26例上呼吸道髓外浆细胞瘤临床分析

Clinical analysis on extramedullary plasmacytoma in the upper airway

目的:分析上呼吸道髓外浆细胞瘤(EMP)的临床特点、治疗情况及转归,提高对上呼吸道EMP的诊治水平。方法:回顾性分析26例上呼吸道EMP患者的临床资料,并与同期因相似症状首诊于耳鼻咽喉科的多发性骨髓瘤(MM)患者对比分析。结果:26例EMP患者中,发生于鼻腔9例,鼻窦7例,咽部6例,喉部4例;临床表现主要为局部肿物及其引起的相应症状,患者局部活检标本均经病理学诊断为浆细胞瘤;其临床表现、内镜下所见及病理组织学改变与MM上呼吸道受累患者无区别,但MM患者常伴贫血、骨质破坏等表现,其侵犯颈部淋巴结者较上呼吸道EMP患者多见。EMP患者中单纯手术治疗10例,综合治疗(手术加放疗)16例;局部复发5例,转化为MM 4例。有颈部淋巴结受累的7例患者均接受了综合治疗,其中4例患者在综合治疗的基础上接受了化疗。该4例患者均未发生局部复发,亦未转化为MM;而未接受化疗的3例患者中1例局部复发,1例转化为MM。结论:上呼吸道EMP的诊断有赖于病理学检查,病理医生对EMP认识的提高及先进分子生物学技术手段的应用,是提高诊断水平的关键。在EMP的诊治过程中必须行相关检查排除MM。EMP的治疗以手术治疗及局部放疗为主,预后较好,但应长期随访。对于淋巴结受累者应加用化疗,以提高疗效,减少局部复发及转化为MM的风险。

Objective： To investigate the clinical manifestation, treatment and prognosis of extramedullary plasmacytoma（EMP） in the upper airway, and to improve the diagnosis and outcome of EMP treatment. Method： Clinical data of 26 EMP cases were reviewed retrospectively, and then compared with multiple myeloma（MM） pa- tients presenting with lesions in upper airway. Result：Of 26 cases, 9 cases with the tumors occurred in nasal cavi- ties, 7 in nasal sinuses, 6 in pharyngeal, 4 in throat, mainly manifesting with local masses ancl relevant symp- toms. The manifestations of clinical, endoscopy findings and pathologic results in EMP patients were not distin- guishable from the lesions of MM patients, while MM patients often accompanied by other findings, such as ane- mia and bone damage. Involvement of neck lymph nodes was more common in MM patients than in EMP patients. Ten patients were treated with surgery, and 16 patients with surgery and radiotherapy. Of the seven EMP patients with involvement of neck lymph nodes, four patients received additional chemotherapy besides surgery and radio- therapy, and no local relapse and MM happened in them, while of the three patients only received surgery and ra- diotherapy, one local relapse were found and one progressed to MM. Conclusion： The diagnosis of EMPs mainly de- pends on pathological results. The judgment of pathologists and application of molecular biology technology are vi- tal for the diagnosis of EMP in upper airway, and MM must be excluded very carefully in the diagnosis of EMP. Surgery combined with radiotherapy is the main treatment for EMP in the upper airway, and the prognosis is good but the follow-up should be taken. Besides surgery and radiotherapy, chemotherapy is beneficial for the EMP pa- tients accompanied with lesions in neck lymph nodes.