Ali Hiba Hassan, Samkari Alaa, Arabi Haitham
Department of Anatomic Pathology, Faculty of Medicine, King Abdul-Aziz University, Jeddah, Saudi Arabia.
Avicenna J Med. 2013 Jan;3(1):15-9. doi: 10.4103/2231-0770.112789.
Congenital adrenal hyperplasia (CAH) refers to group of inherited diseases resulting from impaired adrenal steroidogenesis, and its most common cause is 21-hydroxylase deficiency. Testicular adrenal rest tumors (TARTs) are an important complication of CAH, which probably develop from ectopic remnants of intra-testicular adrenal tissue stimulated by Adrenocorticotropic hormone (ACTH) hypersecretion. These lesions are typically located within the rete testis and are bilateral, synchronous, nodular and multiple. TART usually, but not always, responses to suppressive medical therapy. TART leads to testicular structural damage, spermatogenesis disorders, infertility and most importantly, mass-forming lesions that could be mistaken for Leydig cell tumor (LCT). The later has a significantly different behavior with up to 10% of being malignant. Nowadays, due to advances in diagnosing and treating CAH, mass-forming TART is rarely encountered. As a result, there is the paucity in the medical literature regarding its features from pathological perspective. We herein present a case of mass-forming TART and we discuss the clinical, radiological, and morphological features as well as the major differential diagnosis of this rare lesion.
先天性肾上腺皮质增生症（CAH）是指因肾上腺类固醇生成受损而导致的一组遗传性疾病，其最常见的病因是21-羟化酶缺乏。睾丸肾上腺残余瘤（TARTs）是CAH的一种重要并发症，可能由促肾上腺皮质激素（ACTH）分泌过多刺激睾丸内肾上腺组织的异位残余物发展而来。这些病变通常位于睾丸网内，呈双侧、同步、结节状且多发。TART通常（但并非总是）对抑制性药物治疗有反应。TART会导致睾丸结构损伤、生精障碍、不育，最重要的是会形成可被误诊为睾丸间质细胞瘤（LCT）的肿块性病变。后者的行为有显著差异，高达10%为恶性。如今，由于CAH诊断和治疗的进展，很少遇到形成肿块的TART。因此，从病理学角度来看，医学文献中关于其特征的报道很少。我们在此报告一例形成肿块的TART病例，并讨论该罕见病变的临床、放射学和形态学特征以及主要鉴别诊断。
