小儿睾旁横纹肌肉瘤的诊治

目的 提高小儿睾旁横纹肌肉瘤(PARA RMS)的诊治水平.方法 回顾性分析我院1977至2008年6月收治的14例睾旁横纹肌肉瘤病例.年龄1～15岁,其中≤5岁6例,平均年龄6岁.左侧9例,右侧5例.全组病例均因单侧阴囊肿大就诊,经影像检查都属Ⅰ期病变.14例中有4例曾在外院手术,经患侧阴囊切口;1例做睾丸切除,3例保留睾丸、做肿物切除.这4例都再次经腹股沟切口,高位精索离断,3例切除曾被保留的睾丸;并切除原阴囊皮肤切口及其周闱组织;余10例也是经腹股沟切口,根治性睾丸切除.病理检查除1例是腺泡型外,其余均为胚胎型中的梭形细胞型.术后化疗主要用VAC(长春新碱+放线菌素D+环磷铣胺).结果 14例中13例获得随访,随访时间1～24年,平均6年.随访≥2年者11例,10例(91%)无瘤存活,1例于手术后近2年时复诊,腹膜后转移瘤的最大径达8 cm,家长放弃治疗,该例仅接受化疗6次.1例术后10年复查,精液内无精子.结论 小儿睾旁横纹肌肉瘤易于被检出,故多为Ⅰ期病变,且梭形细胞属良好型,经腹股沟切口高位精索离断、睾丸切除+VAC化疗,存活率达91%.由于环磷铣胺可致精液内无精子,故近年对Ⅰ期良好型病变可不用环磷铣胺.

Diagnosis and management of paratesticular rhabdomyosarcoma in childhood

Objective To improve the diagnostic accuracy and therapeutic effect of paratesticu-lar rhabdomyosarcoma (PARA RMS). Methods Fourteen patients diagnosed with PARA RMS from 1977 to June 2008 were retrospectively analyzed. The patients aged from 1 to 15 years, with the mean age of 6 years. Of all the cases, 6 were younger than 5 years. Nine sinistral and 5 dextral PARA RMSs were diagnosed. All patients manifested as scrotum swollen and diagnosed with PARA RMS at stage Ⅰ by roentgenographic findings. Of all the patients, 10 underwent radical inguinal orchiectomy, and 4 patients who had undergone orchiectomy ( 1 case) or tumor enucleation (3 cases) through a trans-scrotal incision in other hospitals underwent inguinal exploration with removal of the testis or the remaining sperrnatic cord. Excision of the scar of scrotal incision were also performed on the 4 above-mentioned patients. Thirteen cases were diagnosed as the spindle cell type of embryonic RMS and one the as the gland alveolar type. Chemotherapy (VAC, vincristine + actinomycin + cyclophosphamide) was performed on all patients. Results Thirteen of 14 patients were followed up for 1 to 24 years (mean, 6 years). Eleven patients were followed up for at least 2 years, including 10 (91%) survivors without tumor recurrence. For the other one patient with the age of 8 years, who underwent only 6 periods of chemotherapy and whose parents gave up the following chemotherapy, had the recurrence of a retroperitoneal metastatic tumor with the diameter more than 8 cm at 2 years postoperatively. A 9 year old patient who was admitted in 1981 had no sperm in his semen at follow-up. Conclusions PARA RMS often shows as a unilateral scrotal swelling or mass, thus it could be diagnosed earlier than other genitourinary tumors. Most cases of early diagnosed with PARA RMS are at stage Ⅰ, with the pathologic diagnosis of spindle cell type which indicated a good prognosis. The curative rate for pa-tients in stage 1 can be up to 90% after radical inguinal orchiectomy combined with VAC chemothera-py. In recent years, only VCR+ ACTD is suggested for type 1 PARA RMS for 1 year, to avoid infer-tility.