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一名非经典型先天性肾上腺皮质增生患者的双侧睾丸肾上腺残余肿瘤。

Bilateral testicular adrenal rest tumors in a patient with nonclassical congenital adrenal hyperplasia.

作者信息

Roberts Erica C, Nealon Samantha W, Dhillon Jasreman, Tourtelot John B, McIver Bryan, Sexton Wade J

机构信息

Department of Genitourinary Oncology H. Lee Moffitt Cancer Center Tampa Florida USA.

Department of Anatomic Pathology H. Lee Moffitt Cancer Center Tampa Florida USA.

出版信息

IJU Case Rep. 2021 May 3;4(4):243-246. doi: 10.1002/iju5.12299. eCollection 2021 Jul.

Abstract

INTRODUCTION

Solid testis tumors in post-pubertal males usually represent germ cell malignancies; however, other uncommon or rare histologies must be considered.

CASE PRESENTATION

We present a case of an 18-year-old male undergoing attempted bilateral partial orchiectomies for suspected germ cell tumors. Tumor pathology, laboratory results, radiographic studies, and post-surgical elevated adrenocorticotropic hormone levels supported the diagnosis of testicular adrenal rest tumors secondary to previously undiagnosed nonclassical congenital adrenal hyperplasia.

CONCLUSION

Testicular adrenal rest tumors are rare in patients with nonclassical congenital adrenal hyperplasia and may be accompanied by adrenal insufficiency and hypogonadism, which can be treated with glucocorticoid therapy and testosterone replacement. Differential diagnosis of tumors is challenging but necessary for proper symptom-based management.

摘要

引言

青春期后男性的实性睾丸肿瘤通常为生殖细胞恶性肿瘤;然而,必须考虑其他不常见或罕见的组织学类型。

病例介绍

我们报告一例18岁男性,因疑似生殖细胞肿瘤接受双侧部分睾丸切除术。肿瘤病理学、实验室检查结果、影像学研究以及术后促肾上腺皮质激素水平升高支持了继发于先前未诊断的非经典先天性肾上腺皮质增生的睾丸肾上腺残余肿瘤的诊断。

结论

睾丸肾上腺残余肿瘤在非经典先天性肾上腺皮质增生患者中罕见,可能伴有肾上腺功能不全和性腺功能减退,可通过糖皮质激素治疗和睾酮替代治疗。肿瘤的鉴别诊断具有挑战性,但对于基于症状的适当管理是必要的。

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