北京大学学报(医学版) ›› 2021, Vol. 53 ›› Issue (6): 1178-1182. doi: 10.19723/j.issn.1671-167X.2021.06.028

• 疑难/罕见病例分析 • 上一篇    下一篇

睾丸横纹肌肉瘤的诊治分析

叶剑飞,赵磊,王国良,洪锴,马潞林()   

  1. 北京大学第三医院泌尿外科,北京 100191
  • 收稿日期:2019-03-15 出版日期:2021-12-18 发布日期:2021-12-13
  • 通讯作者: 马潞林 E-mail:malulin@bjmu.edu.cn
  • 基金资助:
    北大医学青年科技创新培育基金(BMU2018PYB008);北京大学第三医院临床重点项目(BYSY2018047)

Clinical analysis of testicular rhabdomyosarcoma

YE Jian-fei,ZHAO Lei,WANG Guo-liang,HONG Kai,MA Lu-lin()   

  1. Department of Urology, Peking University Third Hospital, Beijing 100191, China
  • Received:2019-03-15 Online:2021-12-18 Published:2021-12-13
  • Contact: Lu-lin MA E-mail:malulin@bjmu.edu.cn
  • Supported by:
    Peking University Medicine Fund of Fostering Young Scholars’ Scientific & Technological Innovation(BMU2018PYB008);Key Clinical Projects of Peking University Third Hospital(BYSY2018047)

摘要:

睾丸横纹肌肉瘤较为罕见,本文回顾性分析北京大学第三医院自1994年5月至2019年2月收治的4例睾丸横纹肌肉瘤的患者资料,主要治疗方案为睾丸根治性切除联合腹膜后淋巴结清扫,根据情况行术后辅助化疗。4例睾丸横纹肌肉瘤患者平均年龄17.5岁(14~21岁),平均住院时间22.0 d(17~31 d),平均体重指数19.6 kg/m2(14.7~25.8 kg/m2),平均肿瘤最大径为10.0 cm(4.5~15.0 cm)。中位血清绒毛膜促性腺激素(chorionic gonadotropin,HCG)0.20 IU/L(0.06~0.86 IU/L),中位血清甲胎蛋白(alpha-fetoprotein,AFP)1.03 g/L(0.65~1.66 g/L),均为正常。睾丸横纹肌肉瘤患者预后较差,多数在两年内复发。推荐行睾丸根治性切除合并腹膜后淋巴结清扫,腹膜后淋巴结转移是重要的预后不良因素,术后辅助化疗的患者可获得较长生存时间。

关键词: 睾丸肿瘤, 横纹肌肉瘤, 睾丸切除术, 淋巴结切除术

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/m2 (14.7-25.8 kg/m2). 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.

Key words: Testicular neoplasms, Rhabdomyosarcoma, Orchiectomy, Lymph node excision

中图分类号: 

  • R737.21

表1

睾丸横纹肌肉瘤患者的基本资料"

Items Case 1 Case 2 Case 3 Case 4
Basic data
Age/years 19 16 14 21
Hospital stay/d 21 17 31 19
BMI/(kg/m2) 22.03 16.02 25.83 14.67
Side Right Right Left Left
Chief complain Palpable mass in the right
scrotum for 1 month
Palpable mass in the right
scrotum for 6 months
Painful mass in the left
testis for 2 months
Palpable mass in the left
scrotum for 2 weeks
Perioperative data
HCG/(IU/L) 0.2 0.86 0.06
AFP/(μg/L) 0.65 0.76 1.30 1.65
LDH/(U/L) NA NA 0.68 146.00
Maximum dialater/cm 10.0 15.0 10.5 4.5
Approach Inguinal Inguinal Inguinal Inguinal
Surgery Radical orchiectomy Radical orchiectomy Radical orchiectomy Radical orchiectomy
RPLND No No Yes Yes
Follow-up Died 3 years later Relapse after 11 months Relapse twice after
17 months and 41 months
Alive without relapse

图1

睾丸横纹肌肉瘤的超声表现"

图2

睾丸横纹肌肉瘤的病理表现(HE ×200)"

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