2nd Department of Medicine, Semmelweis University, Szentkirályi str. 46., Budapest, 1088, Hungary.
MTA-SE Molecular Medicine Research Group, Hungarian Academy of Sciences and Semmelweis University, Szentkirályi str. 46., Budapest, 1088, Hungary.
Endocrine. 2018 Jan;59(1):7-15. doi: 10.1007/s12020-017-1473-4. Epub 2017 Nov 21.
Adrenal myelolipoma is an invariably benign neoplasm of the adrenal gland that is the second most common primary adrenal incidentaloma following adrenocortical adenomas. It is composed of elements of adipose tissue and extramedullary hematopoiesis. Hypotheses on stem cells and hormonal factors have been formulated regarding its pathogenesis that is still obscure. Despite its benign behavior, adrenal myelolipoma is clinically relevant as it might cause significant difficulties in the differential diagnosis of adrenal tumors.
We have reviewed 420 cases reported between 1957 and 2017 on adrenal myelolipoma retrieved from PubMed and Scopus databases and also 20 of our case series to provide a comprehensive analysis of their pathology, epidemiological and clinical features.
The average age for its diagnosis was 51 years, and no gender difference was observed. The average size of tumors was 10.2 cm. Congenital adrenal hyperplasia was associated to 10% of all cases analyzed, while other adrenal hypersecretory disorders (cortisol, aldosterone) were found in 7.5% of cases. Computed tomography and magnetic resonance imaging can be reliably used for its differential diagnosis. If the diagnosis of an adrenal myelolipoma is unambiguous, and no associated symptoms or hormonal activity are established, surgical intervention is usually not necessary.
肾上腺髓样脂肪瘤是一种始终为良性的肾上腺肿瘤,是继肾上腺皮质腺瘤之后第二常见的原发性偶然肾上腺肿瘤。它由脂肪组织和骨髓外造血成分组成。关于其发病机制,已经提出了关于干细胞和激素因素的假说,但仍不清楚。尽管肾上腺髓样脂肪瘤具有良性行为,但在临床上具有重要意义,因为它可能在肾上腺肿瘤的鉴别诊断中造成重大困难。
我们回顾了 1957 年至 2017 年间在 PubMed 和 Scopus 数据库中检索到的 420 例肾上腺髓样脂肪瘤病例,以及我们的 20 例病例系列,以对其病理、流行病学和临床特征进行全面分析。
诊断时的平均年龄为 51 岁,男女之间无性别差异。肿瘤的平均大小为 10.2cm。分析的所有病例中,有 10%与先天性肾上腺增生有关,而在 7.5%的病例中发现了其他肾上腺分泌过多的疾病(皮质醇、醛固酮)。计算机断层扫描和磁共振成像可可靠地用于其鉴别诊断。如果明确诊断为肾上腺髓样脂肪瘤,且无相关症状或激素活性,则通常不需要手术干预。