Rajkanna J, Oyibo S O
Department of Endocrinology, Peterborough City Hospital Bretton Gate , Peterborough PE3 9GZ , UK.
Endocrinol Diabetes Metab Case Rep. 2015;2015:140080. doi: 10.1530/EDM-14-0080. Epub 2015 Feb 1.
Testicular adrenal rest tumours (TARTs) are benign ACTH-dependent tumours that occur in males with congenital adrenal hyperplasia (CAH) and if left untreated can destroy testicular tissue. Corticosteroid suppressive treatment could result in the regression of these testicular tumours. We present a patient with bilateral large TARTs as a consequence of poor compliance to treatment and follow-up for his CAH, who consequently had to have bilateral orchidectomies and prosthesis replacement.
TARTs are frequently seen in males with CAH, and can be misdiagnosed as primary testicular cancer.Patient compliance to treatment and follow-up are necessary to reduce the risk of testicular damage as a result of TARTs in patients with CAH.Boys with CAH should have periodic ultrasonographic screening from before adolescent age for early detection of TARTs.Regular monitoring of renin, 17-hydroxyprogesterone and androgens levels is required to assess corticosteroid suppressive treatment.Patients with CAH should be offered psychological support and information concerning CAH support groups.
睾丸肾上腺残余肿瘤(TARTs)是一种良性的促肾上腺皮质激素(ACTH)依赖性肿瘤,发生于患有先天性肾上腺皮质增生症(CAH)的男性患者中,若不治疗可破坏睾丸组织。皮质类固醇抑制治疗可使这些睾丸肿瘤消退。我们报告一例因对其CAH治疗和随访依从性差而出现双侧巨大TARTs的患者,因此不得不接受双侧睾丸切除术及假体置换。
TARTs在患有CAH的男性中常见,可能被误诊为原发性睾丸癌。患者对治疗和随访的依从性对于降低CAH患者因TARTs导致睾丸损伤的风险很有必要。患有CAH的男孩应在青春期前定期进行超声筛查,以便早期发现TARTs。需要定期监测肾素、17-羟孕酮和雄激素水平以评估皮质类固醇抑制治疗效果。应为CAH患者提供心理支持以及有关CAH支持小组的信息。