诊断学理论与实践

诊断学理论与实践 ›› 2022, Vol. 21 ›› Issue (05): 588-591.doi: 10.16150/j.1671-2870.2022.05.007

• 论著 • 上一篇    下一篇

21-羟化酶缺陷先天性肾上腺皮质增生患者睾丸肾上腺残基瘤超声特征分析

王之倩, 李敏, 于一飞, 周建桥()   

  1. 上海交通大学医学院附属瑞金医院超声诊断科，上海 200025
  • 收稿日期:2021-10-19 出版日期:2022-10-25 发布日期:2023-01-29
  • 通讯作者: 周建桥 E-mail:zjq11432@rjh.com.cn

Ultrasonographic characteristics of testicular adrenal residue tumor in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

WANG Zhiqian, LI Min, YÜ Yifei, ZHOU Jianqiao()   

  1. Department of Ultrasound, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China
  • Received:2021-10-19 Online:2022-10-25 Published:2023-01-29
  • Contact: ZHOU Jianqiao E-mail:zjq11432@rjh.com.cn

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摘要：

目的：总结存在21-羟化酶缺陷的先天性肾上腺皮质增生（congenital adrenocortical hyperplasia, CAH）男性患者睾丸残基瘤（testicular remnant tumor, TART）的超声特征性表现，提高超声医师对TART的认识。方法：收集存在21-羟化酶缺陷的CAH患者21例，患者在阴囊高频超声检测中发现睾丸内异常回声，并经病理或临床确诊为TART，观察其TART病灶的超声声像图，评估病灶累及睾丸的个数、生长方式、形态、边界、回声、血供特征以及附睾累及情况。结果：21例患者中，14例为双侧睾丸发病，且双侧具有相同的超声特征；7例为单侧发病。2例患者累及附睾。在总共35个睾丸病灶中，29个病灶（82.9%）沿睾丸纵隔生长，6个（17.1%）呈弥漫性生长。18个睾丸病灶呈低回声（51.4%），10个呈不均匀高回声（28.6%），7个为不均匀高回声伴粗大钙化（20%）。病灶形态主要表现为，不规则分叶状及类圆形，分别占85.7%（30/35）及14.3%（7/35）；62.8%（22/35）的病灶为富血供， 22.9%（8/35）及14.3%（5/35）的病灶为乏血供及无血供。结论：TART的超声表现多样化，但仍具有特征性，多表现为双侧睾丸发病，并沿睾丸纵隔生长的富血供、不规则低回声，且双侧病灶的超声特征相同，如结合患者的CAH病史，可为TART诊断提供线索。

关键词: 21-羟化酶缺陷, 先天性肾上腺皮质增生, 睾丸肾上腺残基瘤, 超声

Abstract:

Objective: To summarize the ultrasonographic characteristics of testicular adrenal tumor (TART) in male patients with congenital adrenocortical hyperplasia (CAH) due to 21-hydroxylase deficiency, and to improve the understanding of sonographers on TART. Methods: Twenty-one CAH patients due to 21-hydroxylase deficiency were collected. All patients were found to have abnormal echoes in the testis by scrotal high-frequency ultrasound examination and were diagnosed as TART. The ultrasonic images of TART lesions were observed, and the number and growth mode of the lesions involving the testis were evaluated. The morphology, boundary, echogenicity and blood supply characteristics, and epididymis involvement were observed. Results: Among the 21 patients, 14 patients had bilateral testicular lesions with the same ultrasound features, and seven patients were unilateral. Two patients were epididymal involvement. Among the total 35 testicular lesions, 82.9% (29/35) grew along the testicular mediastinum, 17.1% (6/35) grew diffusely. Hypoechoic lesions accounted for 51.4% (18/35), heterogeneous hyperechoic lesions accounted for 28.6% (10/35), and heterogeneous hyperechoic lesions with coarse calcification accounted for 20%(7/35). The lesion morphology mainly showed irregular lobular shape and round shape, accounting for 85.7% (30/35) and 14.3% (7/35), respectively. The rich blood supply lesions accounted for 62.8% (22/35), and 22.9% (8/35) and 14.3% (5/35) of the lesions were hypovascular or anvascular. Conclusions: Sonographic appearance of TART is variable but still characteristic. The lesions present as bilateral testis with rich blood supply and irregular hypoechoic growth along the mediastinum. The ultrasonic features of the bilateral lesions are the same. With the history of CAH, it might provide clues for diagnosis of TART.

Key words: 21-hydroxylase defect, Congenital adrenocortical hyperplasia, Testicular adrenal rest tumor, Ultrasound

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