Park Se Yoon, Kwak Mi Kyung, Kim Hye Jeong, Park Hyeong Kyu, Suh Kyo-Il, Yoo Myung Hi, Jin So Young, Yun Sumi, Byun Dong Won
Division of Endocrinology and Metabolism, Departments of Internal Medicine Departments of Pathology, Soon Chun Hyang University College of Medicine Division of Endocrinology and Metabolism, Asan Medical Center, University of Ulsan College of Medicine Department of Diagnostic Pathology, Samkwang Medical Laboratories, Seoul, Korea.
Medicine (Baltimore). 2017 Dec;96(52):e9455. doi: 10.1097/MD.0000000000009455.
Adrenal myelolipomas are rare benign tumors, composed of a variable mixture of mature adipose tissue and hematopoietic tissue. These tumors are frequently detected incidentally and are usually asymptomatic, and hormonally inactive.
During a routine health checkup, a 52-year-old man was found to have a tumor on the bilateral adrenal glands. Abdominal computed tomography revealed a well-defined, heterogeneously enhanced bilateral adrenal mass, suggesting a myelolipoma.
The hormonal evaluation revealed adrenocorticotropic hormone (ACTH) dependent Cushing syndrome.
The patient underwent left adrenalectomy, and transsphenoidal resection of a pituitary mass. The final diagnosis was adrenal myelolipoma associated with Cushing disease.
Growth of right adrenal myelolipoma was detected during the 7-year follow-up. There were enhancing pituitary lesions in repeat magnetic resonance imaging of the sellar region, which implies persistent or recurrent pituitary adenoma. This case reinforces relationship between Cushing disease and adrenal myelolipoma.
To the best of our knowledge, this is the first reported pathologically confirmed bilateral adrenal myelolipoma associated with Cushing disease. This report supports the idea that ACTH is associated with the development of adrenal myelolipoma.
肾上腺髓脂肪瘤是一种罕见的良性肿瘤,由成熟脂肪组织和造血组织的可变混合物组成。这些肿瘤常为偶然发现,通常无症状且无激素活性。
在一次常规健康检查中,一名52岁男性被发现双侧肾上腺有肿瘤。腹部计算机断层扫描显示双侧肾上腺有边界清晰、强化不均匀的肿块,提示为髓脂肪瘤。
激素评估显示促肾上腺皮质激素(ACTH)依赖性库欣综合征。
患者接受了左肾上腺切除术和经蝶窦垂体肿块切除术。最终诊断为与库欣病相关的肾上腺髓脂肪瘤。
在7年随访期间发现右肾上腺髓脂肪瘤生长。蝶鞍区重复磁共振成像显示垂体病变有强化,这意味着垂体腺瘤持续存在或复发。该病例强化了库欣病与肾上腺髓脂肪瘤之间的关系。
据我们所知,这是首例经病理证实的与库欣病相关的双侧肾上腺髓脂肪瘤报告。本报告支持ACTH与肾上腺髓脂肪瘤发生有关的观点。