鼻腔鼻窦横纹肌肉瘤的诊断和治疗

Diagnosis and treatment of rhabdomyosarcoma of nasal cavity and paranasal sinus

目的探讨鼻腔鼻窦横纹肌肉瘤的临床病理和综合治疗方法。方法回顾性分析1984～2002年收治的的4例患者的临床资料。结果4例均为女性,年龄19～74岁,病灶位于鼻腔左侧2例,右侧1例,双侧1例。术后确诊3例,术前确诊1例。病理类型:胚胎型2例,葡萄簇型1例,多形性型1例。手术3例,术后接受放化疗2例,单纯放疗1例。随访4个月～6年,死亡1例,其存活期为6年,健在的3例中有2例已存活3年和6年。结论头颈部横纹肌肉瘤好发于鼻窦、鼻腔,其临床表现不具特征性,术前误诊率高,影像学检查和活检是确诊和避免误诊的重要手段;广泛手术切除乃其主要治疗方法,术后应予放化疗,可提高其生存期。

ObjectiveTo discuss the clinicopathologic features and treatment of rhabdomyosarcoma of nasal cavity andparanasal sinus. MethodsThe clinical data of 4 cases with rhabdomyosarcoma of nasal cavity and paranasal sinus from1984 to 2000 in our hospital were studied retrospectively.Results4 patients were female, whose age ranged trom 19 to 74years old. The lesions of 2 cases were in the left, 1case in the right and 1 case in the double sides. 3 cases had been confirmedpostoperatively and 1 case confirmed preoperatively. In the period of following-up for 4 month to 6 years, 1 patient died,whose survival time was 6 years. The rest 3 patients were alive and 2 patients in them had survived for 3, 6 years respectively.ConclusionsThe survival rate can be greatly increased by the combined therapy, which involves operation, radiotherapy andchemotherapy. Early diagnosis, localization, histological subtype, and prompt treatment are decisive for a successful progno-sis.