睾丸横纹肌肉瘤的诊治分析

doi:10.19723/j.issn.1671-167X.2021.06.028

摘要/Abstract

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

叶剑飞,赵磊,王国良,洪锴,马潞林. 睾丸横纹肌肉瘤的诊治分析[J]. 北京大学学报（医学版）, 2021, 53(6): 1178-1182.

YE Jian-fei,ZHAO Lei,WANG Guo-liang,HONG Kai,MA Lu-lin. Clinical analysis of testicular rhabdomyosarcoma[J]. Journal of Peking University (Health Sciences), 2021, 53(6): 1178-1182.

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参考文献 19

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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