發(fā)布時(shí)間：2018-10-31 19:10

【摘要】：目的分析鼻-鼻竇橫紋肌肉瘤(Rhabdomyosarcoma, RMS)的癥狀與體征特點(diǎn),明確影像學(xué)檢查對(duì)鼻-鼻竇RMS臨床分期和術(shù)前評(píng)估的價(jià)值,探討免疫組化染色和分子生物學(xué)技術(shù)在鼻-鼻竇RMS診斷、分型及預(yù)后評(píng)估中的意義。 材料與方法本文研究對(duì)象為2000年12月至2010年12月我科收治的8例鼻-鼻竇RMS病人。從一般臨床資料、癥狀與體征、輔助檢查、臨床分期和治療及結(jié)果5個(gè)方面對(duì)研究對(duì)象的病例資料進(jìn)行分析。其中一般臨床資料包括性別、年齡及病程,癥狀及體征包括鼻部癥狀與體征及鄰近組織器官的異常表現(xiàn),輔助檢查包括電子鼻咽鏡檢查、鼻竇CT和MRI檢查及病理學(xué)檢查(包括免疫組化染色)。結(jié)合文獻(xiàn)資料,討論分子生物學(xué)技術(shù)在鼻-鼻竇RMS分型及預(yù)后評(píng)估中的價(jià)值。 結(jié)果8例鼻-鼻竇RMS病人中男、女病人各4例,1例為未成年人,其余均為成人,年齡范圍為8歲-58歲。病程最短20天,最長(zhǎng)8年。8例鼻-鼻竇RMS病人中,2例單純以鼻部癥狀就診,5例伴有眼部癥狀,1例伴有面頰部疼痛及牙齒脫落,1例以頸部腫痛為首發(fā)癥狀。電子鼻咽鏡見(jiàn)RMS呈紫紅色腫物或灰白色息肉樣物。鼻-鼻竇RMS在CT上表現(xiàn)為軟組織密度影,可清晰顯示骨質(zhì)破壞及腫瘤內(nèi)的鈣化灶。MRI上鼻-鼻竇RMS表現(xiàn)為長(zhǎng)或略長(zhǎng)T1、長(zhǎng)或略長(zhǎng)T2信號(hào)影,信號(hào)不均勻,增強(qiáng)掃描不均勻輕中度強(qiáng)化,可清楚顯示周圍組織受侵情況。部分鼻-鼻竇RMS根據(jù)組織形態(tài)學(xué)診斷及分型,多數(shù)病例依據(jù)免疫組化染色確診。8例鼻-鼻竇RMS病人中,有2例為臨床Ⅳ期,6例為臨床Ⅲ期。8例病人均行手術(shù)治療,2例失訪。1例術(shù)后意外跌倒死亡。1例術(shù)后行化療,術(shù)后2個(gè)月發(fā)現(xiàn)頸椎轉(zhuǎn)移灶,于術(shù)后5個(gè)月死亡。1例術(shù)后行足量放療,放療結(jié)束時(shí)發(fā)現(xiàn)腹腔大量轉(zhuǎn)移灶,于術(shù)后3個(gè)月死亡。1例術(shù)后未放療或化療,術(shù)后3個(gè)月發(fā)現(xiàn)頸部及腋窩淋巴結(jié)轉(zhuǎn)移。1例因病變廣泛,術(shù)后放棄進(jìn)一步治療,于術(shù)后3個(gè)月死亡。1例術(shù)后化療1療程,后發(fā)現(xiàn)頸部及咽部大量轉(zhuǎn)移灶,于術(shù)后4個(gè)月死亡。 結(jié)論1)鼻-鼻竇RMS早期診斷困難,多數(shù)病人就診時(shí)已處于臨床Ⅲ期或Ⅳ期。2)鼻竇CT聯(lián)合鼻竇MRI檢查可為鼻-鼻竇RMS的臨床分期及術(shù)前評(píng)估提供可靠依據(jù)。3)免疫組化染色及分子生物學(xué)技術(shù)對(duì)鼻-鼻竇RMS的診斷、分型及預(yù)后評(píng)估具有重要意義。
[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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