鼻部横纹肌肉瘤12例临床分析

Clinical analysis on 12 cases of nasal rhabdomyosarcoma

目的探讨鼻腔、鼻窦横纹肌肉瘤的临床特征、诊疗方法及预后。方法回顾性分析12例鼻部横纹肌肉瘤的临床资料,原发于鼻腔2例,上颌窦3例,筛窦1例,以上颌窦、筛窦为中心向周围膨胀侵犯4例,原发部位不明2例;单纯手术切除2例,手术加放疗加化疗8例,化疗加放疗2例;采用鼻内窥镜手术6例,鼻侧切开术4例,同期颈清扫5例。结果随访1年、3年生存率分别为75.0%(9/12)、33.3%(4/12);7例1年内复发,其中5例行挽救性手术,2例放弃再次治疗。结论鼻部横纹肌肉瘤预后差,早期诊断、广泛手术切除、综合治疗能提高生存率;鼻内窥镜手术能减少手术创伤,对早期病例有一定优越性。

Objective To discuss the chnical character, diagnosis, treatment and prognosis of nasal rhabdomyosarcoma（RMS）. Methods Integrated with a literature review, 12 cases of nasal rhabdomyosarcoma were analyzed retrospectively. The RMS originated from the nasal cavity, in 2 cases, maxillary sinus in 3 cases, and ethmoid sinus in 1 case, and 4 infiltrated into the maxillary sinus and ethmoid sinus in 4 cases. The primary site was not clear in 2 cases. 2 cases were treated only by operation, 8 by operation, radiotherapeutics and chemotherapeutics, 2 by radiotherapeutics and chemotherapeutics. 6 cases were treated by a nasal endoscopic surgical procedure, 4 by lateral rhinotomy, and 5 by neck dissection at the same time. Results The survival rates for 1 and 3 years were 75.0% （9/12） and 33.3% （4/12） respectively. Recurrence was found in 7 cases within 1 year. Among them, 5 accepted retrieval operation and 2 gave up treatment. Conclusions The prognosis of RIMS was bad, early diagnoses, wide surgical resection and integrated treatment could improve survival rate. Nasal endoscopic surgery could reduce operation trauma and has advantages especially for patients in the early stage.