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睾丸横纹肌肉瘤的临床分析

[Clinical analysis of testicular rhabdomyosarcoma].

作者信息

Ye J F, Zhao L, Wang G L, Hong K, Ma L L

机构信息

Department of Urology, Peking University Third Hospital, Beijing 100191, China.

出版信息

Beijing Da Xue Xue Bao Yi Xue Ban. 2021 Dec 18;53(6):1178-1182. doi: 10.19723/j.issn.1671-167X.2021.06.028.

Abstract

Testicular rhabdomyosarcoma is relatively rare in testicular tumors, but the age of patient is relatively young and the degree of malignancy is high. Therefore, this article introduces 4 cases of testicular rhabdomyosarcoma who were admitted to Peking University Third Hospital from May 1994 to February 2019, and reviews the literature to improve the diagnosis and treatment of this disease. The average age of the 4 patients was 17.5 years (14-21 years), the average hospital stay was 22.0 d (17-31 d), and the average body mass index was 19.6 kg/m (14.7-25.8 kg/m). All the patients underwent routine preoperative blood and urine routine, biochemical tests, as well as serum tumor markers. Preoperative examinations also included chest radiograph, electrocardiogram, ultrasound of the scrotum and groin, and abdominal enhanced CT. Lung CT or other examinations were performed if necessary. The median serum human chorionic gonadotropin (HCG) of the 4 patients was 0.20 IU/L (0.06-0.86 IU/L) (all normal), and the median serum alpha-fetoprotein (AFP) was 1.03 g/L (0.65-1.66 g/L) (all normal). The average maximum diameter of the tumor was 10.0 cm (4.5-15.0 cm). Testicular rhabdomyosarcoma was mainly diagnosed by pathology. The main treatment was radical orchiectomy combined with retroperitoneal lymph node dissection, with or without postoperative adjuvant chemotherapy. The clinical manifestations of the patients with testicular rhabdomyosarcoma had no specific characteristics, but most patients were young at onset with mainly painless masses in the testicles, which were already large when they were found. Patients with testicular rhabdomyosarcoma have a poor prognosis, most of whom recur within two years. Because of the small number of cases of testicular rhabdomyosarcoma, there is no standard treatment currently. It is recommended that patients with testicular rhabdomyosarcoma undergo radical testicular resection combined with retroperitoneal lymph node dissection. Retroperitoneal lymph node metastasis is an important prognostic factor, and patients with postoperative adjuvant chemotherapy can still survive for a longer time. If local recurrence or limited metastasis is found after operation, local resection and salvage radiotherapy are feasible.

摘要

睾丸横纹肌肉瘤在睾丸肿瘤中相对少见,但患者年龄相对较小且恶性程度高。因此,本文介绍了1994年5月至2019年2月北京大学第三医院收治的4例睾丸横纹肌肉瘤患者,并复习文献以提高该病的诊治水平。4例患者的平均年龄为17.5岁(14 - 21岁),平均住院天数为22.0天(17 - 31天),平均体重指数为19.6 kg/m²(14.7 - 25.8 kg/m²)。所有患者均进行了术前常规血常规、尿常规、生化检查以及血清肿瘤标志物检查。术前检查还包括胸部X线片、心电图、阴囊及腹股沟超声以及腹部增强CT。必要时进行肺部CT或其他检查。4例患者血清人绒毛膜促性腺激素(HCG)中位数为0.20 IU/L(0.06 - 0.86 IU/L)(均正常),血清甲胎蛋白(AFP)中位数为1.03 μg/L(0.65 - 1.66 μg/L)(均正常)。肿瘤平均最大直径为10.0 cm(4.5 - 15.0 cm)。睾丸横纹肌肉瘤主要依靠病理诊断。主要治疗方法为根治性睾丸切除术联合腹膜后淋巴结清扫术,术后可根据情况进行辅助化疗。睾丸横纹肌肉瘤患者的临床表现无特异性,但多数患者发病年龄较轻,主要表现为睾丸无痛性肿块,发现时肿块已较大。睾丸横纹肌肉瘤患者预后较差,多数在两年内复发。由于睾丸横纹肌肉瘤病例数较少,目前尚无标准治疗方案。建议睾丸横纹肌肉瘤患者行根治性睾丸切除术联合腹膜后淋巴结清扫术。腹膜后淋巴结转移是重要的预后因素,术后辅助化疗的患者仍可存活较长时间。如果术后发现局部复发或局限性转移,可行局部切除及挽救性放疗。

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