遗传性嗜铬细胞瘤和散发性嗜铬细胞瘤手术切除对比分析:来自单中心经验的启示。

The Kaohsiung journal of medical sciences Pub Date : 2024-06-01 Epub Date: 2024-05-15 DOI:10.1002/kjm2.12836
Narin Nasiroglu Imga, Muzaffer Serdar Deniz, Belma Ozlem Tural Balsak, Yilmaz Aslan, Altug Tuncel, Dilek Berker
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摘要

嗜铬细胞瘤是一种肿瘤,通常起源于肾上腺髓质绒毛细胞,产生一种或多种儿茶酚胺,可表现为遗传性或散发性。虽然大多数嗜铬细胞瘤为散发性,但遗传性嗜铬细胞瘤通常与遗传综合征有关,如冯-希佩尔-林道综合征、多发性内分泌瘤病 2 型和神经纤维瘤病 1 型。本研究旨在分析我们一系列经手术激发的嗜铬细胞瘤患者的数据,并比较遗传性和散发性病例的特征。我们对 33 例确诊的嗜铬细胞瘤患者进行了回顾性评估,记录了遗传性和散发性病例的临床特征、手术并发症和肿瘤特征。其中,21%(7 人)为遗传性嗜铬细胞瘤,79%(26 人)为散发性病例。在诊断期间,遗传性嗜铬细胞瘤患者的平均年龄明显低于散发性患者(26.4 ± 9.9 岁 vs. 50.4 ± 14.0 岁;P<0.05)。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A comparative analysis of surgically excised hereditary and sporadic pheochromocytomas: Insights from a single-center experience.

Pheochromocytoma is a tumor that usually originating from adrenal medullary chromaffin cells and producing one or more catecholamines, can manifest as hereditary or sporadic. While the majority pheochromocytomas are sporadic, hereditary forms are often associated with genetic syndromes such as von Hippel-Lindau, multiple endocrine neoplasia type 2, and neurofibromatosis type 1. This study aims to analyze data from our series of surgically excited pheochromocytoma patients and compare the characteristics between hereditary and sporadic cases. We retrospectively evaluated 33 diagnosed pheochromocytoma patients, documenting clinical features, surgical complications, and tumor characteristics in both hereditary and sporadic cases. Among the patients, 21% (7 individuals) had hereditary pheochromocytoma, while 79% (26 individuals) had sporadic cases. During diagnosis, hereditary pheochromocytoma patients exhibited a significantly lower mean age compared to the sporadic group (26.4 ± 9.9 years vs. 50.4 ± 14.0 years; p < 0.001). The maximum tumor size was also lower in hereditary cases compared to sporadic cases (p = 0.004). Adrenal tumor localization analysis showed that 63.6% were right-sided, 24.2% were left-sided, and 12.1% were bilateral. Laboratory analysis revealed significantly higher urinary norepinephrine levels in hereditary pheochromocytoma patients (p = 0.021). Our findings suggest that hereditary pheochromocytoma cases are characterized by a younger age at diagnosis, smaller tumor size, and a higher prevalence of multiple bilateral adrenal adenomas. We recommend genetic testing for all pheochromocytoma patients, particularly those with early-onset disease and bilateral adrenal tumors.

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