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病例报告:一个引起你注意的偶发瘤——肾上腺髓脂肪瘤。

Case Report: An incidentaloma that catches your eye - adrenal myelolipoma.

作者信息

D'Addosio Rosanna, Rojas Joselyn, Bermúdez Valmore, Ledesma Flor, Hoedebecke Kyle

机构信息

Department of Public Health, School of Medicine, University of Zulia, Maracaibo, 4002, Venezuela.

Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, 02115, USA.

出版信息

F1000Res. 2017 Jul 18;6:1140. doi: 10.12688/f1000research.11766.1. eCollection 2017.

Abstract

: Adrenal incidentaloma refers to the incidental finding of a tumor in the adrenal gland, where nonfunctional forms are the most common variant. Myelolipoma is a rare (0.08-0.4%) occurrence characterized by adipose and hematopoietic tissue. The aim of this case report is to describe the diagnosis and appropriate management of a myelolipoma in an asymptomatic patient, which was originally considered an incidental hepatic hemangioma prior to being identified as a giant adrenal adenoma. The patient was a 54 year old obese female with a recent diagnosis of diabetes type II and dyslipidemia with recent ultrasound imaging suggestive of a hepatic hemangioma. An MRI was performed revealing a 7x6cm lesion in the right adrenal area indicating a giant adrenal adenoma. An adrenalectomy was performed without complications. The pathology report identified a myelolipoma. The incidence of myelolipoma has recently increased due to advances in radiological techniques. Its etiology is unclear and the most accepted theories support a myeloid cell metaplasia in the embryonic stage as a result of stress, infections, or adrenocorticotropic hormone or erythropoietin stimulus. Contributing components may include bone morphogenetic protein 2 and β-catenin, as well as the presence of the chromosomal translocation (3, 21) (q25; p11). Despite its benign nature, the association with other adrenal lipomas must be ruled out. A biochemical evaluation is essential for detecting subclinical states, such as Cushing syndrome and pheochromocytoma. Adrenal myelolipomas are rare benign tumors that are generally asymptomatic. Uncertainty still exists surrounding their etiology. Surgical management depends on hormone production, tumor size, high risk features on imaging and patient consent.  Additional information is needed to better understand myelolipomas, their etiology, and clinical management.  Incidentalomas may confuse the physician and patient. Ensuring proper multidisciplinary management based on the clinical guidelines of endocrinology allowed a satisfactory resolution of this case.

摘要

肾上腺偶发瘤是指在肾上腺意外发现的肿瘤,其中无功能型是最常见的类型。肾上腺髓质脂肪瘤是一种罕见的(发生率为0.08 - 0.4%)肿瘤,其特征为含有脂肪组织和造血组织。本病例报告的目的是描述一名无症状患者肾上腺髓质脂肪瘤的诊断及恰当治疗,该肿瘤最初被认为是偶然发现的肝血管瘤,之后才被确定为巨大肾上腺腺瘤。患者为一名54岁肥胖女性,近期诊断为II型糖尿病和血脂异常,近期超声检查提示有肝血管瘤。随后进行的磁共振成像(MRI)显示右肾上腺区域有一个7×6厘米的病灶,提示为巨大肾上腺腺瘤。患者接受了肾上腺切除术,未出现并发症。病理报告确诊为肾上腺髓质脂肪瘤。由于放射技术的进步,肾上腺髓质脂肪瘤的发病率近来有所上升。其病因尚不清楚,最被认可的理论认为是胚胎期髓样细胞化生,原因是应激、感染、促肾上腺皮质激素或促红细胞生成素刺激。促成因素可能包括骨形态发生蛋白2和β-连环蛋白,以及染色体易位(3, 21)(q25;p11)。尽管其为良性,但必须排除与其他肾上腺脂肪瘤的关联。生化评估对于检测亚临床状态(如库欣综合征和嗜铬细胞瘤)至关重要。肾上腺髓质脂肪瘤是罕见的良性肿瘤,通常无症状。其病因仍存在不确定性。手术治疗取决于激素分泌情况、肿瘤大小、影像学上的高危特征以及患者的意愿。需要更多信息来更好地了解肾上腺髓质脂肪瘤、其病因及临床治疗。偶发瘤可能会使医生和患者感到困惑。依据内分泌学临床指南确保进行恰当的多学科管理,使该病例得到了令人满意的解决。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4973/5538031/1e84cc28d465/f1000research-6-12710-g0000.jpg

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