高雄榮民總醫院 外科部 泌尿外科
Bilateral testicular adrenal rest tumors in an adult patient with congenital adrenal hyperplasia: a case report and review of literature
Chen-Yueh Wen, Chia-Cheng Yu, Yin-Shen Chen, I-Hsuan Alan Chen, Jen-Tai Lin
Divisions of Urology, Department of Surgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan
Intratesticular soft tissue mass usually presents testicular malignancy, benign lesion was relative rare. Among them, the epidermoid cyst was the commonly recognized benign intratesticular lesion. Herein, we presented a patient with benign intratesticular adrenal rest tumor and early diagnosis and management of testicular adrenal rest tumors (TART) with congenital adrenal hyperplasia (CAH) is essential for gonadal functions and prevention from infertility.
The patient was a 43-year-old man who presented with painless scrotal hard masses, growing in size gradually during the past 10 years. He complained heat sensation over scrotum in recent months and therefore came to our clinics for help. The masses were confirmed by ultrasound as left testis multifocal hyperechoic foci, up to 2.5 cm and right testis single focal hyperechoic lesion about 1.7 cm. Laboratory investigations were within normal ranges, with α-fetoprotein 6.7 ng/ml, and β human chorionic gonadotropin measuring less than 1.2 mIU/ml. Computed tomography revealed bilateral adrenal thickening, up to 2.5 cm in thickness and bilateral intra-testicular tumors which favored TRATs. After discussing with him, left radical orchiectomy was performed by personal reason. Grossly, the tumors were solid and grayish with cord lipoma. It was confined within the testicular parenchyma. Microscopically, it comprised sheet proliferation of polygonal cells with round nucleus and small nucleolus. Lipochrome pigment and rarely vacuoles in the cytoplasm was also found. Immunohistochemical stains revealed that the tumor cells were diffusely positive for synaptophysin. Above the findings, it’s consistent with TRAT. A course of Dexamethasone was started and there was marked reduction in the size of right testis with minimal induration after 5 months follow-up.
In summary, we reported a rare case of bilateral TARTs in an adult male with CAH. Histological evaluation is helpful for diagnosis of TART. Adequate steroid replacement with Dexamethasone is the first line treatment and leads to regression of testicular lesions in most of TARTs.