嗅神经母细胞瘤误诊原因分析

目的:探讨嗅神经母细胞瘤的误诊原因。方法:回顾分析1993年1月至2004年12月收治的24例嗅神经母细胞瘤的临床资料,主要包括临床诊断、影像学术前诊断、术前术后病理学诊断。结果:24例中男14例、女10例,4~70岁,平均37.92岁,病程10d~19个月,平均6个月。Kadish分期:A期4例,B期7例,C期13例。临床误诊13例(54.16%),分别为鼻息肉5例(20.83%),鼻窦炎3例(12.50%)、淋巴瘤2例(8.33%)、视神经萎缩1例(4.17%)、血管球瘤1例(4.17%)。术前影像与实际不符16例(66.67%)。病理误诊6例(25.00%),分别为胚胎性横纹肌肉瘤2例(8.33%)、原始神经外胚叶肿瘤2例(8.33%)、浆细胞肉瘤1例(4.17%)。病理诊断不明确为小细胞恶性肿瘤1例(4.17%)。结论:对有鼻塞或鼻出血并伴有鼻腔顶部可疑新生物者要警惕本病;CT及MRI显示中心位于鼻腔顶部和筛窦,密度较均匀的大片状组织肿块,侵犯邻近结构(眼眶、颅内),筛板及眼眶内侧壁被侵蚀性破坏,应首先考虑嗅神经母细胞瘤;对临床可疑而初次病理诊断为非本病的病例,应反复取活检并结合形态学特点及免疫组化结果尽早确诊。

Misdiagnosis of esthesioneuroblastoma

Objective:To explore the reason for misdiagnosis of esthesioneuroblastoma. Methods: The clinical data of esthesioneuroblastoma,including clinical dignosis,CT or MRI,preoperative pathology and postoperative pathology were retrospectively analyzed in a sample of 24 patients(14 males and 10 females) aged 4 to 70 years and treated at our department from January 1993 to December 2004. Results: Patients were classfied into stage A(n=4), B(n=7) and C(n=13)groups by tumor stages using Kadish.The misdiagnosis ratio on clinic,CT/MRI and pathology was 13/24((54.16%),) 16/24(66.67%) and 6/24(25.00%),respectively,and the inexactly pathological diagnosis ratio was 1/24((4.16%).) The disease was clinically misdiagnosed as nasal polyp in 5/24(20.83%),sinusis in 3/24(12.50%), lymphoma in 2/24(8.33%),optic nerve atrophy in 1/24(4.17%) and glomus tumor in 1/24(4.17%),respectively.The disease was pathologically misdiagnosed as embryonal rhabdomyosarcoma in 2/24(8.33%),primitive neuroectodermal tumor in 2/24(8.33%) and plasmacyto sarcoma in 1/24(4.16%),respectively.The disease disagnosed as small cell tumor in 1/24(4.16%). Conclusions: Patients with nasal bleeding,nasal occlusion and superior endonasal mass should be alerted.Esthesioneuroblastoma should be considered with the patient,whose CT or MRI indicates that the epicenter of tumor is in superior nasal cavity and ethmoid sinus,and the orbital and intracraninal are infiltrated;Immunohistochemical analysis is helpful in the diagnosis and the differential diagnosis of esthesioneuroblastoma.