特殊病例的单中心经验:孤立性肾上腺骨髓瘤和伴骨髓瘤成分的肾上腺皮质腺瘤。

IF 0.6 4区 医学 Q4 UROLOGY & NEPHROLOGY
Serhat Çetin, Kadir Şerefhan Erten, Metin Onaran, İlker Şen, Mustafa Özgür Tan, İpek Işık Gönül, Ethem Turgay Cerit, Tevfik Sinan Sözen
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引用次数: 0

摘要

目的:孤立性肾上腺骨髓瘤(IAM)是一种罕见的肾上腺无功能肿瘤,主要由脂肪组织和造血三线状细胞组成。这种肿瘤的发病机制和临床意义仍然知之甚少。然而,类似的骨髓脂肪瘤改变也可发生在肾上腺皮质腺瘤中。本报告介绍了我们对IAM和肾上腺皮质腺瘤伴髓脂瘤成分(AMC)的研究结果,重点比较了这些病例的临床、人口学和病理特征。方法:回顾性分析20例患者的资料,其中12例诊断为IAM, 8例诊断为AMC,均行肾上腺切除术。在IAM队列中,手术干预的指征主要基于肿瘤大小和/或相关症状,而在AMC病例中,指征通常由激素活动驱动。结果:IAM患者平均年龄52.5岁,以女性患者居多(75%)。该组中有相当大比例的人肥胖,并伴有高血压(HT)和/或2型糖尿病(DM)。肿瘤以右侧为主,中位大小69.0±40.0 mm。值得注意的是,58%的IAM患者表现为由肿块效应引起的腹部或腹部疼痛。所有IAM病例均未表现出激素活动。相反,AMC组的平均年龄较年轻,为46岁,女性患者的患病率也很高(63%)。肿瘤一般较小,中位尺寸为40.0±16.0 mm,多位于左侧。本组患者均有HT和/或DM病史,其中6例出现激素活性肿瘤,表现为各种临床综合征,包括库欣综合征、嗜铬细胞瘤和Conn综合征。结论:虽然IAM和AMC有一些共同的特征,但它们也表现出明显的差异。内分泌综合征的出现在AMC病例中更为常见,而IAM病例很少显示激素活动。两组之间最显著的差异是诊断时的肿瘤大小,这导致了入院时不同的临床表现。此外,两组患者中肥胖、HT和DM的高患病率表明,这些合并症可能在这些肿瘤中观察到的骨髓瘤模式的发展中起作用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Single Center Experience of Special Cases: Isolated Adrenal Myelolipoma and Adrenocortical Adenoma with Myelolipomatous Component.

Objective: Isolated adrenal myelolipoma (IAM) is an uncommon, nonfunctioning tumour of the adrenal gland, primarily composed of adipose tissue and hematopoietic trilinear cells. The etiopathogenesis and clinical relevance of this neoplasm remain poorly understood. However, similar myelolipomatous alterations can also occur within adrenocortical adenomas. This report presents our findings on IAM and adrenocortical adenomas with a myelolipomatous component (AMC), focusing on comparing these cases' clinical, demographic, and pathological characteristics.

Methods: Data from twenty patients were retrospectively analyzed, including twelve diagnosed with IAM and eight with AMC, all of whom underwent adrenalectomy. In the IAM cohort, surgical intervention was primarily indicated based on tumour size and/or related symptoms, while in AMC cases, the indication was often driven by hormonal activity.

Results: Patients with IAM had an average age of 52.5 years, with a predominance of female patients (75%). A significant proportion of this group was obese, with concomitant hypertension (HT) and/or type 2 diabetes mellitus (DM). The tumours were predominantly right-sided, with a median size of 69.0 ± 40.0 mm. Notably, 58% of patients with IAM presented with flank or abdominal pain attributed to the mass effect. None of the IAM cases exhibited hormonal activity. Conversely, the AMC group had a younger average age of 46 years, with a high prevalence also in female patients (63%). The tumours were generally smaller, with a median size of 40.0 ± 16.0 mm, and were mostly left-sided. All patients in this group had a history of HT and/or DM, with six exhibiting hormonally active tumours, which manifested as various clinical syndromes, including Cushing syndrome, pheochromocytoma, and Conn syndrome.

Conclusions: While IAM and AMC share several common features, they also demonstrate distinct differences. The presence of endocrinological syndromes was more frequent in AMC cases, whereas IAM cases rarely showed hormonal activity. The most pronounced difference between the two groups was the tumour size at diagnosis, which contributed to the varying clinical presentations upon hospital admission. Furthermore, the high prevalence of obesity, HT, and DM in both groups suggests that these comorbidities may play a role in the development of myelolipomatous patterns observed in these tumours.

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来源期刊
Archivos Espanoles De Urologia
Archivos Espanoles De Urologia UROLOGY & NEPHROLOGY-
CiteScore
0.90
自引率
0.00%
发文量
111
期刊介绍: Archivos Españoles de Urología published since 1944, is an international peer review, susbscription Journal on Urology with original and review articles on different subjets in Urology: oncology, endourology, laparoscopic, andrology, lithiasis, pediatrics , urodynamics,... Case Report are also admitted.
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