鼻眶脑毛霉菌病16例临床病理分析

目的探讨鼻眶脑毛霉菌病(ROCM)的临床与病理特征,提高对该病的认识和病理诊断水平。方法回顾分析首都医科大学附属北京同仁医院1998～2008年16例ROCM患者的病历资料。用HE、PAS和GMS染色显示组织病变特点及真菌的形态特征,对1例石蜡组织行透射电镜观察。结果 14例(87.5%)有基础疾病,2例(12.5%)无特殊病史。死亡5例(31.3%),3例死于ROCM,2例死于原发病。CT示鼻腔鼻窦软组织密度影16例,MRI示眶内(15例)和颅内(5例)异常信号影。真菌培养7例阳性(43.8%)。组织病理:16例均见组织凝固性坏死、真菌性血管炎及肉芽肿,骨质破坏9例,外周神经纤维坏死7例。特殊染色菌丝形态均符合毛霉目真菌;透射电镜见菌丝形态不规则,菌壁电子密度高。结论 ROCM主要见于糖尿病和血液系统恶性肿瘤者,亦见于无基础疾病者。常见致病菌为根霉和毛霉。在病变组织中找到符合毛霉目真菌形态特点的菌丝可确诊。对临床症状、影像学、真菌培养及病理学表现等多方面进行综合分析可明确诊断。

Clinicopathologic study of 16 cases of rhino-orbitocerebral mucormycosis

Objective To characterize clinicopathologic features of rhino-orbitocerebral mucormycosis （ROCM）.Methods Sixteen cases of ROCM diagnosed in Beijing Tongren Hospital from 1998 to 2008 were studied retrospectively.HE,PAS and GMS stains were performed on all the paraffin-embedded tissues,one of which was also observed by transmission electron microscope.Results Fourteen patients （87.5%） had underlying conditions.The mortality was 31.3% （5 cases）,including 3 patients died of ROCM and 2 died of primary diseases.Orbital （n=15） and cerebral （n=5） involvements were observed by CT and MRI.Fungal culture showed positive results in 7 cases.Pathological examination showed coagulation necrosis,fungal vasculitis and formations of granuloma in all cases,bone destructions in 9 cases and peripheral nerve fiber necrosis in 7 cases.Hyphae with high electron density and irregular shape distributed in the necrotic tissue were found under electron microscopy.Conclusions ROCM mainly occurs in immunocompromised patients,such as DM or hematological malignancies.Rhizopus and Mucor are the major pathogens.Diagnosis depends on the characteristic hyphae of Mucorales found in the lesions,combined with clinical manifestation,radiologly,laboratory and histopathologly.