鼻腔鼻窦原发上皮样血管内皮瘤1例  

Primary epithelioid hemangioendothelioma of nasal cavity and sinuses:report of one case

作　　者：冯国仿[1] 杨毅[2] 周成勇[1] 严清红[1] 王胜利 

机构地区：[1]解放军总医院第一附属医院耳鼻咽喉头颈外科,北京100048 [2]解放军总医院第一附属医院病理科,北京100048 [3]河南省武陟县人民医院耳鼻喉科,河南焦作454950

出　　处：《解放军医学杂志》2010年第2期219-221,共3页Medical Journal of Chinese People's Liberation Army

摘　　要：目的探讨鼻腔鼻窦原发上皮样血管内皮瘤的临床病理特征、诊断、鉴别诊断及治疗措施。方法患者于2000年7月因右侧鼻阻及反复右侧鼻出血就诊,行鼻窦CT、MRI检查示:右侧鼻腔鼻窦软组织影,周围骨质无破坏。取活检病理报告为上皮样血管内皮瘤。当时建议其手术治疗,患者拒绝,遂带瘤生存。2007年9月患者因全身多处严重骨质疏松再次住院,查体发现右侧鼻腔肿物。再次取病理活检,证实为上皮样血管内皮瘤。结果光镜下肿瘤细胞呈多角形或类圆形上皮样,无明显核分裂象;其组织结构多样,呈单个细胞条索、实性巢状及管腔样结构排列。鼻腔鼻窦原发上皮样血管内皮瘤的特征性病理表现包括:细胞原始管腔结构;玻璃样间质;网状纤维染色显示血管腔隙结构;瘤细胞表达Vim、CD31、FVⅢRAg,少数细胞表达CD34。结论鼻腔鼻窦原发的上皮样血管内皮瘤是极少见的低度恶性血管性肿瘤,诊断主要依据病理,应注意与转移性癌、黑色素瘤、上皮样血管肉瘤、上皮样血管瘤等鉴别,可行鼻侧切开鼻腔鼻窦肿物切除术或鼻内窥镜下肿物切除术治疗。Objective To study the diagnosis,differential diagnosis and treatment of primary epithelioid hemangioendothelioma of nasal cavity and sinuses.MethodsIn July 2000,the nasal cavity and sinuses of a patient with recurrent epistaxis from right side of nose was scanned with CT and MRI.The image showed soft tissue shadow in right lateral nasal cavity and sinuses,but no bony damage was found around the lesion.The tissue of right lateral nasal neoplasm was obtained for biopsy,and pathological examination revealed epithelioid hemangioendothelioma.However,the patient refused the proposed operative treatment and carried the right lateral nasal neoplasm for 7 years.In September 2007,the patient returned to the hospital because of serious osteoporosis.The right lateral nasal neoplasm was found by physical examination,and epithelioid hemangioendothelioma of nasal cavity and sinuses was again identified with biopsy.ResultsThe tumor cells were polygonal or roundish epithelioid in shape,and no obvious mitotic figure was found under light microscope.The organizational structure of the neoplasm was diverse.The pathological features of epithelioid hemangioendothelioma of nasal cavity and sinuses were summarized as follows：the tumor cells were composed of small intracellular miniature lumens and embedded in hyalinized matrix;the vascular channels were highlighted by reticulin staining,and the tumor cells expressed vimentin,CD31 and FⅧRAg,while CD34 was expressed in a minority of tumor cells.ConclusionsPrimary epithelioid hemangioendothelioma of nasal cavity and sinuses is a rare low-grade malignant angioma,diagnosed mainly on the basis of pathological examination.Differential diagnosis is important to distinguish it from other metastatic carcinoma,malignant melanoma,epithelioid angiosarcoma and epithelioid hemangioma.The treatment of primary epithelioid hemangioendothelioma of nasal cavity and sinuses is resection of the tumor through lateral rhinotomy or endoscopic sinus surgery.

关 键 词：鼻腔 鼻窦肿瘤 血管内皮瘤 上皮样 

分 类 号：R739.62[医药卫生—肿瘤]