睾丸及睾丸旁胚胎型横纹肌肉瘤三例诊疗报告及文献复习

Diagnosis and treatment of reproductive system of embryonal rhabdomyosarcoma of testis and around： a report of 3 cases

目的探讨生殖系统（特别是附睾和睾丸旁）胚胎型横纹肌肉瘤的发病机制、临床表现、治疗方案及病理特征等情况。方法我院于2009年6月至2014年10月间共收治生殖系统横纹肌肉瘤3例，后经术后病理分析证实均为附睾（及睾丸）部位的胚胎型横纹肌肉瘤。该病主要通过术中快速冰冻病理进行肿瘤性质的初步判断，并通过手术进行病变部位切除（患侧睾丸、附睾及部分精索切除术），术后通过与家属沟通，2例进行VCP方案及IeV方案交替应用的规律化疗后出院，1例由于家属拒绝化疗出院。结果术后半年内每月1次病患部位的彩超检查及肿瘤标志物复查；半年后每3个月1次的彩超检查及肿瘤标志物复查。2例接受化疗的患儿均获得2年或5年的无瘤生存，由于另1例患儿未行化疗，同时拒绝术后的肿瘤标志物检测，决定对其进行了更密切的彩超检测，距最后复查患儿已无瘤生存8个月。结论横纹肌肉瘤是一种高度恶性的肿瘤，较易复发及转移，但胚胎型横纹肌肉瘤在该类肿瘤中属预后较好型，生殖系统特别是睾丸旁横纹肌肉瘤手术完整切除较容易，术区种植可能性小。术后观察进行规律化疗患儿获得较长期的无瘤生存，未进行化疗则获得短期内无瘤生存，长期效果有待进一步观察。

Objective To explore the pathogenesis, clinical manifestations, treatment and pathologic characteristics of reproductive embryonal rhabdomyosarcoma （especially ones around epididymis and testes）. Methods The clinical data of 3 children with reproductive rhabdomyosarcoma from June 2009 to October 2014 were retrospectively studied. And a preliminary diagnosis was made by intraoperative rapid frozen pathology. And the lesions were resected by removing unilateral testicle, epididymis and partial spermatic cord. Through postoperative communications with patient relatives, two cases received chemotherapy regimen of alternating vincristine, cytoxan, prednisone （VCP） with ifosfamide, etoposide, vincristine （IeV） and one case declined chemotherapy. Results After discharge, regular color ultrasound examinations and tumor marker detection were performed monthly for 6 months and then every 3 months for another 6 months. Two children on chemotherapy achieved 2/5-year tumor-free survival. Another child was merely examined by color ultrasound and achieved 8-month tumor-free survival. Conclusions Rhabdomyosarcoma is highly malignant with a frequent tendency of recurrence and metastasis. But its prognosis is generally fair. The tumor in reproductive system, especially ones around testis, may be easily resected without intraoperative seeding. After postoperative observations, the patients on regular chemotherapy may achieve a long- term disease-free survival while those not on chemotherapy have a short-term disease-free survival. The long-term survival should be observed further.