:1。白人是最常受影响的种族（76.0%）。恶性下颌骨肉瘤占队列的29.9%（n = 61）。最常见的病理类型是骨肉瘤，占队列的43.6%（n = 89）。在已知组织学分级的患者中（n = 95），26.0%为美国癌症联合委员会（AJCC）III期或IV期。总体而言，5年疾病特异性生存率（DSS）为80.6%。按治疗方式分层时，单纯手术的5年DSS为86.0%，单纯放疗为67.9%，手术联合辅助放疗为75.3%（p = 0.041）。
骨肉瘤、尤因肉瘤和软骨肉瘤是小儿头颈部骨肉瘤最常见的亚型。此类肉瘤在青春期更常影响白人和男性。疾病特异性生存不受原发部位影响。单纯手术是主要治疗方式，与单纯放疗相比，其5年疾病特异性生存率更高。辅助放疗似乎并未提高生存率，但有必要进一步研究。
