Clinical analysis of 35 cases of adult rhabdomyosarcoma of nasal cavity and sinuses
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摘要: 目的 探讨成人鼻腔鼻窦横纹肌肉瘤(RMS)的临床特点及影响预后的因素。方法 成人鼻腔鼻窦RMS患者35例, 其中胚胎型22例, 腺泡型13例。手术+放化疗17例, 手术+放疗6例, 手术+化疗7例(手术及化疗后粒子植入4例); 5例未行手术治疗, 仅用抗肿瘤药物治疗。结果 随访9~62个月, 成人鼻腔鼻窦RMS 5年总生存率为2.9%, 其中IRS分期>Ⅱ期、肿瘤颅底浸润、局部淋巴结转移、肿瘤直径≥5 cm、Ki-67≥50%是预后不良因素。结论 成人鼻腔鼻窦RMS以胚胎型多见, 5年总生存率低, 与成人初诊时原发灶肿瘤体积较大、局部淋巴结转移、颅底浸润、Ki-67占比较高等有关。Abstract: Objective To investigate the clinical characteristics and prognostic factors of adult rhabdomyosarcoma(RMS) of nasal cavity and sinus.Method There were 35 adult patients with RMS, including 22 with embryonal type and 13 with acinar type. Surgery + chemoradiotherapy(17 cases), surgery + radiotherapy(6 cases), surgery + chemotherapy(7 cases)(4 cases of seed implantation after surgery and chemotherapy); Five patients were treated with antitumor drugs instead of surgery.Result The study follow-up 9-62 months, adult nasal sinuses RMS total 5 years survival rate was 2.9%, among them the IRS stage>Ⅱ period, the infiltration of the skull base tumor, local lymph node metastasis, tumor diameter of 5 cm or more, 50% or higher Ki-67 are poor prognosis factor. Conclusion RMS in nasal cavity and sinus are mostly embryonal type in adults, and the 5-year overall survival rate is low, which is related to larger primary tumor volume, local lymph node metastasis, skull base infiltration and higher ki-67 ratio at the first diagnosis in adults.
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Key words:
- rhabdomyosarcoma /
- nasal sinuses /
- adult
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表 1 35例成人鼻腔鼻窦RMS患者的一般资料
项目 例数(%) 项目 例数(%) 性别 首发症状 男 21(60.0) 鼻塞 29(82.9) 女 14(40.0) 鼻出血 15(42.9) 病理组织分型 涕中带血 20(57.1) 胚胎型 22(62.9) 脓涕 8(22.9) 腺泡型 13(37.1) 眼部胀痛 10(28.6) IRS分期 溢泪 6(17.1) Ⅱ期 20(57.1) 视力下降 9(25.7) Ⅲ期 14(40.0) 失明 2(5.7) Ⅳ期 1(2.9) 面部麻木 3(8.6) 初诊病变位置侵及 肿瘤直径/cm 鼻腔及鼻窦 19(54.3) ≥5 20(57.1) 前颅底 16(45.7) < 5 15(42.9) 淋巴结转移 Ki-67/% 有 19(54.3) ≥50 28(80.0) 无 16(45.7) < 50 7(20.0) -
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