15例鼻腔鼻窦横纹肌肉瘤的临床分析
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山西医科大学第二医院

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国家自然科学基金(82171119,82171120),山西省自然科学基金(201901D111386)


Clinical analysis of 15 cases of rhabdomyosarcoma of nasal cavity and paranasal sinus
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The Second Hospital of Shanxi Medical University

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    摘要:

    目的:探讨鼻腔鼻窦横纹肌肉瘤(rhabdomyosarcoma, RMS)的临床特征、治疗方式及预后的影响因素。方法:回顾性分析2012年1月~2022年1月我院收治的15例鼻腔鼻窦横纹肌肉瘤患者资料。应用SPSS 26.0统计软件进行描述性统计分析,用Kaplan-Meier法评估患者的临床特征、病理类型、肿瘤分期、治疗方式等单因素与临床预后的关系。结果:15例RMS患者多为青少年,胚胎型12例,腺泡型3例。1例行单纯化疗,3例行手术+术后化疗,7例行手术+术后放化疗,4例行术前诱导化疗+手术+术后放化疗。随访3月至113月不等。治疗后6例无复发,4例局部复发,5例全身转移。8例生存,7例死亡。1年生存率约为93.3%,5年生存率约为43.6%。病理类型、淋巴结转移、远处转移、治疗方式及IRS分期是影响RMS预后的危险因素(P<0.05)。结论:鼻腔鼻窦RMS临床罕见,易侵及眼眶、颅底,具有高复发率及死亡率。多学科联合诊疗是目前有效的治疗方式,手术联合辅助放化疗可降低复发率、改善预后。提高对该病的临床及病理认识,进行分子学及基因组学研究,寻找新的治疗靶点,有望提高患者的生活质量及生存率。

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    Objective:To investigate the clinical features, treatments and prognostic factors of rhabdomyosarcoma in nasal cavity and paranasal sinus. Methods:Clinical data of 15 patients with rhabdomyosarcoma of the nasal cavity and paranasal sinuses admitted to the Second Hospital of Shanxi Medical University from January 2012 to January 2022 were retrospectively analyzed. SPSS 26.0 statistical software was used for descriptive statistical analysis. Kaplan-Meier method was used to evaluate the relationship between clinical data, pathological features, tumor stage and prognosis. Results:Most of the 15 patients with RMS were adolescents. 12 cases were ERMS, 3 cases were ARMS. 1 case received chemotherapy alone. 3 patients received operation and postoperative chemotherapy. 7 patients received surgery and postoperative CRT. 4 patients received preoperative chemotherapy and surgery and postoperative CRT. Follow-up lasted from 3 months to 113 months. After treatments, 6 cases had no recurrence, 4 cases had locoregional recurrence and 5 cases systemic recurrence. 8 patients survived and 7 died. Survival analysis showed that the overall survival rates for 1 year and 5 years were calculated as 93.3% and 43.6%. Pathological type, presence or absence of regional lymph node metastasis, presence or absence of distant metastasis, treatment method, IRS stage were correlated with the prognosis of nasal and paranasal sinus RMS(P<0.05). Conclusions:RMS in the nasal cavity and paranasal sinus is rare in clinic, easily invading the orbit and skull base, with high recurrence rate and mortality. It is easy to invade the orbit and skull base, with high recurrence rate and mortality. Multiple Disciplinary Team is an effective treatment approach. Operation combined with adjuvant chemoradiotherapy can reduce the recurrence rate and improve the prognosis. To improve the clinical and pathological understanding of nasal and paranasal sarcoma, to conduct molecular and genomic studies, and to find new therapeutic targets, it is expected to improve the quality of life and survival rate of RMS patients.

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  • 收稿日期:2022-03-29
  • 最后修改日期:2022-05-05
  • 录用日期:2022-05-06
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