38例头颈部髓外浆细胞瘤临床分析  

Clinical analysis of 38 patients diagnosed with head and neck extramedullary plasmacytoma

作　　者：杨磊[1] 王明婕[2] 曾立洁 陶建华[3] 王承稷 王亮 YANG Lei;WANG Mingjie;ZENG Lijie;TAO Jianhua;WANG Chengji;WANG Liang(Department of Hematology,Beijing Tongren Hospital,Capital Medical University,Beijing,100730,China;Department of Otolaryngology Head and Neck Surgery,Key Laboratory of Otolaryngology Head and Neck Surgery(Capital Medical University),Ministry of Education,Beijing Tongren Hospital,Capital Medical University,Beijing,100730,China;Department of Radiology,Beijing Tongren Hospital,Capital Medical University,Beijing,100730,China)

机构地区：[1]首都医科大学附属北京同仁医院血液科,北京100730 [2]首都医科大学附属北京同仁医院耳鼻喉头颈外科,耳鼻咽喉头颈科学教育部重点实验室(首都医科大学),北京100730 [3]首都医科大学附属北京同仁医院放射科,北京100730

出　　处：《中国耳鼻咽喉头颈外科》2024年第5期278-282,共5页Chinese Archives of Otolaryngology-Head and Neck Surgery

基　　金：国家自然科学基金(82170181、82370188);北京市自然科学基金(7222027);国家重点研发计划(2022YFF1502000)。

摘　　要：目的探讨头颈部髓外浆细胞瘤(head and neck extramedullary plasmacytoma,HNEMP)的临床特征、危险因素、治疗及预后。方法回顾性分析2008年9月~2022年1月间北京同仁医院收治的38例新诊断且未治疗的HNEMP患者的临床资料,分析影响生存的因素及其预后。结果38例患者中,病变呈现鼻颅眶沟通性者5例(13.2%),肿瘤最大直径≥5 cm者8例(21.1%)。仅接受单纯手术切除的患者17例(44.7%),术后给予放疗者13例(34.2%),化疗者8例(21.1%),接受放化疗联合治疗者5例(13.2%)。治疗后评价疗效,完全缓解32例,部分缓解3例,疾病稳定1例,疾病进展2例。中位随访86个月,5年的无进展生存率和总生存率分别92.1%和94.7%。其中,颅鼻眶沟通病变患者中位无进展生存期仅13个月,5年的无进展生存率和总生存率为40%和60%,而其余组均为100%。此外,病变范围≥5 cm和累及鼻颅眶沟通是影响患者无进展生存率和总生存率的不良预后因素。结论HNEMP发病率低,治疗上以手术联合放疗为主,预后相对较好,颅鼻眶沟通病变及高肿瘤负荷为不良预后因素。OBJECTIVE Investigating the clinical characteristics,risk factors,treatment strategies and prognosis of head and neck extramedullary plasmacytoma(HNEMP).METHODS To analyze indicators affecting survival and prognosis,retrospective study was conducted on the clinical data of 38 newly diagnosed and untreated patients with HNEMP who were admitted to Beijing Tongren Hospital from September 2008 to January 2022.RESULTS Among 38 patients,5 cases(13.2%)displayed a manifestation as cranial-nasal-orbital communication,and 8 cases(21.1%)involved a tumor with diameter≥5 cm.There were 17 patients(44.7%)who underwent surgical resection along,13 patients(34.2%)who received postoperative radiotherapy,8 cases(21.1%)who accepted chemotherapy,and 5 cases(13.2%)who experienced combined radiochemotherapy.Following treatment evaluation,32 cases achieved CR,3 cases showed PR,1 case demonstrated SD and 2 cases experienced PD.The median follow-up time was 86 months,with 5-year PFS and OS rates of 92.1%and 94.7%,respectively.Of note,the patients with cranial-nasal-orbital communication exhibited significantly unfavorable 5-year PFS and OS rates of 40%and 60%,respectively,with median PFS of 13 months,whereas the patients in other groups achieved 100%of 5-year PFS and OS rates.Additionally,tumor diameter≥5 cm and the involvement of cranial-nasal-orbital communication were adverse prognostic factors for both PFS and OS.CONCLUSION HNEMP is a rare disease and the primary treatment approach is surgery combined with radiotherapy.The prognosis for HNEMP tends to be relatively favorable,with the involvement of cranial-nasal-orbital communication and high tumor burden as the adverse prognostic indicators.

关 键 词：浆细胞瘤 头颈部肿瘤 危险因素 预后 头颈部髓外浆细胞瘤 

分 类 号：R73[医药卫生—肿瘤]