颅底鼻腔上皮样血管内皮细胞瘤误诊分析

为了探讨颅底、鼻腔上皮样血管内皮细胞瘤(EH)的临床、影像及病理学特点,结合文献回顾性分析2例颅底鼻腔EH患者的诊断和治疗.该病病因不清,临床症状少且轻微,无特征性.影像学主要表现为不规则占位影,侵袭性生长,血供丰富.病理学上肿瘤呈结节状分布,细胞形态以上皮样或梭形细胞为主,瘤细胞胞质丰富,呈巢团状不规则排列,结节周边细胞较丰富,间质黏液变.瘤细胞一般缺乏多形性,核分裂及坏死少见.免疫组化显示,CD31、CD34阳性,广谱CK偶见灶性阳性,其他抗体阴性.初步研究结果提示,颅底鼻腔EH为一种上皮样血管内皮细胞分化的低度恶性肿瘤,具有独特病理学特征,临床及影像学特征不明显,容易误诊,需要引起重视,组织病理学是确诊关键.

Misdiagnosis analysis of two cases with epithelioid haemangioendothelioma of cranial base and nasal cavity

The objective of this study was to roport two cases of epithelioid haemangioendothelioma of cranial base and nasal cavity and analyze the clinical,radiological and pathological characteristic of this tumor.The clinic pathology feature of two cases of EH of cranial base and nasal cavity was investigated by light microscopic observation and an immunohistochemical study.The etiology of this rare disease remained unknown.Symptoms were scanty and usually mild.The radiograph or computed tomography usually revealed unregulate improprite image,invasive growth and with enrich blood supply.Histologically,crown-like clusters of epithelioid tumor cells or spindle cells were obtained which filled in the alveoli locating at the periphery of the tumor nodules,while the central part of the nodules contained myxoid to hyaline matrix.Tumor cells generally lacked pleomorphism,mitotic activity and necrosis.They were immunohistochemically positive for CD31 and CD34.CK staining was positive in some cases.In conclusion,the epithelioid hemangioendothelioma is designated low grade malignancy and represents a distinct clinical pathological entity.It is rare and often misdiagnosed as other diseases.Histopathological detection is the key for physicians from misdiagnosis.