15例鼻腔鼻窦横纹肌肉瘤的临床分析

Clinical analysis of 15 cases with rhabdomyosarcoma of nasal cavity and paranasal sinus

目的 探讨鼻腔鼻窦横纹肌肉瘤(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月收治的15例鼻腔鼻窦RMS患者资料。15例RMS患者多为青少年,胚胎型12例,腺泡型3例。其中单纯化疗1例,鼻内镜手术+术后化疗3例,鼻内镜手术+术后放化疗7例,术前诱导化疗+鼻内镜手术+术后放化疗4例。应用SPSS 26.0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、治疗方式等因素与临床预后的关系。结果 随访3~113个月。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、治疗方式及美国RMS研究组(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。

Objective To investigate the clinical features, treatment and prognostic factors of rhabdomyosarcoma(RMS) in nasal cavity and paranasal sinus.Methods Clinical data of 15 patients with RMS of nasal cavity and paranasal sinuses admitted to our department from Jan 2012 to Jan 2022 were retrospectively analyzed. Most of the 15 patients were adolescents. The pathological diagnosis was embryonal RMS in 12 cases, alveolar in 3. As for the treatment strategies, 1 case received chemotherapy alone, 3 received operation and postoperative chemotherapy, 7 underwent surgery and postoperative chemoradiotherapy, and 4 received preoperative chemotherapy and surgery and postoperative chemoradiotherapy. 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 Follow-up of 3 to 113 months after treatment revealed no recurrence in 6 cases, locoregional recurrence in 4 and systemic recurrence in 5. During follow-up period, 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, regional lymph node metastasis, distant metastasis, treatment method, intergroup rhabdomyosarcoma study(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, with characteristics of easily invading the orbit and skull base, high recurrence rate and mortality. Multiple-disciplinary treatment is effective, operation combined with adjuvant chemoradiotherapy can reduce the recurrence rate and improve the prognosis. To improve the clinical and pathological understanding, conduct molecular and genomic studies, and find new therapeutic targets are expected to improve the life quality and survival rate of RMS patients.