摘要
髓外浆细胞瘤(EMP)指原发于骨髓和骨骼以外的浆细胞肿瘤,由浆细胞单克隆异常增生形成的恶性肿瘤,又称原发性软组织浆细胞瘤,多以局限性肿块为主要临床特征,多发生于头颈部,也可发生在全身各部位,最常见的部位包括鼻腔、鼻窦、鼻咽部等,男性发病率高于女性。由于该疾病无特异性临床表现,其影像学表现缺乏特征性,也因发病部位不同而表现出多样性,目前对其生物学行为及诊疗的认识还不够深入,常常导致临床误诊、漏诊而影响患者的预后。该疾病在临床上十分罕见,对其合理的治疗可延长生存期,本文根据此罕见病例的临床资料结合文献对该病的临床特点、影像学表现、诊断与鉴别和治疗方法进行分析总结,旨在提高耳鼻喉科医师对该疾病的认识,以减少临床误诊漏诊。
Extramedullary plasmacytoma(EMP),also known as primary soft tissue plasma cell tumor,refers to a malignant tumor formed by monoclonal abnormal proliferation of plasma cells,which originates from plasmacytoma outside the bone marrow and skeleton,and is characterized by limited masses,mostly in the head and neck,but also in various parts of the body.The most common sites of this disease include nasal cavity,sinuses,nasopharynx,etc.,and its incidence is higher in males than females.Since the disease has no specific clinical manifestations,lacks characteristic imaging manifestations and shows diversity depending on the site of onset,the current understanding of its biological behavior and diagnosis is not deep enough,which often affects the prognosis of patients by resulting in clinical misdiagnosis and missed diagnosis.The disease is clinically rare,and reasonable treatment can prolong the survival of patients.This paper analyzes and summarizes the clinical features,imaging manifestations,diagnosis and differentiation,and treatment methods of this disease based on the clinical data of this rare case and the literature,aiming to improve the understanding of otorhinolaryngologists about this disease to reduce clinical mi sdiagnosis and missed diagnosis.
作者
王桂芳
马青
宋洋
陈露
WANG Guifang;MA Qing;SONG Yang;CHEN Lu(Department of Otorhinolaryngology,926th Hospital of the Joint Logistics Support Force of the People’s Liberation Army,Yunnan,Kaiyuan 661600,China)
出处
《中国医药科学》
2022年第10期189-192,共4页
China Medicine And Pharmacy
关键词
髓外浆细胞瘤
鼻肿瘤
组织病理学
基因重排
Extramedullary plasmacytoma
Nasal tumor
Histopathology
Gene rearrangement