先天性肾上腺增生合并巨大肾上腺骨髓瘤1例。

IF 0.4 4区 医学 Q4 PUBLIC, ENVIRONMENTAL & OCCUPATIONAL HEALTH
Puerto Rico Health Sciences Journal Pub Date : 2020-06-01
Texell Longoria-Dubocq, Roberto Torres-Aguiar, Karla Ruiz-Vega, Rafael De Ayala-Hillmann, Reynold Lopez-Enriquez
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引用次数: 0

摘要

肾上腺偶发瘤是位于肾上腺的肿瘤,在与肾上腺疾病无关的影像学检查中发现。在其他情况下,当怀疑肾上腺激素分泌肿瘤时,如嗜铬细胞瘤或库欣病,可在放射学上发现肾上腺肥大。根据肾上腺偶发瘤是否产生激素,如醛固酮、皮质醇、雄激素或儿茶酚胺,可分为功能性或非功能性肾上腺偶发瘤。研究表明,约8%的肾上腺偶发瘤为肾上腺髓磷脂瘤(AGMs)。agm是非恶性肿块,如果肿块足够大,可导致重要器官和血管受压。先天性肾上腺增生(CAH)患者由于缺乏肾上腺素分泌,促肾上腺皮质激素(ACTH)水平趋于升高。CAHs患者用类固醇治疗,抑制ACTH水平,防止肾上腺增生。大约10%的agm发生在未经治疗的cah中。我们的患者是一名36岁的男性,由于CAH和间歇性腹痛而服用类固醇;CT扫描显示一个很大的左肾上腺肿块,使器官向右移位。病理分析显示AGM超过30 x 23.6 x 16.7 cm。本次年度股东大会是有史以来文献报道中规模最大的一次。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Rare Case of Congenital Adrenal Hyperplasia with Giant Adrenal Myelolipoma.

Adrenal incidentalomas are tumors located in the adrenal glands and found on imaging done for purposes not related to adrenal disease. In other cases adrenal mases can be radiologically found when an adrenal hormone secreting tumor is suspected, such as a pheochromocytoma or Cushing's diseases. Adrenal incidentalomas may be classified as functional or non-functional based on whether they produce hormones, such as aldosterone, cortisol, and androgens, or catecholamines. Studies indicate that around 8% of adrenal incidentalomas are adrenal gland myelolipomas (AGMs). AGMs are non-malignant masses that can cause the compression of vital organs and vessels if said masses become large enough. In patients with congenital adrenal hyperplasia (CAH), adrenocorticotropic hormone (ACTH) levels tend to be elevated due to the lack of adrenal-hormone production. Patients with CAHs are treated with steroids that suppress ACTH levels and prevent adrenal gland hyperplasia. Around 10% of AGMs are found in untreated CAHs. Our patient was a 36-year-old male who was on steroids due to CAH and intermittent abdominal pain; a CT scan revealed a large left adrenal mass that was displacing organs towards the right. Pathological analysis revealed an AGM exceeding 30 x 23.6 x 16.7 cm. This AGM is one of the largest ever to be reported in the literature.

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来源期刊
Puerto Rico Health Sciences Journal
Puerto Rico Health Sciences Journal PUBLIC, ENVIRONMENTAL & OCCUPATIONAL HEALTH-
CiteScore
0.60
自引率
0.00%
发文量
34
审稿时长
>12 weeks
期刊介绍: The Puerto Rico Health Sciences Journal (PRHSJ) is the scientific journal of the University of Puerto Rico Medical Sciences Campus. It was founded in 1982 as a vehicle for the publication of reports on scientific research conducted in-campus, Puerto Rico and abroad. All published work is original and peer-reviewed. The PRHSJ is included in PubMed/Medline, SCOPUS, Latindex, EBSCO, SHERPA/RoMEO, Science Citation Index Expanded (SciSearch®) and Journal Citation Reports/Science Edition. All papers are published both online and in hard copy. From its beginning, the PRHSJ is being published regularly four times a year. The scope of the journal includes a range of medical, dental, public health, pharmaceutical and biosocial sciences research. The journal publishes full-length articles, brief reports, special articles, reviews, editorials, case reports, clinical images, and letters arising from published material.
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