鼻—鼻窦横纹肌肉瘤临床资料分析

发布时间：2018-10-31 19:10

【摘要】：目的分析鼻-鼻窦横纹肌肉瘤(Rhabdomyosarcoma, RMS)的症状与体征特点,明确影像学检查对鼻-鼻窦RMS临床分期和术前评估的价值,探讨免疫组化染色和分子生物学技术在鼻-鼻窦RMS诊断、分型及预后评估中的意义。 材料与方法本文研究对象为2000年12月至2010年12月我科收治的8例鼻-鼻窦RMS病人。从一般临床资料、症状与体征、辅助检查、临床分期和治疗及结果5个方面对研究对象的病例资料进行分析。其中一般临床资料包括性别、年龄及病程,症状及体征包括鼻部症状与体征及邻近组织器官的异常表现,辅助检查包括电子鼻咽镜检查、鼻窦CT和MRI检查及病理学检查(包括免疫组化染色)。结合文献资料,讨论分子生物学技术在鼻-鼻窦RMS分型及预后评估中的价值。 结果8例鼻-鼻窦RMS病人中男、女病人各4例,1例为未成年人,其余均为成人,年龄范围为8岁-58岁。病程最短20天,最长8年。8例鼻-鼻窦RMS病人中,2例单纯以鼻部症状就诊,5例伴有眼部症状,1例伴有面颊部疼痛及牙齿脱落,1例以颈部肿痛为首发症状。电子鼻咽镜见RMS呈紫红色肿物或灰白色息肉样物。鼻-鼻窦RMS在CT上表现为软组织密度影,可清晰显示骨质破坏及肿瘤内的钙化灶。MRI上鼻-鼻窦RMS表现为长或略长T1、长或略长T2信号影,信号不均匀,增强扫描不均匀轻中度强化,可清楚显示周围组织受侵情况。部分鼻-鼻窦RMS根据组织形态学诊断及分型,多数病例依据免疫组化染色确诊。8例鼻-鼻窦RMS病人中,有2例为临床Ⅳ期,6例为临床Ⅲ期。8例病人均行手术治疗,2例失访。1例术后意外跌倒死亡。1例术后行化疗,术后2个月发现颈椎转移灶,于术后5个月死亡。1例术后行足量放疗,放疗结束时发现腹腔大量转移灶,于术后3个月死亡。1例术后未放疗或化疗,术后3个月发现颈部及腋窝淋巴结转移。1例因病变广泛,术后放弃进一步治疗,于术后3个月死亡。1例术后化疗1疗程,后发现颈部及咽部大量转移灶,于术后4个月死亡。 结论1)鼻-鼻窦RMS早期诊断困难,多数病人就诊时已处于临床Ⅲ期或Ⅳ期。2)鼻窦CT联合鼻窦MRI检查可为鼻-鼻窦RMS的临床分期及术前评估提供可靠依据。3)免疫组化染色及分子生物学技术对鼻-鼻窦RMS的诊断、分型及预后评估具有重要意义。
[Abstract]:Objective to analyze the characteristics of symptoms and signs of naso-paranasal rhabdomyosarcoma (Rhabdomyosarcoma, RMS), and to determine the value of imaging examination in the clinical staging and preoperative evaluation of naso-paranasal rhabdomyosarcoma. To explore the significance of immunohistochemical staining and molecular biology in the diagnosis, typing and prognosis evaluation of naso-paranasal sinus RMS. Materials and methods from December 2000 to December 2010, 8 patients with naso-paranasal sinus RMS were treated in our department. The data of the patients were analyzed from five aspects: general clinical data, symptoms and signs, auxiliary examination, clinical staging, treatment and results. The general clinical data include sex, age and course of disease, symptoms and signs including nasal symptoms and signs and abnormal manifestations of adjacent tissues and organs, and auxiliary examinations including electronic nasopharyngoscope. Paranasal sinus CT and MRI and pathological examination (including immunohistochemical staining). The value of molecular biological technique in RMS typing and prognosis evaluation of nasal and paranasal sinus was discussed. Results among the 8 patients with naso-paranasal sinus RMS, there were 4 male and 4 female, 1 minor, and the rest were adults. The age ranged from 8 to 58 years old. The course of the disease was shortest 20 days and the longest was 8 years. Of the 8 patients with naso-paranasal sinus RMS, 2 were only diagnosed with nasal symptoms, 5 with ocular symptoms, 1 with buccal pain and tooth loss, and 1 with neck swelling and pain. RMS was seen as a purplish red mass or grayish-white polyp-like substance under electronic nasopharyngoscope. RMS showed soft tissue density on CT, which could clearly show bone destruction and calcification in tumor. RMS on MRI showed long or slightly longer T 1, long or slightly long T 2 signal intensity and uneven signal intensity. The enhancement scan is uneven, mild and moderate enhancement, can clearly show the surrounding tissue invasion. Partial naso-paranasal sinus RMS was diagnosed and classified according to histomorphology. Most of the patients were diagnosed by immunohistochemical staining. Of the 8 patients with naso-paranasal RMS, 2 were in stage 鈪，

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