鼻腔鼻窦嗅神经母细胞瘤的手术径路探讨

Management of esthesioneuroblastoma of the Fulda surgical concept

目的 总结鼻腔鼻窦嗅神经母细胞瘤 (esthesioneuroblastoma,ENB)的临床经验。方法 对Fulda医院耳鼻咽喉 头颈及面部整形外科 1 988年 7月～ 2 0 0 1年 1 2月经治的 1 7例ENB的临床资料、手术治疗的径路、方法及预后进行回顾性分析。结果 据Morita等的临床分期标准 ,1 7例中A期1例 ,B期 6例 ,C期 9例 ,D期 1例。术后 8～ 1 63个月 (平均 44个月 )的随访观察结果表明 :2年既无复发、亦无转移的总生存率 90 90 % (1 0 / 1 1 ) ,其中A期为 1 / 1、B期为 6/ 6、C期为 3/ 3、D期为 0 / 1。所有经鼻内径路手术的 6例患者至今均存活 ,临床和影像学检查都无复发和转移迹象 ,存活最长者已达1 30个月 ;2例经额下径路手术的C期患者在随访的 8、2 7个月内均未发现复发和转移 ;经面正中掀翻径路手术的B、C期患者各 1例已分别存活 1 63个月和 82个月 ,均无复发和转移。 6例在此观察期间死于ENB :其中C期 5例、D期 1例 ;Ⅱ级 1例、Ⅲ级 1例、Ⅳ级 3例 (另 1例未行分级诊断 )。 1例 (C期/Ⅱ级 )于ENB术后 7年死于星形细胞瘤 ,此间一直无ENB复发。结论 联合应用手术和放射治疗ENB的效果最佳。前颅底恶性肿瘤的手术治疗径路选择应按照以下原则 :若病变组织没有浸润至颅内或眶内 ,采用鼻内径路 ;

Objective To summarize the treatment strategy of esthesioneuroblastoma (ENB) Methods Between 1988 and 2001, 17 patients with ENB were treated at the Department of Otorhinolaryngology of the Klinikum Fulda All patients were monitored on an outpatient basis after completed therapy with a median follow up of 44 months In a retrospective review ,the patients′charts, the computed tomography, and magnetic resonance imaging scans, the operation reports, and the follow up data were analyzed, particularly with respect to the surgical approaches Results All tumors were staged according to Morita One patient was classified as stage A, six stage B, nine stage C, and one stage D All patients received surgical resection Ten patients were disease free for at least 2 years, whereas 6 patients died because of ENB and one due to other disease Of 10 patients who were free of disease, the tumors were removed via a transnasal approach in 6 patients using the microscope in combination with the endoscope These tumors resected endonasally were staged as A (1 case) and B (5 cases) In ENB of stage C a craniofacial resection was performed using a subfrontal approach or the midfacial degloving The lateral rhinotomy was applied only in cases in which an exenteration orbitae had to be carried out Conclusion ENB is best managed by complete surgical resection followed by adjuvant stereotactic radiation therapy The Fulda surgical concept in management of anterior skull base tumors is also forwarded to resection of ENB It classifies the following indications: 1) Endonasal approach in cases without tumor infiltration of the orbit and/or the brain; 2) Subfrontal approach in cases with extended tumor infiltration of the intradural space or the brain; 3) Midfacial degloving in cases with far lateral tumor spread, particularly fossa pterygoidea or pterygopalatina; 4) Lateral rhinotomy in all cases where an exenteratio orbitae is needed