Forster Danielle Andrea, Marzouk Sherief, Chakraborti Indrani, Berney Daniel, Wolfe Konrad, Liyanage Sidath H
Department of Radiology, University College London Hospital, London, United Kingdom.
Department of Radiology, Southend University Hospital, England, United Kingdom.
BJR Case Rep. 2022 Nov 1;8(6):20220072. doi: 10.1259/bjrcr.20220072.
Testicular adrenal rest tumours (TART) are found in patients with congenital adrenal hyperplasia (CAH) with the severity of testicular infiltration linearly related to the degree of enzymatic defect and subsequent compliance with treatment. We report a highly unusual case of TART in an adult patient with CAH caused by 21-hydroxylase deficiency who had not engaged with health services over a 3-year period. Typical imaging features of TART include bilateral well-defined lesions adjacent to the rete testes. However, in this rare case, the follow-up imaging found that the entirety of the testicular parenchyma had been replaced with TART and the patient had gone on to develop an adrenal nodule. As these testicular tumours are commonly misdiagnosed as primary germ tumours and tend respond well to treatment with circadian or reverse glucocorticoids, it is essential for the radiologist to be aware of both the common and more unusual imaging features appearances of TART in patients with CAH in order to facilitate early diagnosis and thus timely initiation of treatment.
睾丸肾上腺残余肿瘤（TART）见于先天性肾上腺皮质增生症（CAH）患者，睾丸浸润的严重程度与酶缺陷程度及后续治疗依从性呈线性相关。我们报告了一例极为罕见的TART病例，患者为一名成年21 - 羟化酶缺乏所致CAH患者，其在3年期间未接受医疗服务。TART的典型影像学特征包括紧邻睾丸网的双侧边界清晰的病变。然而，在这个罕见病例中，随访影像学检查发现整个睾丸实质已被TART取代，且患者继而出现了肾上腺结节。由于这些睾丸肿瘤常被误诊为原发性生殖细胞肿瘤，且对昼夜或反向糖皮质激素治疗反应良好，放射科医生必须了解CAH患者中TART常见及更不寻常的影像学表现，以便于早期诊断并及时开始治疗。
