Congenital adrenal hyperplasia and Leydig cell tumor of testis. Case report and review of literature.
Congenital adrenal hyperplasia and Leydig cell tumor of testis. Case report and review of literature.
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先天性肾上腺增生症和睾丸间质细胞瘤。
DOI：
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发表时间：
2010
影响因子：
0.8
通讯作者：
S. Canonico
中科院分区：
文献类型：
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作者：
A. Santoriello;R. Benevento;P. Petronella;G. Perna;S. Canonico
肾上腺生殖器综合征，更准确的定义是先天性肾上腺增生症（CAH），与21- β -羟化酶缺乏有关，伴有糖皮质激素和醛固酮合成受损，ACTH合成增加。本报告描述了一例患有肾上腺生殖器综合征和睾丸间质细胞瘤的monorchid患者。在告知患者阉割的后果并征得其同意后，进行了右侧睾丸切除术并植入睾丸假体。组织学显示睾丸大小为4 × 3 × 2.5 cm，精索长6 cm；可见3.5 × 1.5 cm的淡黄色、界限分明的结节，周围为正常实质。该结节具有间质细胞瘤的形态和免疫组织化学特征，甚至在精索中也能发现；这些细胞对inibine、MART-1和vimentine呈阳性。
Adrenogenital Syndrome, more properly defined as Congenital Adrenal Hyperplasia (CAH), is related to the enzyme 21-beta-hydroxylase deficiency, with impaired glucocorticoids and aldosterone syntheses and increased ACTH synthesis. This report describes a case of a monorchid patient suffering from Adrenogenital Syndrome and Leydig cell tumor of his testis. A right orchidectomy with implantation of testis prosthesis was performed, after informing the patient on the consequences of his castration and obtaining his consent. Histology showed a testis measuring 4 x 3 x 2.5 cm with a 6 cm long spermatic cord; there was a yellowish, well-defined nodule measuring 3.5 x 1.5 cm, surrounded by normal parenchyma. This nodule had morphologic and immunohistochemical characteristics of a Leydig cell tumor, even found in the spermatic cord; those cells showed positivity to inibine, MART-1 and vimentine.